Otosclerosis
Otosclerosis
Otosclerosis
Dear Colleagues,
It is my honour and pleasure to invite you to acquaint
yourselves with the abstracts of speeches presented
at the 4th International Symposium on Otosclerosis
and Stapes Surgery, which this year takes place in
the beautiful city of Cracow.
After such big scientific events held in Poland and
co-organized by the Institute of Physiology and
Pathology of Hearing as IX European Symposium
Paediatric Cochlear Implant (2009), X Congress
of the European Federation of Audiology Society
(2011), XXV International Evoked Response
Audiometry Study Group Biennial Symposium
(2017), XII International Tinnitus Seminar and
I World Tinnitus Congress (2017) this is another
unique meeting which, I hope, will result in reaching valuable conclusions that will be successfully
applied in clinical practice.
The scientific program of the Symposium focuses on problems of the middle ear, seeking to
exchange and propagate knowledge of the latest developments concerning otosclerosis and stapes
surgery. Otosclerosis is one of the most common causes of progressive deafness in young adults.
We know that hearing loss generally begins between the ages of 10 and 30, and, if untreated,
may eventually result in total deafness. Surgical intervention is a common and highly effective
treatment, and its success relies very much on the surgeon’s skill and experience.
Organization of the 2018 Symposium in Cracow is an occasion to commemorate the works
of Prof. Jan Miodoński (1902–63), a Polish otolaryngologist who was professor at Jagiellonian
University, a pioneer of audiometry and ear surgery, creator of Polish neurootology, and inventor
of the tympanoplasty procedure.
My hope is that the invited guests and speakers will bring valuable contributions to this meeting.
I am sure that the 4th International Symposia on Otosclerosis and Stapes Surgery will become
an important event in the history of otolaryngology and will bear fruit in terms of a growth in
expertise which will in turn maximise our success rate and benefit our patients.
I look forward to an interesting and productive meeting.
Sincerely yours,
Prof. Henryk Skarżyński, MD, PhD, dr h.c. (multi)
President of the 4th International Symposium
on Otosclerosis and Stapes Surgery
Dear Guests, Ladies and Gentlemen,
Colleagues and Friends, Fellow Members,
It is a great pleasure and honor to welcome you to
the 4th International Symposium on Otosclerosis and
Stapes Surgery.
We are here, in this distinguished group of specialists,
to spend these three upcoming days on a special
scientific and cultural event. On the scientific program
of the symposium you will find main lectures, round
table discussions, and workshops covering a wide
range of topics in the fields of middle ear problems,
otosclerosis and stapes surgery. I am convinced that
this meeting will be a perfect forum for exchanging
our knowledge and experience in the area.
The Symposium is the fourth in a series begun in
2004 in Saas Fe, Switzerland, and followed by a Symposium in Biarritz, France, in 2008 and
then in Siófok, Hungary, in 2014. The first two Symposia were organized under the auspices of
the Politzer Society; since 2014, it has been an independent Symposium.
The symposium takes place in parallel with the conference Guidelines in the otology of the
Society of Polish Otolaryngologists, Phoniatricians and Audiologists. I have the pleasure to
co-organize such an important scientific event for the third time. This is a continuation of the
meetings started two years ago, organized in Krynica Zdrój and in Zakopane under the patronage
of the National Consultant in the Field of Otorhinolaryngology. Both, first and second edition
gathered over 500 participants.
I believe that the 4th International Symposium on Otosclerosis and Stapes Surgery will meet
your expectations, and prove to be a valuable scientific event as well as a very pleasant social
and cultural experience.
Opening Session
Counselling patient surgery or hearing aids. window velocity in response to active stimulation of the
stapes in fixation and following fenestration.
Fraysse B., Molinier C.E.
Results: Endocochlear potentials and ABR recordings reflect-
Service ORL, Hôpital Pierre-Paul Riquet, Toulouse, France ed essentially identical responses to acoustical and round win-
dow mechanical stimulation. Fixation of the stapes showed
The author will describe the information given to the pa- similar pattern, though reduction of amplitude of response.
tients between surgery or hearing aids based on a pro- Stapes fixation resulted in a 4-13 dB shift in cochlear micro-
spective longitudinal study of 30 patients comparing au- phonics and 2-24 dB in compound action potential, being
diological outcomes with hearing aid then stapedotomy frequency dependent. These losses could easily be compen-
at 2 months. sated by the magnitude of the stimulator output. Similar re-
sults were seen in measuring the round window velocity in
Evaluation include pure tone audiogram speech discrimi- cadaveric specimen with response magnitude related to sev-
nation sound localization binaural hearing GHSI and mu- eral factors that improved coupling of the prostheses. Direct
sic perception. drive of the vestibule via a stapedotomy produced excellent
responses of the round window, equivalent to magnitudes as-
Is There a Role for Active Middle Ear sociated with normal inner ear stimulation.
Prostheses in Management
of Otosclerosis? Conclusion: Direct mechanical stimulation of the round
window with an active middle ear prosthesis is comparable
Jenkins H.A., Lupo E., Greene N., Tollin D. to acoustic stimulation. Effective stimulation can be pro-
duced in a variety of stimulations and pathological states.
University of Colorado School of Medicine, Aurora, Colorado, In situations in which it is difficult to reconstitute the nor-
USA mal conductive mechanism, active middle ear prostheses
offer an acceptable alternative.
Aim: Stapes surgery for conductive hearing loss second-
ary to otosclerotic fixation has been a mainstay in our Differential Diagnosis of Conductive and
treatment for many decades, since its rediscovery by Mixed Hearing Loss. A question of Imaging.
John Shea Jr. in 1956. Refinements have led to less inva-
sive fenestrations, self-crimping prostheses and introduc- Offeciers E.1 , Casselman J.W.2,3, De Foer B.2,
tion of lasers to minimize trauma to the inner ear. Even Zarowski A.1, van Dinther J.1, Somers Th.1
with these innovations, a significant number of patients
have persistent or delayed conductive loss due to com- 1
NT Department – European Institute for ORL-HNS,
E
plications. Repeated attempts to improve this situation St- Augustinus, Wilrijk, Belgium
surgically with revision stapes procedures often are un- 2
Department of Radiology, St- Augustinus, Wilrijk, Belgium
productive, depending on the middle ear changes with 3
Department of Radiology, AZ St-Jan Brugge AV, Brugge,
surgery. Much of the work over the last decade in our Belgium
laboratory has been to study the potential for use of ac-
tive middle ear prostheses. This presentation will discuss The lecture will illustrate how state-of-the-art imaging im-
our laboratory experience in animal and human models proves the pre-operative diagnostic work-up in patients
of simulated otosclerosis and review the current state of presenting with conductive or mixed hearing loss with an
surgical procedures. intact tympanic membrane, which is the typical presen-
tation status of otosclerosis patients. The imaging work-
Material and Methods: Artificial otosclerosis was sim- up helps to establish the differential diagnosis with other
ulated in the chinchilla with acrylic glue applied to the causes, helps to avoid surgical complications or surprises
oval window area. Animals were stimulated acoustically, as well as unwarranted interventions, and thus ultimately
followed by mechanical stimulation of the round window affects the therapeutic outcome.
in fixation and non-fixation states by an active middle
ear prosthesis. Cochlear microphonics, compound ac- The lower radiation burden of Conebeam CT and its
tion potential, and ABR were measured. Human cadav- improved spatial resolution (150 µm versus 500 µm) as
eric studies included laser Doppler measures of round compared to conventional multi-detector CT, has led the
authors to routinely apply it in the pre-operative work-up and breakthrough discoveries are so numerous that it is
of patients presenting with conductive or mixed hearing almost impossible to believe that they were accomplished
loss with an intact tympanic membrane. CBCT plays an by a single person. He was the first otosurgeon in the
important role in pre-operatively confirming the presumed world to perform tympanoplasty, the first otoneurologist
diagnosis, in identifying contra-indications to functional to describe the four vestibular caloric tests, the first head
surgery and in informing the surgeon about potential dif- and neck oncologist to introduce the subtotal partial lar-
ficulties and complications during surgery, thus allowing yngectomy. Why are these pioneer inventions attributed
the clinician to counsel the patient more comprehensive- to other scientists who published their works years after
ly and to better plan and prepare the operation. Miodoński? Why is the name of Jan Miodoński not wide-
ly recognized by the international medical community?
Presenting illustrative clinical examples, the authors will
treat in detail a list of ambiguities, generated by the tradi- To understand this paradox it is necessary to take a
tional diagnostic methods at our disposal (personal and look at the historical context of his life and work.He
family history, otoscopy, audiometry) and solved by pre- started his medical career directly after the World
operative diagnostic imaging. Some examples in point War I. At this time Poland was struggling with many
are: atypical history of hearing loss, suspected congeni- problems as it regained independence after 123 years
tal hearing loss, suspect otoscopic image, asymmetric BC of absence from the maps of Europe. Jan Miodoński
thresholds, profound mixed loss, suspect tympanometry worked with vigor and talent to create a modern lar-
and cases for revision stapes surgery. CBCT helps to doc- yngological clinic. Yet before the task was finished,
ument the diagnosis of the following pathologies: otoscle- another World War ruined his plans. Miodoński was
rotic and other stapes fixations, tympanosclerosis, post- arrested in a Nazi operation called the ‘Intelligenzak-
traumatic and post-inflammatory ossicular lesions, minor tion’ which was a plan to eradicate the Polish intellec-
middle ear dysplasia’s, labyrinth dysplasia’s and SCCD. MRI tual elite. He spent more than a year in a concentra-
can be used to exclude or confirm a schwannoma, a sus- tion camp. When the war was over, Poland fell under
pected congenital cholesteatoma or labyrinth dysplasia. the Soviet influence. Behind the Iron Curtain the sci-
entific ideas developed to some extent independent-
Pre-operative Conebeam CT imaging is very useful for the ly from the Western countries. It was not uncommon
honest and realistic pre-operative counselling of the patient that scientists were forbidden to participate in inter-
regarding the short and long term outcome of the surgical national congresses and conferences or discouraged
procedure. As such its use has medicolegal implications. to publish in languages other than Polish or Russian.
As a result Jan Miodoński lost a chance to present his
The aim of this lecture is to provide the clinician with the discoveries abroad or claim his priority when the in-
state-of-the-art information that allows him to ask his ra- ventions were later introduced by other researchers.
diologist the pertinent questions, thus fostering a closer
collaboration between the clinician and the radiologist. Miodoński’s areas of interest were very vast. He intro-
duced novel ideas in the physiopathology of hearing,
Professor Jan Miodoński - an unappreciated functional surgery of the middle ear, vestibular testing,
Polish pioneer in the history of treatment of otogenic cerebellar abscesses and larynx can-
otolaryngology. cer surgery. He was both a brilliant scientist and an ag-
ile surgeon. The reader studying his works is impressed
Szaleniec J. by the detailed descriptions of meticulous examination
of the patient and thorough logical analysis of clinical
Department of Otolaryngology, Jagiellonian University Medical observations. His educational texts were clear and com-
College, Krakow, Poland prehensive. But above all he was a man of high integ-
rity. He claimed, that a good physician must be a good
Professor Jan Miodoński is considered the greatest Polish man - and to this principle he struggled to remain true
otolaryngologist of the 20th century. His achievements in his whole life.
Our purpose is to analyze the dynamics of hearing reduction the molecular mechanisms involved in the disease patho-
and loss of dockyard workers by comprehensive screening ex- genesis. Although there are about 10 loci reported to date
aminations as a first step in a broad prevention programme. linked with the otosclerosis, only two possibly causative
genes (SERPINF1 and FOXL1) have been identified. Thus,
A total of 270 and 256 male workers from Varna dock- in such cases the optimal solution is to conduct a wide
yard are examined by otorhinolaryngologists in 2002 and research, which has become possible since the introduc-
2009, respectively. Because of subsequent enterprise clo- tion of next generation sequencing (NGS). For this pur-
sure after 2009, the number of the patients followed-up pose we decided to perform the clinical exome sequenc-
until present fell down to 25 only. The workers belong ing, which is a universal and powerful discovery tool to
to the following groups: helpingists (25%), pipe fitters reveal the genetic causes of diseases. The dedicated bioin-
(23%), ship-gear fitters and turners (22% each), moulders formatics pathway combined with thorough experts anal-
and woodworkers (3% each), and millers (2%). Otological ysis was applied to data analysis.
screening consists in comprehensive clinical investigations
such as otoscopy with microscopy, tympanometry, digital Genetic association analysis in Polish
tonal threshold and over-threshold audiometry, otoacous- patients with otosclerosis.
tic emissions, videonystagmography, video head impulse
test, routine blood tests, and, if necessary, consulting ex- Oziębło D., Adamiok A., Domagała S.,
aminations by neurologists, x-ray, magnetic resonance im- Skarżyński H., Ołdak M.
aging, and computer-assisted tomography.
World Hearing Center, Institute of Physiology and Pathology of
In 2002, at 21-50 dB, the workers examined at 500 Hz are Hearing, Warsaw/ Kajetany, Poland
statistically significantly less than those at 1000 Hz and
4000 Hz (p<0,001) and those at 2000 Hz (p<0,05). At 61-70 Otosclerosis (OTSC) is one of the most common causes of
dB, the workers examined at 500 Hz are statistically signif- adult-onset hearing loss in the Caucasian population. De-
icantly less than those at 2000 Hz and 4000 Hz (p<0,001) velopment of OTSC is associated with abnormal bone re-
while at 51-60 dB, the workers examined at 500 Hz and modeling in the region of otic capsule and stapes footplate.
at 1000 Hz are statistically significantly less than those at Etiology of OTSC is complex and there are a number of
2000 Hz (p<0,001). In 2009, at 21-50 dB, the workers ex- genetic variants reported to be associated with OTSC sus-
amined at 500 Hz are statistically significantly less than ceptibility. However, their frequencies have not been ana-
those at 4000 Hz (p<0,001) while at 61-70 dB, the workers lyzed in the Polish population. The purpose of our study
examined at 500 Hz are statistically significantly less than was to investigate the genetic variants most strongly associ-
those at 4000 Hz (p<0,01) and at 2000 Hz (p<0,001). Our ated with OTSC in Polish patients. DNA was isolated from
recent examinations reveal otosclerosis in seven of these blood samples and selected genetic variants in TGFB1 and
25 patients. Acoustic neuritis is diagnosed in 18 patients RELN genes were genotyped in clinically diagnosed OTSC
and 22 workers complain of tinnitus. All the patients ex- patients and control group using Custom TaqMan SNP Gen-
perience vestibular disorders during certain periods of otyping Assays and a real time PCR system. Allele and gen-
time. Surgery is carried out in five otosclerosis patients. otype frequencies were compared between the groups by
Chi square test and odds ratio (OR) with 95% confidence
There is a substantial improvement of the objective and intervals (95% CI) were calculated to estimate risk. Here we
subjective status in four of them. In three patients, addi- will present frequencies of the investigated variants and the
tionally, ossicular prosthesis is implanted upon their re- strength of association between each of them and OTSC.
quest. It remains not clarified if otosclerosis has already Our data will verify the role of the selected genetic variants
occurred long ago, or not. Further research is needed to in OTSC susceptibility in Polish patients.
elucidate the relationship between labyrinth-related oto-
sclerosis, on the one hand, and long-lasting exposure to Genetic factors in the development of
noise and vibrations, on the other hand. otosclerosis.
The conclusion is drawn that prolonged and regular oto- Ołdak M., Oziębło D., Pollak A., Lechowicz U.,
logical screening could timely identify initial hearing dis- Adamiok A., Skarżyński H.
orders among the workers in unfavourable occupational
environment. Department of Genetics, Institute of Physiology and Pathology of
Hearing, Warsaw/Kajetany, Poland
An attempt to dissect the genetic
background of familial otosclerosis. Regardless of intensive research on the pathogenesis of
otosclerosis, its background still remains poorly under-
Pollak A., Skarżyński H., Ołdak M. stood. The aim of the presentation is to give a comprehen-
sive overview of the current state of knowledge in the field
World Hearing Center, Institute of Physiology and Pathology of of genetics of otosclerosis. The intriguing correlation be-
Hearing, Warsaw/ Kajetany, Poland tween geographic descent and prevalence of otosclerosis
has been noted, indicating that this disease is significant-
The pathophysiology of otosclerosis is complex and con- ly more frequent among Caucasians than Japanese and
tains both genetic and environmental factors. Genetic South Americans. Otosclerosis is extremely rare among
analysis conducted in patients with familial form of oto- Afro-Americans, American Indians and oriental popu-
sclerosis with high probability may bring new insight into lations. Taking into account interpopulation differences
60 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
in otosclerosis prevalence together with a positive fam- having an analogous clinical presentation to otosclerosis.
ily history of this diseased in almost 60% of patients, it However, diminished mobility of the stapes in such cases
is highly expected that genetic factors play a significant is induced by excessive pressure of the perilymph. Genet-
role in the pathogenesis of otosclerosis. Familial stud- ic and environmental factors might be involved in both
ies revealed several genetic loci linked with otosclerosis conditions, therefore the genetic analysis might be a help-
but up to now only one potentially causative gene (SER- ful tool in the differential diagnosis.
PINF1) has been identified. The etiology of otosclero-
sis seems complex involving both genetic and environ- Material and Methods: An eight-year old female patient
mental components. with mixed type hearing loss in both ears and repeated ep-
isodes of sudden deafness was consulted at the Institute of
Otosclerosis or otosclerosis-like stapes Physiology and Pathology of Hearing. Pure-tone audiome-
fixation in patient with mixed hearing loss try, magnetic resonance imaging and a temporal bone CT-
and COCH pathogenic variant? scan were performed. Additionally, whole exome sequencing
(WES) was conducted to study the molecular background
Oziębło D.1, Adamiok A.1, Tacikowska G.1, of hearing loss.
Furmanek M.1, Skarżyński H.1, Ołdak M.1,
Skarżyński P.H.1,2,3 Results: Radiological examinations revealed inner ear
malformation corresponding to incomplete partition
1
orld Hearing Center, Institute of Physiology and Pathology of
W type 2. Results of genetic testing showed two heterozy-
Hearing, Warsaw/Kajetany, Poland gous pathogenic variants p.Glu29Gln and p.Leu117Phe
2
Heart Failure and Cardiac Rehabilitation Department, Medical in the SLC26A4 gene. Conclusions: Clinical picture of
University of Warsaw, Warsaw, Poland LVAS may cause diagnostic and therapeutic difficulties.
3
Institute of Sensory Organs, Kajetany, Poland In children with mixed hearing loss and repeated sudden
worsening of hearing, we strongly recommend to look for
The COCH gene encoding cochlin is highly expressed in pathogenic variants in the SLC26A4 gene and complete
the inner ear but the exact physiological function of the pro- the diagnosis by radiological examinations.
tein still remains unknown. Pathogenic variants in COCH
cause autosomal dominant hearing loss with possible ves- This study was supported by National Science Centre
tibular involvement. Interestingly, pathogenic variants in grants: 2011/03/D/NZ5/05592 and 2012/05/N/NZ5/02629.
COCH gene have been also identified with superior semi-
circular canal dehiscence (SSCD) which introduces a third Stapes fixation in female carriers of
window into the inner ear and may mimic otosclerotic hear- POU3F4 pathogenic variants mimicking
ing loss. Using clinical exome sequencing we have identi- otosclerosis.
fied a COCH pathogenic variant p.Ile374Thr causative for
hearing loss in a patient diagnosed with bilateral sensorineu- Pollak A., Lechowicz U., Tacikowska G.,
ral hearing loss. As the patient also suffered from unilateral Niedziałek I., Furmanek M., Skarżyński H.,
otosclerosis we have verified whether the conductive hear- Ołdak M.
ing loss does not result from SSCD. Based on audiological
and vestibular examination and reanalysis of the temporal World Hearing Center, Institute of Physiology and Pathology of
bone imaging we have excluded SSCD as a possible cause Hearing, Warsaw/ Kajetany, Poland
of conductive hearing loss in a patient. Our results do not
support the role of COCH p.Ile374Thr in SSCD develop- Otosclerosis is defined as an abnormal process of bone re-
ment but considering different conditions that may mim- sorption of the labyrinthine capsule followed by deposition
ic otosclerosis-like stapes fixation genetic testing is strong- of novel, immature sclerotic bone localized around the oval
ly recommended in diagnosing patients with hearing loss. window. As a result of this process the stapes footplate fix-
ation occurs causing conductive hearing loss. During the
Stapes fixation in enlarged vestibular time the sclerotic bone has a tendency to increase in size
aqueduct due to SLC26A4 pathogenic and depth. In most cases patients suffering from otoscle-
variants. rosis undergo surgery consisting of replacing all or a part
of the affected stapes with a prosthetic device which re-
Lechowicz U., Pollak A., Tacikowska G., stores hearing abilities. The clinical diagnosis of otoscle-
Skarżyński H., Ołdak M. rosis is typically based on the (i) analysis of pure tone au-
diometry, in particular the presence of air-bone gap, (ii)
World Hearing Center, Institute of Physiology and Pathology of no evidence of middle ear inflammation and (iii) regular
Hearing, Warsaw/ Kajetany, Poland picture of tympanic membrane. However, there are some
rare cases when this common clinical picture is not linked
Aim: Large vestibular aqueduct (LVAS) is diagnosed when with otoslerosis. Here, we present phenotypic features of
diameter of the vestibular canal is wider than 1.5 mm at female carriers of POU3F4 pathogenic variants, in whom
midpoint between the vestibule and its opening in the pos- based on molecular tests and radiological findings mixed
terior fossa. LVAS is supposed to be the most prevalent type of hearing loss with inner ear malformations was es-
reason of hearing loss in patients with congenital malfor- tablished in opposite to the suspicion of otoslerosis.
mations of the temporal bone. Air-bone gap in patients
with LVAS can be mistakenly taken as a disorder of aer- This study was supported by National Science Centre grant
ation of the inner ear or stiffening of the ossicular chain, 2011/03/D/NZ5/05592.
The pre – and postoperative assessment of Evaluating of the prevalence and severity of
tinnitus distress severity among patients tinnitus in otosclerosis patients.
qualified for stapedotomy – preliminary
findings. Skarżyński P.H.1,2,3, Dziendziel B.1, Świerniak W.1,
Bienkowska K.1, Gos E.1, Kutyba J.1,
Skarżyński P.H.1,2,3, Świerniak W.1, Dziendziel B.1, Skarżyński H.1
Bienkowska K.1, Gos E.1, Rajchel J. J.1,
Skarżyński H.1 1
orld Hearing Center, Institute of Physiology and Pathology of
W
Hearing, Warsaw/Kajetany, Poland
1
orld Hearing Center, Institute of Physiology and Pathology of
W 2
Heart Failure and Cardiac Rehabilitation Department, Medical
Hearing, Warsaw/Kajetany, Poland University of Warsaw, Warsaw, Poland
2
Heart Failure and Cardiac Rehabilitation Department, Medical 3
Institute of Sensory Organs, Kajetany, Poland
University of Warsaw, Warsaw, Poland
3
Institute of Sensory Organs, Kajetany, Poland Aim: Subjective tinnitus is, beside the progressive hear-
ing loss, one of the primary symptoms of the development
Aim: Stapes surgery is widely used in the treatment of of otosclerosis. The aim of the study was to evaluate the
otosclerosis in order to improve hearing. Furthermore, prevalence and severity of preoperative tinnitus among a
tinnitus is a frequent symptom related to hearing impair- group of consecutive adult patients with otosclerosis us-
ment. The aim of the study was to the pre– and postop- ing standardized research tools.
erative assessment of severity tinnitus distress among pa-
tients qualified for stapedotomy. Material: The prospective study comprised 462 patients
with clinically confirmed otosclerosis (326 women, 136
Material: The prospective study included 161 patients men), whose age was on average M=48; SD=11.5 years.
with otosclerosis. The sample comprised 123 females and All of them were qualified to stapedotomy in the World
38 males, with age on average M=49; SD=11.5 years. All Hearing Center, Institute of Physiology and Pathology of
patients underwent stapedotomy in the World Hearing Hearing in Poland between April – October 2017. The
Center, Institute of Physiology and Pathology of Hear- main inclusion criteria were: age ≥ 18 years, suspicion of
ing in Poland. otosclerosis and no previous stapes surgery in the ear eli-
gible for surgery. We excluded patients based on intraop-
Methods: All patients were asked to complete the Tinni- erative image excluding otosclerosis or with another sur-
tus and Hearing Survey questionnaire (THS-POL) to dif- gical procedure than stapedotomy.
ferentiate the problems with hearing loss and tinnitus. The
second tool was Tinnitus Functional Index (TFI-Pl) used Methods: The Tinnitus and Hearing Survey questionnaire
for evaluation of changes in tinnitus severity after stape- (THS-POL) was used to assess differentiating the problems
dotomy. This tool measures the impact on 8 domains such with hearing loss and tinnitus. The Tinnitus Functional In-
as: the intrusiveness of tinnitus, the degree of control the dex (TFI-Pl) questionnaire was used to assess the preoper-
patient has over the disease, cognitive interference, sleep ative severity of tinnitus and impact on 8 domains such as:
disturbance, auditory issues, relaxation issues, quality of the intrusiveness of tinnitus, the degree of control the pa-
life, and emotional distress. tient has over the disease, cognitive interference, sleep dis-
turbance, auditory issues, relaxation issues, quality of life,
Results: Before stapedotomy, 108 patients (67.1%) expe- and emotional distress. In addition, during the preopera-
rienced unilateral chronic tinnitus in the ear qualified for tive interview information on the age, sex of participants,
surgery or bilaterally. Using guidelines created by Meikle the duration of tinnitus and hearing loss were collected.
et al. (2012) for group TFI scoring, before surgery the re-
sults of the TFI-Pl questionnaire amounted to an average Results: Among 462 patient diagnosed with otosclerosis,
of M=31.2; SD = 21.4 points, indicating a moderate prob- 298 (64.5%) reported chronic tinnitus. In 136 patients
lem with tinnitus. In early post-operative follow-up, the (45.6%) tinnitus occurred unilaterally, only in the ear qual-
TFI-Pl total scores decreased to 22.1 points (SD=22.8). ified for surgery, in the other patients – on both sides. The
results obtained in this group using a questionnaire THS –
Conclusion: Based on the results of the study, it was ob- POL show that for nearly 22% patient, tinnitus was a great-
served that more than half of patients with otosclerosis ex- er or similar problem as hearing loss. The results of the
perience tinnitus, mostly of moderate problem. It is stat- TFI-Pl questionnaire amounted to an average of M=32.1;
ed that after a stapedotomy, patients with otosclerosis can SD=21.4 points, indicating a moderate problem of tinni-
not only get better hearing but also experience reduced tus before surgery.
severity of tinnitus.
Conclusion: The number of women in the study was al-
most twice as high as that of men, which confirms the
higher prevalence of otosclerosis in this group of patients.
The results obtained by means of validated questionnaires
indicate that over half of patients qualified for surgical
treatment of otosclerosis experience tinnitus, mostly of
moderate severity.
Genetic background of osteogenesis be described and how we envision its use for otosclerosis
imperfecta in Polish patients. surgery. At last, innovative methods for surgery teaching
using modified artificial bones and a virtual simulator of
Adamiok A., Osińska K., Oziębło D., Skarżyński the otosclerosis procedure will be presented.
P.H., Ołdak M., Skarżyński H.
Keynote Lecture
World Hearing Center, Institute of Physiology and Pathology of
Hearing, Warsaw/ Kajetany, Poland
Same or one-day surgery for stapes surgery:
Osteogenesis imperfecta (OI) caused by dominant auto- clinico-economical issues.
somal mutations is a phenotypically and genetically het-
erogenous disease characterized by bone fragility, skeletal Barbara M., Covelli E., Bianchi A., Talamonti R.,
deformities and ossicles malformations. Clinical presen- Margani V., Monini S.
tation and family history are two main diagnostic param-
eters in OI, although genetic test should be used to con- ENT Clinic, NESMOS Department, Sapienza, Rome, Italy
firm the diagnosis. To date, only few genes were found to
be causative for OI. In 85% of individuals OI results from Aim: Otosclerosis is characterised by abnormal sponge-
mutations in genes encoding type I collagen (COL1A1, like bone growth in the middle ear causing progressive
COL1A2). Collagen is known to be the main component hearing loss. It mainly affects the ossicular chain and it
of the extracellular matrix of bone and skin. Since our can be treated surgically by removing (part of) the sta-
knowledge on OI genetic causes is still so obscure it is im- pes and replacing it with a prosthesis—stapedotomy and
portant to better understand the etiology of this disease. stapedectomy, respectively. Increasingly, stapes surgery is
Our aim is to identify the genetic background of hearing performed as a One day-case procedure. The main rea-
loss in OI patients. son of choice towards One day-case surgery is the result
of economic considerations. Nevertheless, it is also an ex-
In the study genomic DNA of clinically diagnosed OI pa- plicit patient request and leads to shorter waiting times for
tients was used to perform clinical exome by next-genera- surgery, reduced risk of infection and most of all it pos-
tion sequencing. Segregation analysis of detected variants itively influences the patient’s quality of life as a result of
in OI families was performed by Sanger sequencing. We rapid discharge and rehabilitation. According to the latest
will present genetic data confirming the diagnosis of OI guidelines of day surgery procedures issued by National
in the investigated families. We expect that in the future Healthcare System, stapedotomy needs a maximum of two
genetic testing for OI will be routinely used to identify OI days of hospitalisation. The primary objective of this study
patients and offer them appropriate treatment. is to evaluate effectiveness of one day-case stapes surgery
compared to in hospital stapes surgery in terms of days
of hospital stay, complications and costs.
Lecture Session I
Material and Methods: 327 patients (116 male and 211
Keynote Lecture female; range 20-75 years, mean age 48.3) who underwent
surgery for otosclerosis between 2008 and 2018 were ret-
rospectively studied. The inclusion criterion was a diag-
Otosclerosis: from manual to robot-assisted nosis of otosclerosis based on a clinical history of progres-
surgery. sive hearing loss, normal otoscopic findings, an audiogram
showing a mean conductive hearing loss greater than 20
Sterkers O., Bernardeschi D., Ferrary E., dB HL in the range of 0.5–4 kHz, the absence of cochleo-
Nguyen Y. stapedial reflexes and surgery findings.
1
S orbonne Université, Inserm, UMR-S 1159 “Minimally Invasive We have divided the patients into two separate groups. The
Robot-based Hearing Rehabilitation”, Paris, France first one includes 160 patients who underwent stapes sur-
2
AP-HP, GHU Pitié-Salpêtrière, Otolaryngology Department, gery between 2008 and January 2012 under ordinary ad-
Unit of Otology, Auditory Implants and Skull Base Surgery, mission. The second group is composed by 167 patients
Paris, France who underwent stapes surgery under One day surgery be-
tween 2012 and 2018. Of these two groups, we considered
Otosclerosis surgery is performed through a transcanal mean days of hospitalisation, complications as vertigo,
approach and requires long and thin instruments with nystagmus, nausea, vomiting, hearing loss and hospitali-
sub-millimetric precision and precise amplitude of mo- sation costs according to the Regional Healthcare Service.
tion. The functional outcomes and complications of oto-
sclerosis surgery are dependent on the experience of the Results: In the ordinary inpatient group mean day of stay
surgeon. Thus, any technological assistance that enhanc- was 6 days, while in the one day-case patient group it
es the surgeon’s dexterity and reduces the learning curve was 2,2. Most patients of both groups did not show any
could yield an even safer surgical procedure. One of the complications.
options is to use robotic assistance to achieve this goal.
This lecture will first address the management of difficult Conclusions: According to our opinion, One day Surgery
surgical cases. The RobOtol system, a tele-operated de- is the best choice for stapes surgery in terms of costs and
vice designed for middle and inner ear surgery will then improvement in quality of life. Indeed the impact of long
hospitalisation not influences the incidence of post-oper- cicumferentially using post aural soft tissue harvested
ative complications. using non touch technique. In the end i place chorda
in front of piston as additional support to the piston.
Keynote Lecture After repositioning the flap, I check hearing on table.
Keynote Lecture
Stapedotomy – How I Do It!
Vijayendra H. Nitibond - Systematic development of an
optimized prosthesis.
Vijaya ENT Care Centre, Bangalore, India
Huber A.
Stapes surgery is one of the most delicate surgery in ear
surgery. Utmost importance should be given to this sur- University Hospital Zurich, Department of Otorhinolaryngology,
gery as middle ear is sterile in these cases and sterility of Head and Neck Surgery, Zürich, Switzerland
middle ear should be maintained. Most of my cases, I op-
erate under local anaesthesia. Great advantage being, hear- The optimal prosthesis to use for otosclerosis surgery is
ing can be tested on table. If piston length is more, then still a matter of debate. It has been proposed that pros-
giddiness and nystagmus can also be checked. theses made of shape-memory alloy (SMA), produce
better functional results with less variability and re-
Using proper and quality instruments is key in stapes duced risk because heat activation rather than manual
surgery. One should always have good set of all instru- crimping of the prosthesis loop forms a tighter fixation.
ments. Via transcanal approach, I create tympanomeatal However, risks in terms of mucoperiosteum lesions and
flap. First secure tympanomeatal flap anteriorly using incus necrosis are still discussed controversially. There-
wet saline soaked gelfoam. Because as a beginner one fore, the necessary properties for optimized stapes pros-
will take more time to perform procedure. By the time thesis were identified based on theoretical considera-
procedure gets over, flap which has been raised in be- tions, clinical experiences as well as mechanical tests.
ginning will shrink because of dryness and then will be Then, a new generation of SMA stapes prosthesis was
difficult to approximate. To obtain exposure, I curette developed and tested in preclinical as well as clinical
posterosuperior canal wall. For adequate exposure and environments. A self-fixing coupling area with multi-
easy insertion of piston superiorly facial canal and pos- ple contact points and recess spaces for the mucoperi-
teriorly pyramidalis process should be visible. Chor- osteum appear optimal for stapes prosthesis. A spring
da tympani nerve if coming in way then should be de- characteristic of the prosthesis loop allows a defined
compressed and secured away from working area so contact pressure on the incus and adaption to the ge-
that it won’t come in way during the procedure. I al- ometry of different incus shapes. Optimizing the shape
ways first make controlled fenestra of 0.3mm in pos- allows good intraoperative handling capabilities. Clini-
terior half of the footplate. The advantage of making cal testing revealed no adverse reactions and functional
controlled fenestra is that it is very safe once contolled results were “non inferior” to conventional SMA pros-
fenestra is made. If while widening fenestra something theses. Therefore, the newly developed NiTiBOND sta-
goes wrong with footplate then I have another instru- pes prosthesis overcomes limitations of existing pros-
ment, 0.2mm Fisch pick with which i can easily ma- theses. Our results suggest safety and reliability.
nipulate footplate as it can be passed through 0.3mm
diameter fenestra. Fenestra should be always made in Keynote Lecture
posterior 1/3rd of the footplate in order to avoid injury
to saccule and utricle which are placed near to anterior
part of footplate as compared to posterior part. Then Total stapedectomy. Technique and long
I drill posterior crus using 0.5mm diamond burr, dis- term results.
articulate incudostapedial joint and cut stapedius ten-
don near pyramid. Then using 45 degree angles pick, Garcia-Ibañez E.
keeping it at the neck of stapes, stapes suprastrucutre is
fractured towards promontory. Fenestra gradually wid- Clinica Garcia-Ibañez, Instituto de Otología, Barcelona, Spain
ened to 0.6mm using first 0.4mm and then to 0.6mm
perforator. In my experience, I have seen that manu- The use of total stapedectomy has been always the surgi-
al perforators are always better as surgeon can feel the cal option in our Institute for otosclerosis treatment. We
pressure at the tip of his fingers and always have con- present the technique, together with the short term and
trol over the procedure. Then using measuring rod, I long term results. Also a small series of stapedotomies are
measure the distance between the long process of incus compared with the total stapedectomy results.
and fenestra. I always use Teflon piston of 0.4mm di-
ameter because sound conduction and ultimately hear- These results and the results obtained in revisions of a big
ing results will be better. I use lower magnification to number of stapedotomies demonstrate that in our hands
insert piston as both fenestra and long process of in- total stapedectomy is the technique of choice for us. How-
cus should be visible in one view only. Teflon piston is ever, we understand that for not experienced surgeons sta-
inserted into fenestra, anchored to long process of in- pedotomy is safer for avoid inner ear damage and post-
cus and crimped. Surrounding of fenestra I always seal op complications.
64 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
Conclusions: The above results indicate that postoper- No relationship was found between APHAB Averseness
ative quality of life is related to subjective hearing bene- subscale (p>0,05). Age and hearing loss duration were not
fits after stapedotomy. Obtained results showed a positive related to the GBI results (p>0.05).
change after of stapedotomy in aspect of social relation-
ship which can significantly affect the general assessment Conclusions: Stapes surgery conducted by an experienced
of quality of life. surgeon is a beneficial procedure leading to the increased
health-related quality of life. Lower tinnitus severity and
It needs to be highlighted that conception of general qual- improved hearing ability in different acoustic situations
ity of life can be related with other factors such as family seem to contribute to the increased health-related quality
life, social status and other disorders not directly associ- of life reported by adult patients with otosclerosis.
ation with hearing problems. It is important to use ques-
tionnaires which measure general quality of life and also Stapes surgery in patients with a small air-
dedicated for group of patients with hearing problems. It bone gap.
is important to assess areas which are directly associated
with hearing and changes after treatment of hearing for McClenaghan F., Lavy J.A.
example after stapes surgery.
The Royal National Throat, Nose and Ear Hospital, London, UK
Measuring the postoperative change in
quality of life and its significant correlates Aim: To determine hearing outcomes in patients undergo-
among patients with otosclerosis – the ing stapes surgery with a preoperative air-bone gap (ABG)
preliminary findings. <21.25dB.
Skarżyński P.H.1,2,3, Dziendziel B.1, Świerniak W.1, Method: Patients with a unilateral or bilateral preoper-
Bieńkowska K.1, Rajchel J. J.1, Gos E.1, ative ABG <21.25dB undergoing primary stapes surgery
Skarżyński H.1 were identified from a database of all stapes surgery per-
formed by one surgeon in a tertiary centre. 254 ears met
1
orld Hearing Center, Institute of Physiology and Pathology of
W the inclusion criteria.
Hearing, Warsaw/Kajetany, Poland
2
Heart Failure and Cardiac Rehabilitation Department, Medical Intervention: All patients underwent stapes surgery under
University of Warsaw, Warsaw, Poland local anaesthetic. Ossicular reconstruction was achieved
3
Institute of Sensory Organs, Kajetany, Poland using a Smart 360 nitinol fluoroplastic piston (Gyrus) and
complete posterior crurotomy performed using KTP laser.
Aim: Aside from measuring the audiometric results of Hearing was assessed immediately postoperatively with
the surgical interventions, there is a trend of assessing the clinical voice testing and with pure tone audiometry at 6
broader aspects of postoperative benefits, such as health- weeks postoperatively.
related quality of life. Patients’ perception of the postop-
erative outcomes can be influenced by many factors, in- Main Outcome Measures: The primary outcome measure
cluding the subjectively perceived hearing benefits or the was the degree of closure of the preoperative ABG. Sec-
coexistence and severity of tinnitus. The aim of the cur- ondary outcome measure improvement in bone conduc-
rent study was to evaluate the quality of life change after tion thresholds at 4kHz.
stapes surgery and its significant correlates in adult pa-
tients, using a self-report measures. Results: 248 ears (97.6%) demonstrated ABG closure to
less than 10dB. Bone conduction thresholds showed an
Material and Methods: The study group consisted of 110 increase in 114 (44.8%), no change 75 (29.1%) and de-
subjects, including 82 women and 28 men with the mean crease in 66 (25.9%).
age of M=47.68; SD=10.12. All patients signed an informed
consent form to participate in the study. The eligibility cri- Conclusions: There is a slight increase in the risk of sta-
teria were age ≥ 18 years, lack of contraindications to take pes mobilisation in ears with a small ABG when compared
part in the questionnaire study, primary stapes surgery for those with larger ABGs however this can be overcome by
otosclerosis and a minimum of 6 months postoperative ob- using a laser-assisted technique in combination with good
servation period. To assess the postoperative health-related surgical experience. The benefit in terms of hearing aid
change in quality of life, Glasgow Benefit Inventory (GBI) avoidance and the restoration of symmetrical hearing is
was used. Tinnitus complaint was assessed using Tinnitus both achievable and significant for the patient.
Functional Index (TFI) and hearing benefits in different
acoustic situations were measured with Abbreviated Pro- Intra-operative prognostic factors in stapes
file of Hearing Aid Benefit (APHAB). surgery.
Results: Statistically significant, moderate to strong neg- Quaranta N.1, Lucidi D.2, Sergi B.2
ative correlations were observed between the GBI results
and different domains evaluated by the TFI (r=-0.545- 1
Università di Bari, Italy
,0.652; p<0.001). Additionally, strong negative correla- 2
Università Cattolica Roma, Italy
tions were found between the APHAB Ease of Commu-
nication (r=-0.63; p<0.001), Background Noise (r=-0.68; Aim: To assess the results obtained in a large cohort of pa-
p<0.001) and Reverberation subscales (r=-0.58; p<0.001). tients undergoing to otosclerosis surgery and to determine
the impact of intra-operative variables on post-operative Conclusions: Our study confirms the strong relationship
hearing function and complications. between otosclerosis and sound localization disability and
sheds some light on the complexity of the relationship.
Material and Methods: We enrolled 384 patients affected
by otosclerosis who were subjected to stapes surgery be- Impact of stapes surgery on the Carhart
tween 2004 and 2013. Surgery was performed in all cases effect.
under local anaesthesia, using manual perforator and/or
microdrill. Teflon-piston prosthesis were used in all pa- Rotimi T., Nip L., Manjaly J.G., Nash R., Jain A.,
tients. Audiological data obtained preoperatively and at last Lavy J.A.
follow up examination (minimum 12 months) were com-
pared. Statistical analysis was performed using the multi- Royal National Throat, Nose and Ear Hospital, University
ple regression model. College London Hospitals, The London Ear Clinic, London, UK
Results: peripheral rim otosclerosis and diffuse otosclero- Otosclerosis is associated with elevated air conduction
sis were associated with better functional results compared thresholds and an air-bone gap. However, depressed bone
to the obliterative pattern (p<0.05). Mean post-operative conduction may also be observed in this condition. Often
ABG was significantly greater in the 0,4 mm, compared this may be attributed to the Carhart effect and has impli-
to 0,6 mm piston group at 0.5 kHz (p<0.001) and 1 kHz cations when assessing patient suitability for stapes surgery.
(p<0.02). Stratification according to the surgical technique
shows that in the partial stapedectomy group mean post- Aim: This study aims to examine the audiological impact
operative ABG does not significantly vary between 0,4 and of stapes surgery with particular examination of pre- and
0,6 mm piston groups (9.1 dB in both), while in the sta- post-operative bone conduction thresholds and their re-
pedotomy group a statistically significant difference was lation to air conduction thresholds.
found between 0,4 and 0,6 mm piston groups, in favour
of the latter (p<0.05). Materials and Methods: Retrospective study of 336 oto-
sclerotic patients managed by stapedotomy and SMART
Conclusions: our data might be helpful to predict the post- 360° prosthesis placement (either primary surgery or re-
operative outcome and to address patients’ expectations. vision). Audiometry was performed pre- and post-oper-
atively. Air conduction (AC) and bone conduction (BC)
Does otosclerosis surgery change the spatial thresholds were recorded at various frequencies (0.5kHz,
hearing? 1kHz, 2kHz, 3kHz and 4kHz). Patients were subdivided
by pre-op BC thresholds.
Przewoźny T. 1, Kuczkowski J.1, Sierszeń
W.1, Tretiakow D.1, Mollin E.1, Szmuda T.2, Results: AC thresholds improved by 22.98dB in patients
Markiet K.3 with pre-op BC <30dB average across all frequencies
(p<0.05). BC improved by 2.54dB in the same group. AC
1
epartment of Otolaryngology, Medical University of Gdańsk,
D improved by 26.04dB in patients with pre-op BC > 30dB.
Gdańsk, Poland BC improved by 4.41dB in this group. There was a marked
2
Department of Neurosurgery, Medical University of Gdańsk, decrease in the air bone gap (ABG) post-operatively at all
Gdańsk, Poland frequencies. Carhart’s notch remained visible post-opera-
3
II Department of Radiology, Medical University of Gdańsk, tively albeit there was improvement in the hearing loss as
Gdańsk, Poland audiological frequencies increased.
Aim: Otosclerosis seems to negatively influence the central Conclusion: Stapes surgery improves AC and BC thresh-
auditory system, causing spatial hearing deficits. olds in otosclerosis. In an era when surgeons are offer-
ing surgical intervention for smaller air-bone gaps than
Material and Methods: We tested several adult patients traditionally considered worthwhile, one must consider
with otosclerosis together with 50 age and sex-matched the possibility that the air conduction could be improved
healthy volunteers. Our patients were tested before sur- to beyond the pre-operative bone conduction threshold.
gery and 1 month after surgical treatment due to otosclero-
sis. We used pure tone audiometry (PTAud), stapedial re-
flex test (SRT) and the horizontal minimum audible angle
test (HMAAT) for 8 azimuths with binaural stimulation.
Effect of the stapedotomy on early post- Material: From over 235 stapes surgeries performed be-
operative bone conduction. tween April and June 2017 in the World Hearing Cent-
er, there was selected group of 66 patients with otosclero-
Przewoźny T.1, Kuczkowski J.1, Sierszeń sis who underwent first-time stapedotomy and fulfil the
W.1, Tretiakow D.1, Mollin E.1, Szmuda T.2, study inclusion criteria. Presented group consists of pa-
Markiet K.3 tients aged from 25 to 70 years. The number of women
predominated in the surveyed group - about 73%.
1
epartment of Otolaryngology, Medical University of Gdańsk,
D
Gdańsk, Poland Methods: All patients underwent pure-tone audiometry
2
Department of Neurosurgery, Medical University of Gdańsk, before and after stapedotomy. In addition, patients were
Gdańsk, Poland asked to complete the Abbreviated Profile of Hearing Aid
3
II Department of Radiology, Medical University of Gdańsk, Benefit (APHAB) questionnaire for the self-assessment of
Gdańsk, Poland communication ability.
Aim: To analyze changes in bone conduction (BC) thresh- Results: The average hearing thresholds (for frequency
olds before and after stapedotomy in patients with clini- from 0.5 to 4.0 kHz) for the air and bone conduction in
cal otosclerosis. the operated ear significantly increased after stapedoto-
my. There was also observed air-bone gap reduction by
Material and Methods: Retrospective case review of pure almost 16 dB after surgery. Statistically significant corre-
tone audiometry (PTA) in 116 patients who underwent lation between pure-tone audiometry results and particu-
a stapedotomy at a tertiary referral center between 2007 lar APHAB scales was perceived.
and 2017. The patients were grouped by age and degree
of hearing loss. We measured a mean preoperative and Conclusion: The results of pure-tone audiometry in pre-
postoperative hearing level for single frequencies 0.5, 1, sented group of patients indicate improvement of hearing
2, 3 and 4 kHz and groups of frequencies (0.5-1-2-3 kHz after stapedotomy, which confirms the success of surgery.
and 0.5-1-2-4 kHz). The research also indicates that auditory benefits based
on pure-tone audiometry results correspond with self-
Results: The mean preoperative hearing levels for sin- perceived improvement in APHAB questionnaire. Usage
gle frequencies was 22.1dBHL, 24.4dBHL, 32.8dBHL, of this questionnaire is very important - it is a great com-
30.3dBHL and 27.9dBHL and for group frequencies (0.5, plementary tool to audiometric tests, because it provides
1, 2 and 3, or 4 kHz), was 26.8dBHL and 27.5 dBHL. The valuable information how big problem is hearing loss for
mean gain in BC, defined on PTA (0.5, 1, 2 and 3, or 4 the patient.
kHz), was 3.4 dBHL and 2.6 dBHL after stapedotomy.
Assessment of tinnitus oppressiveness using
Conclusions: Reconstruction of the ossicular chain in pa- the TFI questionnaire after the implantation
tients did not produce significant changes in the average of the CODACS system – a case study.
values of bone conduction.
Fludra M., Ratuszniak A., Król B., Skarżyński
The assessment of the auditory benefits P.H., Skarżyński H.
after stapedotomy using audiometric tests
and self-report questionnaire. World Hearing Center, Institute of Physiology and Pathology of
Hearing, Warsaw/ Kajetany, Poland
Skarżyński P.H.1,2,3,4, Ratajczak A.1,
Dziendziel B.1, Świerniak W.1, Gos E.1, Aim: Assessment of TFI (Tinnitus Functional Index)
Bieńkowska K.1, Skarżyński H.1 changes in patients with severe to profound mixed hear-
ing loss before and after the implantation of the CODACS
1
orld Hearing Center, Institute of Physiology and Pathology of
W system – case studies.
Hearing, Warsaw/Kajetany, Poland
2
Heart Failure and Cardiac Rehabilitation Department, Medical Material: The analysis concerns four patients with di-
University of Warsaw, Warsaw, Poland agnosed otosclerosis, severe to profound mixed hearing
3
Institute of Sensory Organs, Kajetany, Poland loss and tinnitus. The patients after numerous surgeries
4
International Center of Hearing and Speech Medincus, of both ears have undergone the implantation of the CO-
Kajetany / Nadarzyn DACS implant in the Institute of Physiology and Pathol-
ogy of Hearing.
Aim: The evaluation of the success of stapes surgery in
otosclerosis is measured by audiologists on the basis of an Method: The TFI questionnaire was performed as part of
increase in air conduction threshold and a reduction in efficiency assessment of the applied solution. They were
the size of air-bone gap. Important question is, whether used to evaluate changes in oppressiveness of tinnitus. The
the results of audiometric test correspond to the change tests and questionnaires were performed before the im-
in hearing perceived by patients. The aim of the study is plantation and after the system had been activated.
to assess the correlation between auditory benefits based
on audiometric tests and perceived improvement of hear- Findings: The results of the questionnaire assessment in-
ing using self-report questionnaire in the group of patients dicate significant changes in the value of the TFI rate in
who underwent stapedotomy.
all the tested areas, confirming a reduction in the oppres- surgery from September to December 2011 at World Hear-
siveness of tinnitus. ing Center of the Institute of Physiology and Pathology of
Hearing in Warsaw. Patents were analyzed retrospectively.
Conclusions: The gathered preliminary findings indicate Medical histories, surgery protocols and hearing exami-
that using the CODACS system may efficiently affect low- nation outcomes were particularly reviewed.
ering oppressiveness of tinnitus.
Symptoms presented in our group were: progressive hear-
Clinically important change in tinnitus ing loss, also tinnitus (>70%) and 20% vertigo. Comparing
sensation after stapedotomy. pure tone audiometry were mentioned frequencies: 0,25,
0,5, 1,2,4 kHz. After surgery the mean bone and air con-
Gos E., Raj-Koziak D., Dziendziel B., Skarżyński duction decreased, and air bone gap also decreased. For
P.H., Skarżyński H. frequencies range 0,25-2 kHz data were statistically sig-
nificant (p<0,05). Mean gap reduction was from 22,7dB
World Hearing Center, Institute of Physiology and Pathology of for 0,5kHz to 13,1dB for 2kHz.
Hearing, Warsaw/ Kajetany, Poland
Summing up this study and our previous experiences,
Aim: Statistically significant difference in the outcome of stapedotomy is effective otosclerosis treatment method.
treatment does not necessarily imply clinical importance, Complications are rare, and hearing effect is satisfactory
i.e. whether the observed improvement is important or both for patients and surgeons. It’s crucial due to frequent
meaningful to the patient. The aim of the study is to de- young age and professional activity of patients.
termine a change in tinnitus which is clinically important
to patients with otosclerosis.
Do we need always a CT Scan in the Results: With AC stimulation, both Psv and Pst drop by
diagnosis of otosclerosis. 30 to 40 dB after the interruption. In BC stimulation, Psv
decreases after interruption by about 10 to 20 dB, but Pst
Fraysse B., Escude B., Lagleyre S., Mary M. is little affected. For fixation of the stapes, Pst changed less
than Psv. The differential pressure changed significantly
Service ORL, Hôpital Pierre-Paul Riquet, Toulouse, France between 1 and 3 kHz.
The author will discuss the interest of imaging for the fol- Conclusions: The results suggest that the inner-ear mech-
lowing reasons: anisms play a large role in bone-conduction stimulation
in the chinchilla at all frequencies. However, the data also
- To confirm a doubtful diagnosis. suggest the pathways that conduct vibration to the in-
ner ear via ossicular-motion make a significant contribu-
Of 209 CT-scans of operated ears, 84% were classified pos- tion to the response to BC stimulation in the 1 to 3 kHz
itive, 9% doubtful and 7% negative. The sensitivity of CT- range, such that interruption of these path leads to a 5
scan to otosclerosis was 95.1%. In the operated ears, the dB reduction in total stimulation in that frequency. This
mean preoperative and postoperative bone conduction
phenomenon is similar to the Carhart’s notch observed in experimental ones. For example, experiments show that
patients with otosclerosis. for intensity level equal to 90 dB we SF displacements
drop from 16.4 nm to 4.63 nm, when frequency increases
Keynote Lecture from 400 Hz to 2000Hz. SF displacements get from the
analytical rule do not depend on the sound frequency.
The rule gives the value 10.2 nm, which corresponds to
A simplified analytical model of sound the frequency 1000 Hz.
transmission in the human ear.
Keynote Lecture
Gambin W.
Warsaw University of Technology, Warsaw, Poland Revisiting the learning curve for stapes
surgery.
Aim: The subject of interest is behavior of the sound wave
running from the sound source, through the outer, middle Barbara M., Covelli E., Monini S.
and inner ear, up to the round window membrane. Our
goal is to find simple analytical rules for the level of sound ENT Clinic, NESMOS Department, Sapienza, Rome, Italy
intensity, amplitude and pressure of the going wave. It as-
sumed that the intensity level of sound, the sound wave Stapes surgery is a challenging procedure that needs a long
frequency, as well as, the dimensions of the ear and/or and intense specific activity by the otologists. Although res-
the parameters of ear prosthesis are given. Such the rules idency programs include this procedure within the core
might be used for an analyses of ear activity, before and of the course, very rarely a resident achieves to perform
after the implantation of prosthesis. Among others, these the entire procedure and is uniquely approaching single
rules show the effect of changes in the anatomy of the ear steps, usually the initial ones, such as the tympano-meatal
on the reception of sound. flap. So, the real learning curve is actually starting after
the residency and exclusively in those academic or hospi-
Materials and Methods: At every stage of the sound prop- tal Centres where this type of surgery is performed with
agation process, all looked rules yield from the energy con- great numbers. Considering the reported worldwide de-
servation law. These rules can be determined on the basis crease of otosclerotic patients, even in the major otologic
of the following assumptions. (1) The outer ear is wave- Centres the operated cases just cover the maintenance of
guide filled with air, in which propagates an elastic plane dexterity of the senior staff so that teaching the technique
wave at a given frequency and sound level. The wave- is left to temporal bone lab or occasional access to the op-
guide ends with a flexible tympanic membrane on which erative theatre. After this premise, it is worth adopting the
a partial reflection of the wave appears. (2) Three ossicles safest technique for allowing the beginner to get knowl-
of the middle ear transmit oscillations of tympanic mem- edge of the different steps of the procedure. In this regard,
brane to the inner ear. The whole is a vibrating mechani- the reversal step Stapedotomy assisted by Laser could be
cal system. This system converts the sound energy passed considered an appropriate procedure.
through the tympanic membrane on the work of the force
moving the stapes footplate. (3) The inner ear is taken as
Diagnostic Approaches
a rectilinear waveguide filled with liquid, in which prop-
agates an elastic plane wave forced by the vibration of the
stapes footplate. Here propagates this part of the sound en- Keynote Lecture
ergy which has been passed through the tympanic mem-
brane, transmitted by the mechanism of the ossicles and
absorbed by the fluid of the cochlea. Along this waveguide Imaging of patients with suspected
extends a basilar membrane assumed to be a set of inde- otosclerosis.
pendent springs. The other end of the waveguide is closed
by the elastic membrane of the round window, which com- Szymański M.
pletely absorbs the energy of the running wave. It is as-
sumed that the sum of the energy of the running wave, Department of Otolaryngology Head and Neck Surgery, Medical
the rippling basilar membrane and the deformed round University of Lublin, Poland
window membrane is equal to the energy of the vibrat-
ing stapes footplate. The main diagnosis of otosclerosis is based on audiolog-
ical tests. However there are several entities resembling
Results: For a set sound intensity level with a given fre- otosclerosis which may require different management or
quency, the rules for sound intensity, amplitude and pres- surgery carries higher risk of complication. The use of
sure of sound wave in outer and inner ear were obtained. high resolution CT and/or Cone Beam CT enables vis-
In addition, associated displacements of the stapes foot- ualisation and localisation of otosclerosis foci. Advanced
plate and round window membrane, were found. imaging studies can differentiate patients with otosclero-
sis from other causes of conductive hearing loss. The lec-
Conclusions: The proposed approach neglect all vis- ture presents typical picture of fenestral otosclerosis, the
cous effects, a bone conduction and the real interaction use of imaging in preoperative and postoperative assess-
of the basal membrane and the cochlea fluid. Howev- ment and differential imaging diagnosis in patients with
er, it gives some averaged results, which are close to the audiologicaly detected oosclerosis.
72 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
Comparison of high-resolution CT, pure niche and 3 cases of facial nerve canal overhanging, that
tone audiometry and surgical findings in identified in HRCT.
patients with otosclerosis.
Conclusion: HRCT is sensitive method for diagnose oto-
Kunelskaya N.L., Garov E.V., Zelikovich E.I., sclerotic foci prevalence; however, it remains vulnerable
Zagorskaya E.E., Kurilenkov G.V., Kiselyus V.E. for lesions less than 0.5 mm. HRCT is suitable for plan-
ning surgical treatment, allowing to predict difficulty and
Sverzhevsky Otorhinolaryngology Clinical Research Institute, complication risk.
Moscow, Russia
Wideband absorbance measures for
Introduction: Nowadays high-resolution CT (HRCT) is assessment of otosclerotic ears: A
widely used for planning stapes surgery. This method is preliminary study.
helpful for localization of otosclerotic foci, anatomic ab-
normalities and determines risks of complications. How- Kochanek K., Śliwa L., Skarżyński H.
ever, the correlation between CT-signs of prevalence oto-
sclerotic foci and audiological testing are various. World Hearing Center, Institute of Physiology and Pathology of
Hearing, Warsaw/Kajetany, Poland
Aim: to evaluate correlation between results of HRCT,
pure tone audiometry (PTA) and surgical findings in pa- Background: Wide Band Tympanometry (WBT) is a reli-
tients with otosclerosis. able methods for assessment of middle-ear disorders. Lit-
erature reports indicate usefulness of WBT for qualifica-
Materials and Methods: 691 stapedoplasties were per- tion to reconstructive surgery and assessment of surgery
formed between January 2010 and October 2017 in Sver- results, especially in otosclerosis.
zjevsky Otorhinolaryngology Healthcare Research Institute
(Moscow, Russia). 89 patients (102 ears) who underwent Material and Methods: Energy absorbance (EA) measure-
stapedoplasty by the same surgeon were selected for ret- ment was performed, along with other diagnostic tests, in
rospective study; 67 were females (75.3%) and 22 males a group of patients diagnosed for otosclerosis. In some of
(24.7%), the mean age was 44.6 years (from 25 to 71 years). them the tests were repeated in postoperative period and
All patients with intra-operatively confirmed otosclerosis the effects surgery were assessed.
has preoperative temporal bone HRCT (GE Discovery 750
HD, 0.625-mm-thick axial imaging with multiplanar re- Results: EA value shows significant changes in otoslerotic
construction, USA), performed by the same radiologist. ears compared to normal-hearing ones. A meaningful de-
Patients with any additional non-otosclerotic reason for crease in EA value is observed especially in mid-frequen-
hearing loss were excluded. PTA was performed by the cy region, 500-1500 Hz. In many cases, 226Hz tympano-
same clinician. grams in otoseritic ears are normal.
HRCT data: fenestral localization of otosclerotic foci were Conclusion: WBT is an effective method for detecting
identified in 68 ears (66,7%), fenestral and retrofenestral middle-ear disorders, like ossicular chain fixation, disar-
(mixed) – in 18 (17,7%) and retrofenestral – in 2 (1,9%). ticulation, etc. It proves much more sensitive than stand-
In 14 ears, there were no significant signs of otosclerosis. ard low-frequency tympanometry. It may also be used for
Unilateral lesions identified in 7 cases. Otosclerotic foci assessment of surgery results.
ranged from 0.5 to 10 mm, mean density of fenestral foci
was 1074,3 HU and retrofenestral - 970,8 HU. Oblitera- Audiological profile of patients with large
tion of oval window niche detected in 5 cases (4.9%), and vestibular aqueduct.
facial nerve canal overhanging – in 3 (2.9%). Evaluation of
PTA conducted at 0.5, 1, 2 and 4 kHz. Conductive hearing Raj-Koziak D. 1, Krasnodębska P.1, Gos E.1,
loss (CHL) was identified in 16 cases (15,6%) and mixed Skarżyński H.1, Skarżyński P.H.1,2,3
hearing loss (MHL) – in 86 (84,4%), 12 were unilateral.
Mean PTA bone-conduction levels were 28.4 dB (from 8.3 1
orld Hearing Center, Institute of Physiology and Pathology of
W
to 53.8 dB), mean preoperative air-bone gap (ABG) was Hearing, Warsaw/Kajetany, Poland
31.6 dB (range 19.8 to 55 dB). Intra-operative prevalence 2
Heart Failure and Cardiac Rehabilitation Department, Medical
of otosclerotic foci was: promontory - 77 cases (75,5%), University of Warsaw, Poland
footplate - 58 (56,9%), anterior part of oval window niche 3
Institute of Sensory Organs, Kajetany, Poland
and footplate - 33 (32,4%). Narrow oval window niche ≤0.3
mm identified in 5 cases (4.9%). Aim: Audiological characteristics and treatment of pa-
tients with large vestibular aqueduct (LVA).
Results: Sensivity of HRCT in diagnosis of otosclerosis
was 86.3%. Comparison of HRCT and PTA revealed ap- Material: Retrospective analysis of 15 patients (10 wom-
propriation of fenestral form and CHL in 10 cases (9.8%), en and 5 men), mean age 25,1 (SD 11,4) years with radi-
mixed form and MHL – in 19 (18.6%). Analysis of surgi- ologically confirmed large vestibular aqueduct (LVA) has
cal findings and prevalence of otosclerotic foci on HRCT been carried out. Bilateral LVA was diagnosed in 9 pa-
revealed appropriation in 28 cases (27.4%). During sur- tients, right ear localization was in 1 patients and left ear
gery were confirmed 5 cases of obliteration of oval window localization in 5 patients.
Method: All patients underwent audiological diagnos- I - isolated lesion involving the footplate and/or pericoch-
tic procedure including pure tone threshold specified in lear lesion without endosteum involvement (7,8%);
tonal audiometry, impedance audiometry, otoacoustics
emissions (DP-gram) and auditory brainstem respons- II – pericochlear lesion with endosteum involvement
es (ABR). Possible deterioration of hearing was mon- (50%);
itoring by tonal audiometry tests conducted at two-
year intervals. III – Complete obliteration of the round window and/
or pericochlear lesion with ossification of the basal turn
Results: LVA was confirmed by CT or MRI examina- (42,2%).
tion, secondary confirmed by CT. LVA was associated
with Pendred syndrome in three and hypothyroidism in Changes in word recognition scores (WRS) were com-
one patient. Mixed hearing loss as a main syndrome, ex- pared between the three groups after a minimum of 12
tended from mild to profound, was either stable or pro- months follow up. The mean post op WRS for groups
gressive. Associated tinnitus was reported by 5 patients. A,B and C were 49% ,63% and 77% respectively. 60%
Episodes of one sided or bilateral sudden deafness were of patients in the stapedotomy group had a postoper-
diagnosed in 53% of patients, mostly with no results af- ative WRS ≥ 50% requiring no further treatment, sub-
ter standard treatment. Imbalance disorders were noticed sequent cochlear implantation due to deterioration of
in 5 patients. Eight patients were supplied with hearing hearing. This is compared with 72% for group B (coch-
aids. Six patients with progressive severe and profound lear implant alone), and 95% for group C (both). The
hearing loss were qualified as candidates for cochlear preoperative audiometrical first was correlated with
implantation. the outcome of stapedotomy. However, the specific-
ity was very low.
Conclusions: LVAS should be taken into account with
differential diagnosis of otosclerosis. Cochlear implanta- Previous stapedotomy has not impact on cochlear im-
tion is a possible solution for LVAS patients with progres- plant outcomes. The rate of surgical difficulties during
sive hearing loss. cochlear Implantation (n=37) was 51%, these includ-
ed round window and/or basal turn extra drilling, scala
vestibuli insertion, incomplete insertion or perilymphat-
Lecture Session III ic gusher. These complications occurred in patients with
grade III lesions 79% of the time. Non acoustic stimula-
Keynote Lecture tion occurred in 29,5% of patients, and these two tend-
ed to occur more frequently in patients with grade III
lesions (70%).
Far advanced otosclerosis: Stapedotomy or
cochlear implantation. Conclusion: Although cochlear Implantation gives better
overall results in terms of speech discrimination, we be-
Fraysse B., Kabbara B. lieve that stapedotomy with a well fitted hearing aid should
be first attempted due to a relatively high success rate, its
CHU Purpan, Service ORL, Toulouse, Paris low cost and low complication rate.
Inclusion criteria: All patients within the cochlear im- Stapes surgery is one of the most successful surgical in-
plant range with a pure tone average below 85 dB (HL) terventions for the restoration of hearing with excellent
and a preoperative dissyllabic word recognition score be- results being achievable in over 90% of patients by ex-
low 50% at 60 dB (HL) with maximal hearing aid, along perienced surgeons. However at each step of the opera-
with evidence of otosclerosis on CT Scan. tion complications can occur. These include perforations
of the tympanic membrane, damage to the chorda tym-
Results: Overall, 58 patients fit the inclusion criteria and pani, mobilisation of the stapes, subluxation of the incus,
were divided into 3 groups: A- stapedotomy group (n=32) injury to the facial nerve and sensorineural hearing loss.
B- Cochlear implant as a first option (n=11) C- Cochlear The speaker has carried out around 2000 stapes opera-
Implant with previous stapedotomy (n=23). tions and at present does 150-200 primary and revision
procedures per year. This personal, practical experience
Otosclerotic lesions were classified radiologically based and surgical tips acquired to reduce the risk of complica-
on a new CT Scan grading system: tions will be presented.
74 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
conduction evaluation for 4000 Hz and 8000 Hz before qualities. While long-term results from larger studies are
and after surgery does not show noteworthy differences. needed, this case series demonstrates that the hearing out-
In the majority of the patients it was shown the closure of come is comparable to other prostheses.
air-bone gap in the postoperative period. Bone-conduction
hearing thresholds were stable in both group. Differential diagnosis of otosclerosis vs.
superior semicircular canal dehiscence
Conclusion: Our experience on a large series of patients syndrome.
confirms the absence of damages for the inner ear and op-
timal functional results that remained stable at the post- Hüttenbrink K.B.
operative follow up. Our data suggest that laser Revolixx
JR for stapedotomy is an effective and safe procedure for Department of ORL, University Clinic Cologne, Germany
the treatment of otosclerosis.
Since the first description of a dehiscence of the superi-
Keynote Lecture or semicircular canal (SSCD), debates have emerged on
its implication for a loss of conductive hearing. This idea
was based on animal experiments with an artificial open-
Experiences with laser-assisted stapes ing for the SSCD towards the air of the tympanic cavity.
surgery using the NiTiFLEX stapes This, however, is not a correct approach, as in humans the
prosthesis. canal is covered by dura and loaded by the mass of the
brain and cerebrospinal fluid. Due to physiologic princi-
Plontke S.K., Zirkler J., Gey A., Rahne T. ples, a third window anywhere in the labyrinth will have
only in a minuscule effect on sound transmission. This
Department of Otorhinolaryngology, Head and Neck Surgery; had been confirmed by the data of animal temporal bone
Martin Luther University Halle-Wittenberg, Halle, Germany experiments with an opening of the labyrinth towards the
air. A 10-15 dB decrease of sound transmission could be
Background: Coupling of the prosthesis to the long pro- recorded only in the low frequencies; above 1kHz, no ef-
cess of the incus is a critical step in stapes surgery. We here fect was measurable. Further temporal bone experiments
report our experiences with a titanium stapes prosthe- with covering the dehiscence with a piece of dura showed
sis with a superelastic nitinol clip (NiTiFLEX®), a further no change of sound transmission as compared to the pre-
development of the Soft CliP® stapes prosthesis (KURZ, operative intact canal.
Dusslingen, Germany). Instead of pure titanium, the CliP®
now consists of nitinol, a superelastic nickel-titanium al- Therefore, no significant change of sound transmission,
loy. This further reduces the pressure exerted on the long nor a presumably otosclerotic air bone gap with a trans-
process of the incus, aiming to improve coupling and min- mission loss higher than 20dB, perhaps with the negative
imize the risk of incus luxation. rinne can be expected in case of a defect of the superior
semicircular canal.
Methods: In a monocentric, retrospective cohort study, we
evaluated air-bone gap reduction (4PTA0.5-4kHz), under- Furthermore, it must be respected, that even modern high
standing of monosyllables at 65 and 80 dB SPL stimulation resolution CTs cannot exclude a very thin bony closure
levels, and adverse effects, such as changes in bone con- of a semicircular canal, even if radiologist cannot dem-
duction (4PTA0.5-4kHz). Stapes surgery with the NiTi- onstrate this bone. Many other symptoms, also related to
FLEX® titanium prosthesis with a superelastic nitinol clip SSCD, like Tullio and Hennebert vestibular symptoms,
was performed in 73 otosclerosis patients. The footplate are also not concurrent to a presumed fistula, as these as-
was perforated using a CO2 laser (scanning mode; 0.7-0.8 sumptions forget that the interposed ossicular joints, due
mm; 20-21W) in nearly all cases. The diameter of the ti- to their gliding, will eliminate any significant pressure
tan piston was 0.4 mm. Immersion at an angle of almost transfer from the external ear canal into the inner ear. A
90° thus results in a gap between the footplate perfora- bulging of the labyrinth through a bony defect into the
tion and the piston of 0.15-0.2 mm. Positioning onto the dura is unrealistic.
long incus process was performed with a 90°microhook
(0.6 mm). Prosthesis length varied from 4,25 to 4,75 mm. Arguments of unsuccessful stapes surgery with unresolved
conductive loss must be attributed to other factors, for ex-
Results: In all cases, the NiTiFLEX® stapes prosthesis was ample not mentioned surgical failure, bony obliteration
inserted without complications. A preliminary evaluation of the round window membrane, malleus fixation etc., to
of the audiological results showed no significant change name only a few. Furthermore, experts in the older gen-
in the mean air-bone gap (4PTA0.5-4 kHz) and an aver- eration of ear surgeons, like Schuknecht etc., had already
age improvement of the mean air-bone gap (4PTA0.5-4 attributed these unspecific symptoms, which are nowadays
kHz) comparable to results from other reports with dif- correlated to SSCD, to connective tissues strands between
ferent prostheses. A recent update of the audiological as- the footplate and the membranous labyrinth.
sessment is awaited. Handling of the prosthesis was rat-
ed as very good. Some dramatic improvements of uncharacteristic vertigo
symptoms and even hearing disorders after surgery for
Conclusion: This study suggests that the NiTiFLEX® stapes SSCD should also consider the beneficial effect of drill-
prosthesis is a successful further development of the Soft ing in the temporal bone, well known from “sham”-oper-
CliP® technique with very good intraoperative handling ations for Meniere`s disease.
76 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
Rapier J.J., Theodoraki G.E., Manjaly J.G., Royal National Throat, Nose and Ear Hospital, University
Nash R., Jain A., Lavy J.A. College London Hospitals, The London Ear Clinic, London, UK
Royal National Throat, Nose and Ear Hospital, University Objective: To evaluate the efficacy of stapes surgery in
College London Hospitals, The London Ear Clinic, London, UK patients with otosclerosis and profound hearing loss, who
meet audiometric criteria for cochlear implantation.
Otosclerosis is associated with elevated air conduction
thresholds and an air-bone gap. However, depressed bone Study design: Retrospective study and questionnaire.
conduction may also be observed in this condition. This
may imply a cochlear component to the hearing loss but Setting: Tertiary care hospital. Patients: Thirty-five adult
may also be attributed to the Carhart effect and has im- patients (thirty-seven ears) with profound hearing loss.
plications when assessing patient suitability for stapes sur- Ages ranged from forty to eighty-five, with a mean age
gery. Patients with bone conduction thresholds of <30dB of 63.4. Intervention: Stapedotomy with prosthetic
are not traditionally considered good candidates for sta- implantation.
pes surgery, if the aim of surgery is to avoid the need for
hearing aid use. Main outcome measures: Benefit from stapes surgery in
this patient population was measured audiometrically,
Aim: This study aims to determine if there is benefit in using pure tone threshold averages (PTA) from 0.5kHz,
performing stapes surgery for otosclerosis in patients with 1kHz, 2kHZ and 4kHz, before and after surgery. In a sub-
better ear bone conduction (BC)<30dB. group of eight patients, the Glasgow Benefit Inventory
(GBI) tool was used to assess impact on quality of life.
Methods: Retrospective review of 111 patients with oto-
sclerosis and BC thresholds <30dB undergoing stapedot- Results: Thirty-one of thirty-seven ears demonstrated im-
omy and SMART 360° prosthesis placement by the same provement on PTA. The mean PTA from thirty-six ears
surgeon. Assessment was made of pre-and post-operative was an improvement of 32.3dB (one patient was exclud-
audiometric data, pre- and post-operative hearing aid us- ed because their thresholds were not recordable, and thus
age and change in patient quality of life using the Glasgow could not be included in numerical analysis). Of those that
Benefit Inventory (GBI). did not benefit, there was no PTA change in four patients,
while two patients experienced elevated PTA. Only two pa-
Results: 94 patients had complete audiometric data and tients in this cohort went on to have cochlear implanta-
were included. Average postoperative air conduction im- tion. The mean GBI score was +19.8. Seventy-five percent
proved from 63.7dB to 36.9dB. Average air-bone gap was of patients demonstrated functional improvement on GBI.
closed from 29.5dB to 7.85dB. Bone conduction improved One patient experienced very significant improvement
from 34.1dB to 29.1dB. One patient experienced signifi- with a score of +77.8. Twenty-five percent demonstrated
cant increase in air conduction of 49dB. Importantly 35% a decrease in function, with GBI scores of -5.50 and -25.0.
of patients in the study achieved postoperative air con-
duction thresholds of <30dB, even though their preop- Conclusion: Patients with otosclerosis and profound hear-
erative BC threshold was >30dB. This would have been ing loss who would meet audiometric criteria for cochle-
an unexpected result, if the Carhart effect hadn’t taken ar implantation derive benefit from stapes surgery both
place and provides evidence that preoperative bone con- quantitively and qualitatively. In most circumstances, sta-
duction thresholds in otosclerosis patients are not an ac- pes surgery improves hearing thresholds to a level where
curate marker of cochlear function. Patients’ GBI scores hearing aids become beneficial to the patient, thereby
showed a significant increase in their quality of life post- avoiding the need to change quality and tonality of sound
operatively. There was also a significant decrease in daily and commit the patient to further rehabilitation through
hearing aid usage post-surgery, with a number of patients cochlear implantation.
going from wearing a hearing aid at all hours of the day
to never wearing one. Stapedoplasty as a method of hearing
loss treatment in patients with
Conclusions: This study results suggest that patients with tympanosclerosis.
otosclerosis and BC thresholds of >30dB can achieve sig-
nificant benefits from surgery, both in objective audiom- Kryukov A., Garov Y., Zelenkova V.,
etry data, as well as in subjective increase in quality of Zagorskaya E., Sudarev P.
life and decrease in hearing aid usage needs. When as-
sessing suitability for surgery, surgeons should consid- Sverzhevsky Otorhinolaryngology Clinical Research Institute,
er the fact that pre-operative bone conduction thresh- Moscow, Russia
olds may be a poor indicator of the true cochlear reserve
and therefore the potential for improvement in air con- Aim: To evaluate the efficacy of stapedoplasty as a meth-
duction thresholds. od of hearing loss treatment in patients with stapes
Materials and Methods: The first group included 25 pa- Sverzhevsky Otorhinolaryngology Clinical Research Institute,
tients with CPOM and TSC who underwent a two-stage Moscow, Russia
surgery (2nd stage – stapedoplasty). The second group in-
cluded 25 patients with CPOM and TSC who underwent Many surgeons face with active forms of otosclerosis dur-
a multistage surgery with a typical stapedoplasty as a final ing stapes surgery. Frequency of cochlear complications
step, but using a surgical CO2 laser. As a method of hear- increases and functional results of stapedoplasty decreas-
ing loss treatment in patients with TSC 50 stapedoplast- es in case of active otosclerosis.
ies were performed (27 vestibuloincudopexies, 19 vestib-
ulomyringopexies, 4 vestibulomaleolopexies). To evaluate Aim: To reduce complications caused by active otosclerosis.
efficacy of stapedoplasty techniques, tonal threshold au-
diometry (TTA) in the frequency range of 0.125 to 8 kHz Materials and Methods: 959 patients with otosclerosis
as a method of air conduction (AC) and bone conduction were examined in Sverzhevsky Otorhinolaryngology Re-
(BC) were performed before surgery, then – before dis- search Institute from 2009 to 2017 year. Diagnosis was
charge, and three more times – in 1, 6, and 12 months af- based on anamnesis, pure tone audiometry (PTA), tym-
ter surgery, also we calculate mean air-bone gap (ABG). panometry, measurement of ultrasound threshold and its
lateralization, high resolution multispiral computer tomog-
Results: Analysis data of ABG mean values (35.8±6.4 dB raphy with multi-planar reconstruction and densitometry
before surgery) in TSC1 group showed their decrease in (areas of otospongiosis with density less then +900 HU
1 month up to 27.1±7.3 dB; in 6 months – up to 18.3±8.3 was marked as “active”, normal density of bone labyrinth
dB, and in 12 months – up to 13.3±9.2 dB. Hence, in- is +2000-+2200 HU). All patients with active otosclero-
strumental stapedoplasty was effective during whole fol- sis receive special drug therapy (bisphosphonates, calci-
low-up period, but its best efficacy was determined in 12 um, natrium ftoride, vitamin D). Treatment was regularly
months after surgery. ABG values < 20 dB were reached controlled by blood tests (Calcium free and bound, vita-
in 71.4% patients; from 20 to 30 dB – in 85.7%, and ≤ 30 min D, urea, creatinine), PTA and CT. Duration of treat-
dB – in 100% patients. Analysis data of auditory threshold ment was selected invidually and was based on initial den-
by BC values (36.8±5.4 dB before surgery) showed their sity of otosclerosis foci (1 standard course – 3 months).
decrease in 1 month after surgery up to 44.1±5.5 dB; in
6 months – up to 17.0±8.0 dB, and in 12 months – up to Results: In 288 patients (30%) active otosclerosis was de-
28.2±2.1 dB determined by inner ear reaction on surgery termined (28 - cochlear type, 221 – mixed type, 39 – tym-
treatment. Analysis data of ABG mean values (35.3±8.6 dB panic type). Tinnitus was determined in 64 patients. In
before surgery) in TSC2 group showed their decrease in case of unilateral hearing impairtment by PTA data, signs
1 month up to 22.1±9.5 dB; in 6 months – up to 17.0±8.0 of bilateral process were determined in 73,3% by CT data.
dB, and in 12 months – up to 14.9±7.4 dB. Stapedoplasty Treatment was considered successful when the density of
with laser assistance was effective during whole follow-up otosclerotic foci reached 1000 HU or higher. During the
period, but its best results were shown in 6-12 months af- treatment air conduction (AC), bone conduction (BC) and
ter surgery. ABG values <20 dB were reached in 76% pa- air-bone gap (ABG) was regularly measured. In most cas-
tients; from 20 to 30 dB – in 92%, and ≤ 30 dB – in 100% es this data was stable (5 dB). In 6 cases were matched im-
patients. Analysis data of auditory threshold by BC val- provement of BC and AC for 5-15 dB with the same ABG;
ues (30.3±11.3 dB before surgery) showed their consisten- in 14 cases – decreasing of AC with growth of ABG (BC
cy in 1 month after surgery – 31.1±13.2 dB; in 6 months – were stable). 34% of patients reported reduction of tinni-
28.9±13.1 dB, and in 12 months – 28.3 ± 12.8 dB. tus. Stapedoplasty were performed in 166 cases, after sev-
eral courses of treatment, when the density of otosclerot-
Conclusion: Stapedoplasty is effective with ABG decrease ic foci reached 1000 HU or higher and ABG was 30 dB
<20 dB in 73.7% TSC patients. Laser assistance during sta- or more. Functional results were good in all cases. Also,
pes crus crossing stage and stapedotomy in the early fol- there were no signs of active otosclerosis during all 166
low-up period results in absence of inner ear reaction for surgical procedures.
surgery; moreover, ABG mean values decrease more rap-
idly than these during instrumental surgery technique. Conclusion: Special examination (including PTA, tympa-
nometry, CT scan with densitometry) allows us to diag-
nose active forms of otosclerosis. Also, it helps us to form
plan the drug treatment, its duration and and evaluate its
effectiveness. Complex inactivating therapy stabilizes au-
ditory function and creates condition for safe and effec-
tive stapedoplasty.
Objectives: As an otologist the aim is to solve the conduc- Results: The absence of anterior limb of stapes, fixation
tive type of hearing loss in tympanosclerosis by surgery. of stapes were noticed in 7 patients; the absence of long
limb of incus, stapes superstructure, fixation of its foot-
Methods: With my vast experience of two decades after plate were noticed in 1 patient; the absence of stapes head,
performing more than 250 cases of tympanosclerosis de- the stiffness of stapes were recorded in 1 patient; the fu-
pending upon the surgical classification each case is tack- sion of anterior and posterior limbs of stapes, the stiff-
led according to the pathology. At least in 85% of the cas- ness of stapes were recorded in 5 patients; the absence
es, the footplate is made mobile ( with documentation) of stapes and OW was recorded in 5 patients; the ab-
and good hearing results are achieved. sence of stapes, OW and long limb of incus was record-
ed in 1 patient; the absence of stapes and OW, long limb
Results: With my documented special surgical tech- of incus and promontory mattered together were record-
niques in 85% of the patients servicable hearing results ed in 1 patient. An anomaly of the location of the facial
are achieved. nerve canal, which consisted of its bifurcation, overhang-
ing the OW or passing in the middle of the promonto-
Conclusion: As per surgical classification i.e Myringo- ry was found in 7 patients. The ABG in patients under-
sclerosis, attic tympanosclersosis, Stapes tympanosclero- going stapedoplasty decreased to an average of 15.25 dB
sis ( Mild, Moderate, Massive), Mastoid tympanosclero- and remained at this level throughout the entire obser-
sis, Tympanosclerosis associated with bony ankylosis and vation period. The formation of the fenestra nov-ovalis
multiple combination of the above classification I have my allowed to reduce the ABG to an average of 21.50 dB in
own documented surgical techniques to make the ossicu- the early post-surgery period. However, the bone-air in-
lar chain mobile to achieve good hearing results, which is terval of one patient increased to 35 dB in the late post-
the goal of an Otologist. operative period, which may indicate the formed fenestra
nov-ovalis ossification.
Keynote Lecture
Conclusions: The patients with isolated middle ear anom-
alies are good candidates for hearing-enhancing surgery.
Congential isolated middle ear anomalies: Certain difficulties during surgery may occur in case of
surgical treatment tactics. abnormal path of the facial nerve canal, but with an ad-
equate choice of surgical tactics, it is possible to achieve
Diab Kh.M., Daykhes N.A., Korvyakov V.S., good functional results. However, the cases of OW apla-
Gulyamov Sh.B., Kondratchikov D.S., sia require modification of the applied surgical techniques.
Zagorskaya D.A.
Keynote Lecture
Clinical Centre of Otorhinolaryngology of FMBA, Moscow,
Russia
Revision stapes surgery.
Aim: To increase the efficiency of surgical treatment of
isolated middle ear malformations by developing and im- Lavy J.
plementing optimal surgical tactics depending on the fea-
tures of the middle ear structures. Royal National Throat, Nose and Ear Hospital, University
College London Hospitals, The London Ear Clinic
Material and Methods: 21 patients with isolated middle
ear malformations examined and operated at our clin- In the assessment for revision stapes surgery the history
ic from January 2015 to October 2017. Indications for is essential for predicting the potential for a good hearing
80 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
outcome. A wide variety of conditions can be encoun- operations. Visualization of the entire oval window niche
tered in the middle ear and the surgeon needs to be pre- and identification of the facial canal is very important
pared for every eventuality. Advances in material sciences during stapedotomy. The facial canal and nerve may
including piston design and non-ototoxic bone cements show a morphological variations and anomalies (abnor-
have boosted the success rate of this challenging surgery. mal course, dehiscence, prolapsed and bifurcations of
A very practical approach to revision stapes surgery will the nerve and other).
be presented with emphasis on how to achieve the best re-
sults with minimal complications. The purpose of this paper is to review an important an-
atomical variations and relations in the middle ear that
Keynote Lecture may occur on the human temporal bones. We present-
ed findings of specimens that demonstrated a morpho-
logical variations involved: the facial nerve canal, area of
Revision surgery in otosclerosis. the oval window niche, ossicular chain, and upper jugu-
lar bulb and point out of their importance in stapes sur-
Szymański M. gery. The studies were performed on the large collection
of the temporal bones (2000 temporal bone specimens)
Department of Otolaryngology Head and Neck Surgery, Medical in our laboratory for anatomical and surgical examina-
University of Lublin, Poland tions, for many years.
Stapedotomy is a highly successful and safe procedure, In conclusion, better surgical techniques are not always
however, in case of complications or bad hearing result sufficient to receive good functional outcome from stapes
patients require revision surgery. Management of differ- surgeries, and the surgeon should also be aware of possi-
ent clinical situations like erosion of the long process of ble variations that affect the result of the surgery.
incus, prosthesis dislocation, perilimphatic fistula is dis-
cussed. The use of KTP and CO2 laser in revision sur-
Implants in Treatment of Otosclerosis
gery is also described.
Academy of Medical Science of the Serbian Medical Society, World Hearing Center, Institute of Physiology and Pathology of
Belgrade, Serbia Hearing, Warsaw/ Kajetany, Poland
The goal of stapes surgery is to re-establish sound trans- Aim: The study aims at preliminary evaluation of the ef-
mission through an ossicular chain stiffened because of fectiveness and efficacy of the CODACS system in pa-
otosclerosis. Various techniques have been used to ac- tients with otosclerosis.
complish this goal, including stapes mobilization, frag-
mentation, small fenestration, and partial or total sta- Material: The material consists of 4 patients with diag-
pes footplate removal. Even at the beginning of stapes nosed otosclerosis and severe to profound mixed hearing
surgery, authors emphasized the significance of ana- loss after many surgeries, who were implanted unilater-
tomical factors. The success of stapedoplasty depends ally with CODACS system. Procedures were performed
on the extension of otosclerotic process, surgical tech- in the Institute of Physiology and Pathology of Hearing.
niques, experience of the surgeon and morphological
variations and relations in the operative filed. Stapes Method: The free-field audiometric tests (free-field audi-
surgery is one of the most standardized and success- ometry and speech audiometry) were performed as part of
ful procedures in otology. Many discussions and pub- efficiency assessment of the applied solution. They were
lications have dealt with successful results obtained by used to evaluate hearing improvement. The tests were per-
numerous surgeons. A precise knowledge of anatomi- formed before the implantation and in a few intervals af-
cal characteristics has great influence , because of sta- ter the system had been activated.
pes surgery already carries some risk of complications
(additional hearing loss, tinnitus, vertigo, facial nerve Results: The outcomes of audiometric evaluation in
palsy and other). free field indicates lowering of the hearing threshold
and enhancement of speech discrimination while using
Morphological variations and relations that are seen dur- the CODACS system. Analysis of the APHAB question-
ing the operation may predispose the occurrence of com- naire revealed subjective benefits in hearing and speech
plications. Special attention should be given to the ana- discrimination.
tomical variations related to the stapes , the oval window
and the facial nerve. The deeply located narrow oval Conclusions: The gathered preliminary findings indicate
window and various facial nerve anatomic variations that using the CODACS system may efficiently affect hear-
are the problem that are met commonly during primary ing improvement.
Power Stapedectomy with Vibrant of the VSB. Excellent vocal and speech outcomes. There
SoundBridge SP coupler in Mixed Hearing were no surgical or audiological complications until now.
Loss. The follow up time is 16 months.
Carvalho G.M.1 , Ferreira J.P.1 , Loureiro L.R.2, Conclusion: Stapedectomy & VSB is a good option for
Mota F.2, Alves M.2, Silva P.B.3, Bernardes C.S.3, surgical rehabilitation for treating MHL in Otosclerosis.
Sousa Vieira A.1
Cochlear Implantation in Adult Patients
1
ENT - Hospital Lusiadas Porto, Porto, Portugal with Far Advanced Otosclerosis - Systematic
2
Audiology - Hospital Lusiadas Porto, Porto, Portugal Review.
3
Speech Therapist - Hospital Lusiadas Porto, Porto, Portugal
Kowalczuk A., Ludwikowski M., Skarżyński H.,
The therapeutic options in otosclerosis are limited to med- Skarżyński P.H.
ications (acute disease/active symptoms) as well as the re-
habilitation of the hearing loss (HL). Rehabilitation can be World Hearing Center, Institute of Physiology and Pathology of
with hearing aids (HA), stapedectomy, middle ear devic- Hearing, Warsaw/ Kajetany, Poland
es, osseointegrated implants and even with cochlear im-
plants, depending on the HL degree. Conventional HA Introduction: Otosclerosis is a chronic pathological pro-
stimulates the hearing acoustically, with sound amplifi- cess of the labyrinthine capsule, which leads to the con-
cation, so there are feedback limitations, distortions, lim- ductive or mixed hearing loss. Typically it starts in the
ited auditory gain, eczema of the external auditory canal 4th or 5th decade of life, but pathology has a tendency to
and other disadvantages. HA advantages remain on aes- gradually worsen so it is possible that the first otosclero-
thetic aspects, it does not require any surgical approach, sis changes occur earlier. The pathomechanis of otoscle-
with less morbidity. rosis is multifactorial and incompletely understood, the
most frequently mentioned causes of the disease include
In MHL, the ideal is to combine technologies to achieve a genetic predisposition, viral infections and various types
complete improvement of hearing. Therefore, the option of disorders: immunological, hormonal, mineral and en-
of middle ear/osseointegrated implants is a great solution, zymatic activity. The treatment (stapedotomy) enables re-
already well established in the world medical literature as storing the normal functions of the ossicular chain and
one of the gold standard treatments for the correction of gives a chance of eliminating or reducing hearing loss. In
mixed/moderate hearing loss. Such devices achieve great patients with far advanced otoslerosis, when stapes sur-
auditory benefits in cases of MHL of mild/moderate de- gery failed, cochlear implantation should be considered.
gree, because of the power of a mechanical auditory stim-
ulation technology. This allows them to provide a great au- Aim: The aim of this study was to analyze of the bene-
ditory gain without sound distortion and with great sound fits from cochlear implantation in patients with far ad-
quality. Their disadvantages lie in not being as aesthetic vanced otosclerosis.
compared to HA and the need for the patient to under-
go surgery, which implies obvious risks. The therapeutic Material and Methods: This study was prepared as a re-
options in your health and health, as well as the hearing sult of literature review. The preliminary analysis covered
rehabilitation. The patient can be rehabilitated only with 120 works. After the selection of the articles, some of the
hearing aids, stapedectomy surgery, AMEI or OI and even works were removed, including the duplicates, non-Pol-
CI, one dependent on the degree of deafness and involve- ish and non-English works, articles about ossification of
ment of his hearing. It is worth mentioning that conven- the cochlea due to meningitis as well as cochlear implan-
tional hearing aids stimulate hearing in an acoustic way, tation in children. Ultimately, 40 pieces of literature were
with an amplification of the children, on the other with qualified.
limitations of feedback, distortions, difficulty in obtain-
ing adequate auditory gain, eczema of the external audito- Results: Cochlear implantation in patients with far ad-
ry canal, within other limitations. Its advantages are more vanced otoslerosis leads to statistically greater improve-
aesthetic and does not require any surgical surgery, which ment in speech recognition scores than stapedotomy. How-
greatly reduces the risks for the patient. The therapeutic ever it is worth mentioning that outcomes after surgery are
options in otosclerosis are limited to medications (when highly variable and speech recognition results were sim-
the disease is in its acute stage, with active symptoms) as ilar in patients with cochlear implants and group of suc-
well as the rehabilitation of its hearing. cessful stapetodomy.
Materials and Methods: To evaluate the surgical and clin- Conclusion: Stapes surgery is a simple and effective meth-
ical outcomes of the Treatment for MHL in Otosclerosis od of otosclerosis treatment and should be considered be-
with Stapedectomy & VSB. All patients receive a SP (short fore cochlear implantation. Cochlear implant seems to be
process) coupler of VSB/Medel and a stepedectomy (tef- a good option for patients with poor or none benefits af-
lon prostheses/0.4 mm). ter stapedotomy.
Basilar membrane excitation mechanism A tool which can complement the experimental knowl-
in bone conduction hearing pathway in ear edge are the numerical analysis. The aim of the work is
with otosclerosis of the oval window. to present two numerical models of the cochlea, one di-
mensional (1D) and three dimensional (3D), developed
Tudruj S., Kamieniecki K., Piechna J., in the time domain and to show the advantages of numer-
Borkowski P. ical modeling in understanding the cochlea’s operation.
Warsaw University of Technology, Warsaw, Poland Material and Methods: 1D and 3D approach the coch-
lea is modeled as uncoiled. In 1D model the velocity pro-
Aim: The aim of the work is to understand the mechanism file and the pressure are assumed to be uniform across the
of the basilar membrane (BM) excitation in the bone con- cross section of the cochlear scalas. The basilar membrane
duction hearing pathway in the ear with the otosclerosis (BM) is modeled as a set of resonators tuned to differ-
of the oval window. The investigation was preformed us- ent frequencies. In 3D model the velocity profile and the
ing a numerical simulation. pressure distribution at cross sections area are three and
two dimensional fields respectively. The BM is modeled
Material and Methods: The cochlea is modeled as un- as a solid, thin membrane, fixed at the boundaries. The
coiled. The model consists of an oval window (OW), a models were excited by applying a harmonic oscillations
round window (RW), a BM, a scala vestibule (SV), a sca- to the stapes footplate (SF) of the oval window (OW). A
la tympani (ST) and a helicotrema. Interaction between coupling between the fluid (perilymph) and the structural
structural and fluid parts (the perilymph) is taken into parts (BM, OW and round window) is taken into account.
account. The cochlear scalas are represented by a three
dimensional canals. The BM is modeled as a set of reso- Results: Results of the models are the pressure – time his-
nators tuned to different frequencies. The model is excit- tories in the cochlea scalas and the displacement – time
ed by applying a harmonic velocity to the rigid cochlea’s histories of the basilar membrane. As the unsteady pro-
walls in the direction along the basilar membrane (X) and cess is simulated in the time domain, it is possible to ob-
perpendicular to the BM (Z). Oscillating walls of cochlea serve a mechanism of the basilar membrane excitation by
transmit the boundary motion to fluid inside. The model a pressure waves traveling in the perylimph.
was calculated for 10kHz excitation frequency.
Conclusions: The presented models are able to mimic the
Results: Results of the models are the pressure – time his- physiological cochlea operation according to the literature
tories in the cochlea scalas and the displacement – time data [Bekesy 1960, Stenfelt 2003]. As the models, described
histories of the basilar membrane. Based on the analysis, the hearing physics in the time domain, they are helpful
it was shown that the vibration amplitude for the direction in better understanding the cochlea’s operation, especial-
perpendicular to the BM is much smaller than the ampli- ly in the first moments after applying the excitation. The
tude for the excitation direction along the BM. Referring three dimensional model can be used to assess the effec-
to the time domain solution it was shown that this behav- tiveness of the middle ear prostheses (piston or chamber
ior is related to the geometrical and mechanical properties prostheses) or investigate the cochlear macro – mechan-
of the cochlea with otosclerosis of the OW. ics in the bone conduction hearing path way.
Conclusions: In bone conduction hearing pathway the Bibliography: [Bekesy 1960] Von Békésy, Georg, and Er-
largest amplitude of the BM vibrations is for the excitation nest Glen Wever. Experiments in hearing. Vol. 8. New
in the direction along the BM. This fact is related due to York: McGraw-Hill, 1960.[Stenfelt 2003] Stenfelt, Stefan,
the geometrical and mechanical properties of the cochlea et al. Basilar membrane and osseous spiral lamina motion
in the otosclerosis state, especially due to a stiff OW. Un- in human cadavers with air and bone conduction stimuli.
derstanding a mechanism of such behavior can be helpful Hearing research 181.1 (2003): 131-143.
in design of hearing recovery devices in otosclerosis. It is
expected that the behavior of the cochlea in the normal Is the human cochlea and basilar membrane
state (both windows has ability to move) will be different. length a coincidence?
Time domain one and three dimensional Kamieniecki K., Piechna J., Tudruj S.,
models of the cochlea. Borkowski P.
Kamieniecki K., Piechna J., Tudruj S., Warsaw University of Technology, Warsaw, Poland
Borkowski P.
Aim: The inner ear, especially the cochlea, is one of the
Warsaw University of Technology, Warsaw, Poland most sophisticated human sense organs. It is able to pre-
cisely distinguish thousands of tones. The cochlea has a
Aim: To design good and efficient hearing recovery de- specific length which determines the length of the basilar
vices, for example middle ear prostheses, it is necessary to membrane. It is interesting whether the dimensions of the
properly understand the cochlear physiology. The experi- cochlea scalas and then the basilar membrane are the co-
ments are one of the ways to gain the knowledge but they incidence or not? The aim of the work is to answer why
are difficult to conduct due to difficult to access location the human’s cochlea canal length and basilar membrane
of the inner ear, small size of the inner ear and due to the length equals to approximately 31.9mm and how does this
very dynamic character of the phenomena in the inner ear. affects hearing. Additionally it was checked if the inner ear
size is related to the body size or to the hearing frequency finite element analysis was performed in ANSYS system.
range. The proper understanding of the influence of the The model with the excitation applied on the stapes was
cochlea’s geometry on the mechanics of hearing may be validated on the basis of the literature. Then a harmonic
helpful in better treatment of the conductive hearing loses. analysis for the frequency range from 0.4 to 10kHz was
performed to determine the amplitude-frequency char-
Material and Methods: To answer the above question, a acteristics used as the reference to assess the efect of the
one dimensional model of the cochlea was used. The mod- stimulator. In the bone conduction analysis the stimula-
el assumed that the scala vestibule and the scala tympa- tor was represented by a lumped mass. The two force am-
ni are a pipes of a constant cross section. A coupling be- plitudes were considered which caused the amplitudes of
tween the perylimph and the basilar membrane and the vibration close to those obtained for the cochlea stimulat-
pressure wave reflection in the helicotrema are taken into ed by the vibration of the stapes. The first force amplitude
account. The model was excited by a harmonic vibration for bone conduction concerned the round window vibra-
of the stapes footplate of the oval window. tion and the second was related to the basilar membrane.
Results: The results of the analysis showed that the hu- Results: For the bone conduction analysis the six direc-
man cochlea and basilar membrane lengths are not a co- tions of the harmonic force applied at the point located
incidence. The length equal to 31.9mm enables a specific on the labyrinth capsule over the lateral semicircular ca-
pattern of the pressure wave distribution along the coch- nal were considered, assuming that the directions were
lea. This leads to amplification of the basilar membrane available after the mastoidectomy. For the fixed stapes
vibrations at the high frequencies and to decreases vibra- (otosclerosis) there was a relationship between the direc-
tions at the low frequencies. tion of the force and cochlear response. The force ampli-
tudes were decreasing with the increase of frequency. The
Conclusions: The human cochlea and the basilar mem- next simulation of bone conduction was performed for
brane lengths are not the coincidence. The length of the stimulator placed in the center of the anterior semi-
31.9mm allows on amplification of the basilar membrane circular canal loop. The four depths from 0 to 8 mm were
vibrations at high frequencies (10kHz) and to decrease the considered assuming the fixed force direction. The force
vibrations at low frequencies (0.4kHz). This is consistent amplitudes that caused the same vibration amplitudes on
with the basilar membrane geometrical and mechanical the round window and basilar membrane as obtained for
properties. In the basal end, where the high frequencies are the cochlea stimulated by the stapes were approximately
sensed, the basilar membrane is narrow and thick, while one order lover than those obtained for the stimulator sit-
in the apex it is wide and thin. Thus at the base the mem- uated at the reference point located at the outer surface of
brane is stiff and the amplification is needed, while at the the squamous part. The force amplitudes decreased about
apex it is flexible, thus the amplification is not necessary. twice when the point of excitation was moved to the lab-
Additionally the length of the human basilar membrane yrinth capsule over the lateral semicircular canal. The in-
was compared with other mammalians. This also confirms crease of the implantation depth caused the increase of
that its length is not a coincidence but it is rather relat- the force amplitudes.
ed to the hearing frequency range not to the body size.
Conclusions: The results of FE analysis showed a signifi-
Bone conduction stimulator position and cant influence of the force direction and the position and
direction investigation using numerical depth of the implantation on the effectiveness of the bone
simulation. conduction stimulation.
Results: The presence of FMT changes middle-ear imped- displace a piston up to 0,5mm in the oval window. These
ance and influences energy absorbance in a specific fre- movements are transmitted from the pressure-induced dis-
quency range. Low-frequency and high-frequency EA re- placement of the tympanic membrane, which can reach
mains virtually unchanged. An influence of FMT fixation up to 1000 µm, and the lack of the attachment of the pis-
method on EA is also observed. ton in the annular ligament, unlike the normal stapes. This
movement can explain the risk of a short piston to be lifted
Conclusions: Middle-ear implant, of FMT type, influenc- out of the footplate perforation (for example after sneez-
es acoustic impedance and absorbance characteristic due ing). In case of an underpressure in the middle ear, or a
to the increase of mass of the vibrating ossicular system. pressure rise in the external ear canal, an impalement of
The observed effects conform qualitatively well to those a piston with excessive length into the membranous lab-
predicted with the middle-ear model. yrinth can occur. The inward movement of the piston can
cause vertigo, as can be experienced also to during stapes
surgery in local anesthesia.
Lecture Session V
In conclusion of the experiments, flying or diving can be
Keynote Lecture allowed for patients after stapesplasty, if the piston had
been placed in the posterior part of the oval window niche,
where the distance towards the saccule and utricule is more
Age and otosclerosis. than 1 mm. A further control can be obtained in surgery
under local anesthesia: If the pressing down of the piston
Tange R.A. in its attachment to the lenticular process towards the inner
ear and in its correct place does not provoke a nystagmus
Utrecht / Naarden, the Netherlands nor vertigo, no danger can be assumed in the postopera-
tive life due to the stability of the ligaments of the ossicles.
The onset of hearing problems due to otosclerosis is of-
ten difficult predict. In the 20’s the majority the disease Postoperatively, a test can confirm the lack of danger, if the
otosclerosis commenced between 21 and 25 with hardly patient wants to fly or dive after surgery: A tympanometry
any appreciable difference in the sexes. The beginning of with fast changes between 400 mm positive and negative
deafness started in 50% of the cases in the period between pressure, with continuous recording of the eye movements
the 16th and 30th year according to Nager(1927). Now, in ENG. If the recording is nystagmus free, and if the pa-
almost a century later the onset of the symptoms of oto- tient does not describe any vertigo, no postoperative risk
sclerosis seem to appear in a later fase of life. Recent stud- must be anticipated for flying and diving after stapesplasty.
ies have demonstrated that the disease otosclerosis com-
menced between 36 and 45 of age. The age onset of the Keynote Lecture
symptoms of otosclerosis has been increased during the
last 80 years. The reason for the age onset increaseof oto-
sclerosis is still unclear. Patient satisfaction after stapes surgery for
otosclerosis.
Keynote Lecture
Maniu A., Necula V.
Flying and diving after stapes surgery. Department of Otorhinolaryngology, Iuliu Hatieganu University
of Medicine and Pharmacy, Cluj-Napoca, Romania
Hüttenbrink K.-B.
Aim: Health-related quality of life after stapes surgery has
University Hospital of Cologne, Germany become a major concern nowadays as in other areas of
the otology field. Therefore, correct reporting of results
The ear works as a pressure receptor. A piston prosthe- requires more complex data both technically (depending
sis, which significantly modifies the function of the nor- on technique and surgeon) and functionally (depending
mal ossicular chain, will be displaced at changes of the on patient satisfaction). The objective of this study was
atmospheric air pressure quite differently as compared to to review the current methods of reporting results after
the normal stapes. These pressures may a reach significant stapes surgery, and to correlate the health related quali-
dimensions in daily life, as during swallowing, with tubal ty of life outcomes after surgery with technical audiomet-
opening, wind gusts at the external ear. Extreme pressures ric parameters.
can occur during flying and diving, a condition, which evo-
lution had not foreseen for the development of our ears. Materials and Methods: A prospective study on 230 pa-
tients who underwent stapes surgery for otosclerosis was
Fortunately, the middle ear is designed to compensate even conducted at Cluj-Napoca University Hospital between Jan
elevated and rapid changing static pressures. This could be 2015 and Jan 2016. The audiometric results, at 12 months
shown in temporal bone experiments with measurements after surgery, were analyzed according to the guidelines of
of stapes displacement during tympanometry. These pres- the Committee on Hearing and Equilibrium of the Academy
sures, which are the maximum driving force also during of Otolaryngology-Head and Neck Surgery (AAO-HNS),
physiological stresses, due to the stability of the collagen and Glasgow plot. Surgical trauma to the inner ear was also
fibers in the tympanic membrane at higher pressures, can evaluated using the Amsterdam plot. The subjective hearing
Vijayendra V.K. Materials and Methods: 165 patients (182 ears) with oto-
sclerosis, who underwent stapedoplasty, were included in
Vijaya ENT Care Centre, Bangalore, India this study. We used self-crimping superelastic NITINOL/
PTFE piston-prosthesis in 143 cases. Piston-type stapedo-
Stapes surgery is one of the most delicate surgery in ear sur- plasty was performed in cases of simple non-complicated
gery. As middle ear and tympanic membrane are absolute- forms of otosclerosis without incomplete dislocation of in-
ly normal, utmost importance should be given to this sur- cus, facial nerve overhang, obliteration of oval window and
gery. In cadaveric temporal bone, one should learn stapes cochlea. In cases when patient had certain anatomical fea-
surgery very meticulously as if it is being done in live pa- tures (dehiscence and overhand of facial nerve, oblitera-
tient. Using proper and quality instruments is key in learn- tion of oval window, obvious perilymph leakage), in cases
ing stapes surgery. We should have good set of all instru- of revision surgeries (aseptic necrosis of process) or in cas-
ments. In cadaver, reverse stapedotomy is done as most of es of unintentional mobilization of stapes foot we practiced
the time footplate will be normal and if we try to remove methodology created in the Russian Federation – that is to-
suprastructure before inserting piston, footplate will get tal platynoctomy with stapedoplasty using “auto-cartilage
dislodged and procedure won’t be possible. I create tym- on vein” placement (39 cases). Shaped oval window after
panomeatal flap via transcanal approach and secure it an- platynoctomy is covered with auto-vein sized 3*5 mm and
teriorly using wet saline soaked gelfoam. Because as a be- is pressed by auto-cartilage prosthesis, fixed under lentic-
ginner one will take more time to perform procedure. By ular incus process, that helps to provide total hermitization
the time procedure gets over, flap which has been raised in of inner ear. Pure tone audiograms analyzed to evaluate the
beginning will shrink because of dryness and then will be functional results.
difficult to approximate. To obtain exposure, I curette pos-
terosuperior canal wall. For adequate exposure and easy in- Results: The average air conduction was 31,5±5,6 dB be-
sertion of piston superiorly facial canal and posteriorly py- fore surgery and significantly decreased to 11,5±3.2 dB
ramidalis process should be visible. Chorda tympani nerve at 6 months postoperatively in patients who underwent
if coming in way then should be decompressed and secured stapedoplasty using a NITINOL/PTFE piston-prosthesis
away from working area so that it won’t come in way dur- (n=130). In 10 cases, the piston in Titanium was use due
ing the procedure. I always first make controlled fenestra to thick long incus process. Analysis of the functional re-
of 0.3mm in posterior half of the footplate and then grad- sults showed a statistically significant (p <0.05) decrease
ually it is widened to 0.6mm. Fenestra should be always air-bone gap to 10,2 ± 2.3 dB (before operation 34,2 dB)
made in posterior 1/3rd of the footplate in order to avoid in patients who underwent stapedoplasty using method-
injury to saccule and utricle which are placed near to ante- ology of “auto-cartilage-on-vein” placement. Displacement
rior part of footplate as compared to posterior part. Using of piston prostheses were not observed during research
measuring rod distance between the long process of incus study. In 4 cases (3 cases after piston type stapedoplasty
and fenestra is measured. Use lower magnification to insert and 1 case after stapedoplasty with cartilage) development
piston as both fenestra and long process of incus should be of sensorineural hearing loss was observed.
visible in one view only. Teflon piston of 0.4mm diameter
inserted into fenestra, anchored to long process of incus and Discussion and Conclusion: Usage of NITINOL/PTFE pis-
crimped. Bend test and Lift test is performed to ensure that ton-prosthesis decrease time of surgery and consequent-
Piston length is correct. In Bend test when shaft of piston ly impact on inner ear’s structures. The hook elasticity re-
is bent, piston should not come out of fenestra. In Lift test, duces the surgery steps and the difficulties that may occur
while lifting incus, piston should not come out of fenestra. during the closing phase. The hook closes softly, uniformly
86 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
wrapping the pressure along the total periphery of the long accurately the image of the sound wave from the external
incus process, and minimize the risk of compressive necro- ear to the inner ear. Commonly used piston prostheses
sis. Positive aspect of stapedoplasty using methodology “au- do not meet that requirement. Due to the small contact
to-cartilage on vein” placement is possibility to shape auto- surface of the piston with the perilymph, these prosthe-
cartilage prosthesis (creation of auto-cartilage with a certain ses transform the plane wave in the ear canal into a dis-
angle position) that helps to go round overhang of facial persed spherical wave in the cochlea.
nerve, saving the pressure on it. Observation and analysis
of surgeries showed high effectiveness of mentioned treat- Materials and Methods: In order to generate in the coch-
ment methods, that allows ENT surgeon to have certain al- lea an image of a sound wave the same as that enter-
gorithm of his activities in situation of any difficulty. ing to the ear canal, a device known in the optics as a
‘camera obscura’ was used in the chamber prosthesis.
Audiological results of the new Nitinol A diaphragm with a small opening, placed between an
Stapes Prosthesis. illuminated object and a screen, focuses light wave run-
ning from the object, creating an inverted image on the
Teschner M., Lilli G., Lenarz T. screen. This idea was applied to the sound wave. A de-
sign of the chamber prosthesis design, which focuses the
Hannover Medical School, Dept. of Otolaryngology, Hannover, sound wave at the entrance to the cochlea, has been pre-
Germany pared. It consists of five elements: a chamber (1) filled
with physiological fluid, a disc (2) suspended on a mem-
Aim: Stapes plasty is a well-established method for improv- brane (3), a pendant (4) connecting the disc with the
ing hearing in case of otosclerosis. Continuous optimiza- malleus and a protective cover (5). The basic conditions
tion leads to periodically new developed prosthesis. The to be met prosthesis, have been formulated. It was as-
aim of the current study was to evaluate the audiological sumed that the main elements of the prosthesis will be
results of Stapes plasty with new Nitinol stapes prostheses. made by 3D printing.
Material and Methods: The surgeries were performed be- Results: With help of the Autodesk Inventor program,
tween May and November 2017. Nitinol Prostheses were few proposals of the chamber prosthesis have been pre-
implanted in the patients. Preoperative and postoperative pared. The chamber, shield and cover of the prosthesis
pure tone audiograms of patients were evaluated. Postop- was planned be made by 3D printing from a biocompat-
erative measurements were taken 3 weeks after surgery. ible polymer. The pendant will be prepared from a plat-
Furthermore, data on the intraoperative surgical method inum wire. Two ways of connecting the pendent with
and possible complications were recorded. As a control the disc were proposed. Three designs of the membrane
group, 10 patients were selected, who were treated by the made of a thin Teflon film or a flexible silicon hydrogel
same surgeon with a conventional prosthesis. have been analyzed.
Results: To date, nine stapes plastic surgeries using the Ni- Conclusions: The proposed solution for the middle ear
tinol Alloy prosthesis were evaluated. The control group prosthesis has the following features:
consists of patients with conventional Stapes plasty. The
postoperative average air-bone gap in the study group was – the prosthesis provides a tight seal to prevent infection
13.5 ± 3.9dB (MV ± SD) while that of the control group after surgery;
was 13.9 ± 7dB (MV ± SD). Moreover, the average reduc-
tion in the air-bone gap was 18.5 ± 9.4 dB (MV ± SD) in – the proper size of the prosthesis enables a safe surgery, in
the study group and 12.7 ± 3.5 dB (MV ± SD) in the con- particular it allows to avoid a damage of the facial nerve;
trol group. Intraoperative and postoperative serious com-
plications were not observed in both groups. – the stiffness of the membrane is close to the stiffness of
the stapes ligament;
Conclusions: Stapes plasty using the new Nitinol pros-
thesis is a feasible procedure in otosclerosis surgery with – the contact surface of the disc with the cochlea fluid is
less conductive hearing loss. As this prosthesis needs not a spherical surface, to avoid an absorption of the sound
to be crimped intraoperative, it represents a practicable energy by the chamber walls;
alternative to conventional prosthesis.
– the plane of the chamber outlet opening is inclined to
Keynote Lecture the plane of the basilar plane to ensure energy transfer
deep into the cochlea interior; in a healthy ear, this trans-
fer ensure the stapes footplate rotations. The main obsta-
Principle of work of the chamber prosthesis cle in the realization of the project are small dimensions
and its realization. of the prosthesis elements. We have hope that a new 3D
printer built at our Institute and adapted to manufacture
Gambin W. the chamber prosthesis, will solve that problem.
Prospective clinical study on the influence Materials and Methods: 263 patients (296 ears) with
of stapes prosthesis diameter on hearing chronic suppurative otitis media, who underwent tympa-
outcomes. noplasty with ossiculoplasty, were included in this study.
We used partial titanium ossicular prosthesis in 170 cas-
Bernardeschi D., Sterkers O. es and total titanium ossicular prosthesis in 126 cases. We
used prosthesis with extensible (adjustable) stem (the stem
Otology, Auditory Implants and Skull Base Surgery Department, is extensible through the traction and shortable through
NF2 and Auditory Implants, Ile de France Referral Center, Pitié- the compression), that showed good sound wave con-
Salpetrière Hospital, Paris, France ductivity by oscillometric tests (unchanged conductivity
of the sound wave in all stem configurations). The effec-
Objectives/Hypothesis: To evaluate the influence of the tiveness of surgical interventions were assessed as short-
diameter of stapes prosthesis on functional outcomes in term (up to 3 months post-operatively) and long-term (6
stapes surgery. to 12 months) anatomical and functional outcomes. The
anatomical results considered satisfactory if there was a
Study design: Prospective cohort study. well-formed mobile neotympanic membrane, air tympanic
cavity and dry postoperative cavity. Pure tone audiograms
Methods: Fifty consecutive small fenestra stapedotomies analyzed to evaluate the functional results.
performed using a 0.4-mm-diameter prosthesis were com-
pared with 50 consecutive small fenestra stapedotomies Results: Satisfactory anatomical results obtained in 93.5%
carried out using a 0.6-mm-diameter piston. Audiolog- of patients with the safe type suppurative otitis, 88.9% of
ical assessment following the recommendations of the patients with the unsafe type and 91.2% of patients after a
Committee on Hearing and Equilibrium was performed 1 revision surgery. The average air conduction was 33,7±7,1
month after surgery. Postoperative complications between dB before surgery and significantly decreased to 17.5 ± 5.8
the two groups were noted. dB at 6 months postoperatively in patients who underwent
ossiculoplasty using a partial ossicular prosthesis with satis-
Results: There were no statistically significant differences factory anatomic results. Analysis of the functional results
in demographic data between the two groups, and no dif- showed a statistically significant (p<;0.05) decrease average
ferences in preoperative bone-conduction (BC) or air-con- air conduction to 35.5 ± 5.7 dB (before surgery 57.1 ± 5.3
duction (AC) hearing thresholds for all frequencies (analy- dB) and air-bone gap to 18.5 ± 2.3 dB (before surgery 36.5
sis of variance [ANOVA] and χ2 tests). No differences were dB) in 91.4% of cases in patients who underwent ossiculo-
found in the mean preoperative BC and AC pure-tone av- plasty with a total prosthesis with satisfactory anatomical
erage and air-bone gap (ABG). In the postoperative eval- results. Displacement of total ossicular prostheses was the
uation, a statistically significant difference was found for main cause of poor functional results (9 cases). The most
the mean AC gain (20 ± 8.7 vs. 24 ± 11.5, P = .042, ANO- common causes of unsatisfactory anatomical results were
VA) as well as for the postoperative AC threshold at 0.125 perforation (15 cases), cholesteatoma recurrence (3 cases)
and 0.25 kHz and the postoperative BC threshold at 0.25 and lateralization of the neotympanic membrane (3 cases).
kHz (P < .01, ANOVA). A postoperative ABG ≤10 dB was
obtained in 90% and 94% of patients in the 0.4-mm- and Discussion and Conclusion: Formation of a reliable sound
0.6-mm-diameter piston groups, respectively (difference conducting system with ossicular prostheses allows for
not significant, χ2 test). No postoperative dead ear and/ persistent improvement of hearing. Various surgical tech-
or sensorineural hearing loss was noted in either group. niques, such as extended posterior tympanotomy, endo-
scopic assistance, provide a good effect with the remov-
Conclusions: The 0.6-mm piston allowed a statistical- al of non-aggressive cholesteatoma while preserving the
ly significant higher AC gain compared with the 0.4-mm bone structures, which are not involved in the disease. A
diameter piston. A larger diameter piston may be prefer- complete removal of an advanced aggressive cholesteato-
able if there are no anatomical or technical reasons that ma with the opening of the temporal bone cell system en-
would favor a smaller prosthesis. sures good functional and anatomical outcomes, makes it
possible to prevent the spread of the pathological process
Keynote Lecture and development of intracranial complications.
Keynote Lecture
The efficiency of prosthesis with extensible
(adjustable) stem in ossicular chain
reconstruction: our experience. The nature of vibrations of disc suspended
on flexible rings in the chamber prosthesis.
Diab Kh.M., Kondratchikov D.S., Pashchinina O.A.,
Mikhalevich A.E., Medeulova A.R., Umarov P.U. Gambin W.
Clinical Centre of Otorhinolaryngology of FMBA, Moscow, Russia Warsaw University of Technology, Warsaw, Poland
Aim: To analyze the effectiveness of surgical treatment Aim: Due to small dimensions of the chamber prosthesis,
with ossiculoplasty of patients with chronic suppurative the basic difficulty is a way to hang the prosthesis disc. A
otitis media operated in a single clinical center from 2015 suspension of the disc on a membrane is the most pop-
to 2017. ular solution. Linear vibrations of the membrane enable
88 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
transfer of the sound wave from the outer ear to the in- or mixed hearing loss with or without tinnitus, some-
side of the chamber without any disturbances. However, times with vertigo. Very often the first symptom is tin-
to form the membrane a separate process is necessary. Our nitus. Purpose of our study was to asess hearing results
aim is to investigate the character of vibrations of the disc and complications after stapedotomy performed using ti-
suspended on two flexible rings instead of on the mem- tanum prosthesis.
brane. Such rings, alike the other elements of the prosthe-
sis, could be produced by 3D printing. In addition, due to After 25 years of succesfull experiences with platinum
an increasing rigidity of the rings with the sound ampli- prosthesis surgeons from World Hearing Center of Insti-
tude, such a suspension can create a natural protection of tute of Physiology and Pathology of Hearing in Warsaw
the ear against an excessive noise. started performing stapedotomies with KURZ Skarżyński
Pistons. From October 2012 to October 2013 we performed
Materials and Methods: Two rings between the disc and 317 surgeries with new prosthesis. Data, as medical his-
the cover and the prosthesis chamber are made of a soft tories, surgery protocols and hearing examination out-
hydrogel with viscoelastic properties. Slightly concave con- comes were reviewed and audiological results are grad-
tact surfaces of the cover and the chamber with the rings ually collected.
fix the rings position. The fixed distances between the
contact surfaces of the rings with the cover and the pros- All patients presented progressive, conductive or mixed,
thesis chamber cause the rings to pre-squeeze. When the unilateral or bilateral hearing loss, ears had never been
disc oscillates, its cyclic displacements w(t) are transferred operated before. Comparing pre- and post-operative pure
to the both rings by additional forces P(t) on the contact tone audiometry on frequencies: 0,5, 1,2,4 kHz, results are
surfaces. These forces should be much smaller than the quite satisfactory. After surgery the mean bone and air con-
forces P0 caused by their initial squeeze w0 of the discs. duction decreased, and air bone gap also decreased. Peri-
According to the Hertz contact theory, a force P(t) com- od of observation, due to initiation time of titanum pros-
pressing a cylindrical ring is proportional to the square thesis in our Center was 6 months.
of the pressure points displacement w(t). Because the dis-
placement w(t) is much smaller than the initial squeeze Stapedotomy with KURZ Skarżyński Piston is good treat-
w0, one can assume a linear approximation of the relation ment method. Satisfiying hearing effects for patients and
between P(t) and w(t). surgeons, frequent tinnitus reduction, and chance for nor-
mal social living is enough to risk this statement. To be
Results: A nonlinear equation for forced and dumped vibra- sure of that, further studies are needed.
tions of the disk has been formulated. Its linear vibrations
was found when the equation has been linearized. It was
Difficult Situations
noted, that if the initial compression of the ring is too small,
some nonlinear disk vibrations may appear. They may have
a fractal character, a typical one for chaotic vibrations. For a Decision making in far advanced
disk suspended on the pre-compressed ring, vibrations are otosclerosis and in otosclerosis with mixed
more stable, but they still may have an anharmonic character. hearing loss.
Conclusions: A disc suspended on a flexible ring is sen- Lavy J.
sitive to vibrations caused by sounds of the low intensi-
ty and stops when sound of the high intensity appear. On Royal National Throat, Nose and Ear Hospital, University
the other hand, an influence of non-linear vibrations of College London Hospitals, The London Ear Clinic, London, UK
the disc may disrupt the sound wave transmitted from
the external ear to the cochlea. It should be examined Knowing what is the true bone conduction threshold in
whether the influence of these disturbances, in the range cases of mixed and far advanced otosclerosis can be diffi-
of amplitudes of the sound wave received by the human cult. We know from the surgical treatment of simple oto-
ear, is significant. sclerosis that the Carhart effect can be considerable. In
addition in bilateral far advanced disease it can be prob-
Audiometric results after stapedotomy with lematic to ascertain which ear is providing the bone con-
KURZ Skarżyński prosthesis. duction thresholds. The author will present his results in
a series of these cases and debate decision making in how
Skarżyński H.1,2, Król B.1,2, Skarżyński P.H.2,3,4, best to treat this difficult group of patients.
Osinska K.1,2
Solutions in Complicated Stapedectomy and
1
epartment of Oto-Rhino-Laryngology, Institute of Physiology
D Revision Stapedectomy.
and Pathology of Hearing, Warsaw/Kajetany, Poland
2
World Hearing Center, Institute of Physiology and Pathology of Harguindey Antolí-Candela A., Olarieta Soto F.J.,
Hearing, Warsaw/Kajetany, Poland Antolí-Candela Cano F.
3
Heart Failure and Cardiac Rehabilitation Department, Medical
University of Warsaw, Warsaw, Poland IOM Institute, Madrid, Spain
4
Institute of Sensory Organs, Kajetany, Poland
Introduction: Stapedectomy is a surgical procedure that has
Otosclerosis is disease of the bones of the middle ear and undergone little variation since its inception and most vari-
inner ear. Vast majority of patients presents conductive ations are related to technological advances (microsurgical
drills, lasers, prosthesis). Still, anatomic variability makes diagnosis of Osteogenesis imperfecta, 19 underwent sur-
modifications on the surgical technique necessary. Treat- geries due to hearing loss. Stapedotomies, revisions after
ment options are also diverse in Revision Stapedectomy. stapedotomy, restapedotomies and ossiculoplasties were
performed. We excluded ears which underwent surger-
Material and Methods: We studied 250 cases of Stapedec- ies in other hospitals. We analyzed results of surgeries of
tomy and Revision Stapedectomy. The variations in the 22 ears. We assessed pure tone audiometry results before
surgical techniques used to solve the complications en- and after surgeries in short -term and long-term follow up.
countered have been studied. The complications encoun-
tered are divided into preoperative (Osteomas, Exostosis, Results: Results of postop air bone gap were very good
Trauma Reconstruction or other), intraoperative (Prom- [ABG <=10dB] and good[ABG >=11dB and <20dB] in
ontory Prolapse 1%, Facial Nerve Dehiscence 1%, compli- 95,5% of cases. Hearing gain >=10dB was obtained in 86%
cated Persistent Stapedial Artery (PSA) 1/3000), narrow cases in short term observation, 14% of results were be-
oval window niche 0.5%, obliterative Type IV and V Fo- tween >0dB and <10dB. Sensorineural hearing loss [BC
cus requiring drilling (6-1%), angled Incus 0.5%, an angle threshold decrease >15dB] was observed in 1 case.
of the ear cannal with the Incus which requires a broad
atticotomy recess 5%) and postoperative (Necrosis of the Conclusions: Hearing loss in Osteogenesis imperfecta in
long process of the Incus 6%, displaced prosthesis, adhe- most cases is connected with disorders within middle ear.
sions, cholesterol granuloma or iatrogenic cholesteatoma The most common is stapes pathology. Surgeries of sta-
and tympanic perforation).The solutions employed are pes allow to obtain air bone closure in many cases of Os-
described, they range from drilling of the Promontory or teogenesis imperfecta. However some cases need reop-
Footplate to modifying the prosthesis, or fixation by Oto- erations and hearing gain diminish. Due to high level of
mimix cement in Revision Stapedectomy. malformations of the structures of middle ear it is advised
to perform surgery in this group of patients only by the
Results: The functional results with intrasurgical compli- most experienced surgeons.
cations are solved successfully in 96% of the cases a year
after surgery. In revision surgery the functional results are Hearing loss treatment in patients with
directly related to the state of the oval window niche. The imperfect osteogenesis.
presence of scar tissue or grafts may condition the out-
come since in several cases its resection may involve in- Kryukov A.I., Zelikovich E.I., Fedorova O.V.,
jury to the inner ear. Therefore, in Revision Stapedecto- Garova E.E., Meparishvili A.S.
my, and depending on the findings, an optimal functional
result will mean an average air bond gap of 10dB or less Sverzhevsky Otorhinolaryngology Clinical Research Institute,
in 80% of the cases. Moscow, Russia
Discussion/Conclusion: We can provide an adequate Objective: To analyze the causes of hearing loss and the
functional surgical solution for each case except for ex- effectiveness of surgical treatment in patients with imper-
treme cases of anatomical variations or revision cases when fect osteogenesis.
there is a high risk of injury to the inner ear.
Materials: In the otosurgery department of the Institute,
Hearing loss among Osteogenesis 13 people (25 ears) with IO (imperfect osteogenesis) were
imperfecta patients- experience of World observed from 2009 to 2015, including 11 women (age
Hearing Center. from 26 to 48 years) and 2 men (21 and 27 years old).
In 8 people, the diagnosis of HB was first established af-
Osińska K.1, Skarżyński P.H. 2,3, Skarżyński H.1 ter the onset of symptoms of hearing loss and ear noise
based on CT of temporal bones, despite the large num-
1
orld Hearing Center, Institute of Physiology and Pathology of
W ber of bone fractures of limbs, scoliosis and kyphoscolio-
Hearing, Warsaw/Kajetany, Poland sis of 3 and 4 degrees. In 2 patients the diagnosis was es-
2
Heart Failure and Cardiac Rehabilitation Department, Medical tablished in early childhood. In all patients, ear noise and
University of Warsaw, Warsaw, Poland hearing loss appeared much later than other symptoms
3
Institute of Sensory Organs, Kajetany, Poland of the disease, from 4 to 8 years before resorting to a sur-
dologist. According to the tone audiometry (TA), in 12
Introduction: Osteogenesis imperfecta is a congenital dis- patients bilateral bradyacoustic hearing was noted and in
order of connective tissue, which results in malformations 1 case it was one-sided. Conductive hearing loss was ob-
of organs with high amount of collagene. The main symp- served in 20 ears (1 and 3 degrees) and mixed - in 5 (2-3
toms are numerous fractures of bones, blue sclera, hyper- degrees). The thresholds of bone conduction (CP) aver-
mobility of joints, flexible skin, dentinogenesis and in al- aged 14.7 dB, and the bone-air interval (QVI) was 32.1 dB.
most half of the patients - different types of hearing loss. According to tympanometry in some patients, the type Ad
was noted, which indicates the hypermobility of the tym-
Aim: The aims of this study are to characterize group panic membrane and / or rupture of the auditory ossicles.
of patients with hearing loss treated in the World Hear-
ing Center and to assess the hearing results of surgeries. Methods: Surgical treatment was performed in 13 patients,
12 of them in both ears (25 ears). In 22 cases, stapedoplasty
Material and Methods: In the World Hearing Center (partial stapedectomy) with laser assist and using an car-
21 patients were treated due to hearing loss with clinical tilage prosthesis (3.5 mm long) installed on the autovein
90 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
graft. The choice of cartilage prosthesis is due to its elas- Taste disorders after stapedotomy.
ticity, which reduces the risk of necrosis of the long anvil
leg in this category of patients. Wojciechowski M.1, Król B.1, Skarżyński P.H.1,2,3
Surgical treatment of the 5 – year – old boy Material and methodology: The youngest boy described
with otosclerosis. in literature with otosclerosis was submitted to surgical
treatment at the age of 5. Frontal tympanotomy was per-
Skarżyński H.1, Boruta M.1, Dziendziel B.2, formed through the external ear canal. After noting the
Skarżyński P.H.2,3,4 immobility of crus of stapes and lodgment of otosclerosis
on thickened plate, superstructure of the stapes was re-
1
torhinolaryngology Clinic, Institute of Physiology and
O moved. Then a calibrated hole with a diameter of 0.5 mm
Pathology of Hearing, Warsaw, Poland was performed. Titanium piston of the KURZ Skarżyński
2
Teleaudiology Department, World Hearing Center, Institute Piston prosthesis with a diameter of 0.4 mm was placed
of Physiology and Pathology of Hearing, Warsaw/Kajetany, in the hole.
Poland
3
Institute of Sensory Organs , Kajetany, Poland Results: The results of surgical treatment indicate a com-
plete closure of air–bone gap in the ear operated on and
Introduction: Otosclerosis is a disease that reveals itself rise of bone conductive threshold on 3 frequencies from
in the form of unilateral or bilateral hearing deteriora- 5 to 10 dB. In the ear operated on, after the removal of
tion practically in every age. The results of surgical treat- dressing, the child did not complain anymore of tinnitus,
ment of the youngest boy described in available literature which previously was persistent. In non–operative ear
are presented below. which is already planned for treatment tinnitus is varia-
ble and periodic.
Aim: The aim of this work is to present that progres-
sive, bilateral moderate hearing loss accompanied by tin- Conclusion: Surgical treatment of otosclerosis is indi-
nitus is the indication for surgical treatment, even at the cated regardless of the patient’s age. The additional in-
age of 5 years. dication for surgical treatment to small children, besides
hearing loss, are tinnitus and deterioration of bone con-
ductive threshold.
Posters
Neuroendocrine adenomas are tumors with double dif- Material and Methods: Data between 2013-2016 was col-
ferentiation. Middle ear location of those tumors is rare. lected from the Swedish Quality register of Otosclerosis
NAME causes various clinical signs. One of them could be Surgery (SQOS) including 1330 patients. Sixty-five pa-
tinnitus. This paper presents a case study of patient with tients (68 cases) with a preoperative PTA AC > 70 dB
tinnitus related to NAME. A 23-years old patient was ad- on the non-operated ear were evaluated to determine au-
mitted to the World Hearing Center because of hearing diologic improvement and correlated to the results of the
loss and tinnitus in the right ear. Physical examination sug- patient´s questionnaire one year after surgery.
gested tumor of the middle ear. Patient underwent surgi-
cal treatment (atticoantromastoidectomy) with excision Results: Sixty-eight cases, mean age 63 years (33-83) were
of pathological tissues and myringoplasty. Tinnitus was included in the study. Hearing aid was used preoperative-
evaluated using the battery of questionnaires (TFI, THI, ly in nearly all cases (98.5%) and surgery was previous-
THS). The postoperative period was uneventful. Patient ly performed on the other ear in 43% of the cases. In six
reported hearing improvement and tinnitus reduction. patients hearing thresholds were not measurable and de-
Histological examination revealed neuroendocrine ade- fined as deaf preoperatively but all improved with an air
noma of the middle ear. conduction gain. Successful surgery, defined as an ABG
closure ≤ 10 dB, improvement in AC ≥ 20 dB and BC not
BOR syndrome – case report. worsens by > 5 dB was achieved in 31% of all cases. Two
cases became deaf postoperatively necessitating a cochle-
Buksińska M.1, Błażejewska A.1, ar implantation. The patient questionnaire was responded
Skarżyński P.H.1,2,3, Skarżyński H.1 by 66% of the patients and a majority were satisfied with
the aided hearing postoperatively.
1
orld Hearing Center, Institute of Physiology and Pathology of
W
Hearing, Warsaw/Kajetany, Poland Conclusion: Stapedotomy can be beneficial even in oto-
2
Heart Failure and Cardiac Rehabilitation Department, Medical sclerotic patients with profound hearing loss fulfilling the
University of Warsaw, Warsaw, Poland inclusion criteria for cochlear implantation candidacy.
3
Institute of Sensory Organs, Warsaw/Kajetany, Poland
Optical pressure sensor for intracochlear
Branchio-oto-renal syndrome (BOR syndrome) is a rare, measurement – design and making.
autosomal dominant genetic disorder. BOR syndrome is
characterized by an association of I and II branchial arch Girejko G., Michałowski M., Kamieniecki K.,
anomalies and renal malformations. Ear malformations Kwacz M.
can include outer ear, middle ear and inner ear as well.
IMiF, Warsaw University of Technology, Warsaw, Poland
Objectives: We report 7,5 year-old girl who presented to
World Hearing Center with bilateral hearing loss. Past Aim: The aim of this study was the analysis of the sen-
medical history included right-sided renal agenesis. sor making process suggested by Elizabeth S. Olson, PhD,
and building intracochlear pressure sensor due to this
Materials and metods: Patient had bilateral mixed hear- instruction.
ing loss. We present diagnostic evaluation and treatment
of this patient. Methods and Results: The sensor operates as follows: light
emerges from the optic fiber, disperses and is reflected
Conclusion: I and II branchial arch anomalies present in from the membrane. The amount of light which returns
patients with BOR syndrome require proper diagnosis. to the fiber is correlated with pressure-induced membrane
Treatment depends on the severity of symptoms and va- motion and distance to the membrane. This is the way
riety of ear malformations. how sensor detects pressure. Making the sensor requires
making sensor tip, putting the membrane on the tip, etch-
ing optic fibers, making sensor rig and connecting the rig
and tip together. Then, the calibration process is needed. requires extended facial recess approach and perform-
To make the sensor we used a diode with ST connector, ing stapedotomy or cochleostomy.3) Placing measuring
multimode optic fiber, a glass capillary tube and a pho- tools: firstly, insert earphone in external ear canal, place
todiode. Firstly, glass capillary tubing was cut into piec- the probe microphone with a tube 2 mm and a speaker
es about 1 cm long. Once, the tips have no rough edges, about 4-5 mm from tympanic membrane. 4) Insertion of
adding the membrane was performed (Fig.1). We have pressure sensors consists in thinning the cochlear prom-
made the membrane from adhesive glue (NOA 68, Nor- ontory and fenestration of 0.5 mm diameter in SV and
land Products INC, USA). A drop of the NOA 68 was ST. Each tip of the transducer is supposed to be inserted
placed on surface of deionized water. After spreading, about 100-300 µm into the scalae and sealed with alginate
the membrane was pre-cured, placed on the tip and fi- material. 5) During experiment immersing the TB in sa-
nally cured. All the presented images are self-made pho- line helps prevent air presence. Figure 1 Measuring sys-
tographs. Fig. 1 Preparation the membrane from adhesive tem. Figure 2 Temporal bone and measuring tools, on the
glue NOA 68. A: The amount of demineralised water, B: right: an example of TB (1 – earphone, microphone probe
a drop of NOA, C: the drop spreading on the surface, D: with a tube, 3 – round window membrane). The differ-
experimental membranes on straws. Evaporating the tip ential intracochlear pressure, which correlates with inner
(Fig. 2) is necessary to make the membrane as reflective. ear excitation, is measured simultaneously in SV and ST.
We performed the evaporation process in a special vacu-
um chamber. Fig. 2 Evaporation process. A: a laboratory Results: Different teams from all over the world have ex-
chamber uncovered, B: the amount of Aluminium, C: the amined intracochlear pressure. In the study by Nakajima
chamber during the process. Subsequently, we have etched (2008) there is a major difference between sound pressure
(Fig. 3) the optic fiber, as its outer diameter (OD; 125 um) in scala vestibuli and scala tympani for middle frequen-
is too large to fit into the tip. We etched one end of the cies, pressure in SV is much greater. As far as differential
fiber by immersing it in a 6% hydrofluoric (HF) acid so- pressure is considered it was reduced by 30 to 50 dB for
lution for several minutes. In order to quicken the pro- disarticulated ossicular chain. Olson (1998) presented re-
cess, the solution temperature was kept between 65-75 de- sults showing the SV pressure for upper frequency limit
grees. After etching, the OD was about 65-70 um. Then, was approximately 30 dB higher than pressure in ear ca-
the etched fiber was inserted into the tip. Fig. 3 Control- nal. In these measurements the SV input pressure has a
ling of the OD during the etching process. A: after 5 min, range between 105 and 120 dB SPL. The phase of SV de-
B: after 10 min, C: after 15 min. Our team is during the creases linearly with frequency. The study by Nakajima
process of constructing the rig with a Fiber Optic coupler. (2010) resulted in a higher magnitude of pressure in SV
The system will be supplied in light with Laser Emitting between 0.6 to 8 kHz. The phase of differential pressure
Diode (LED). The light, reflected by the sensor tip will be below 0.6 kHz was between 0-150 degrees and became
detected by a Photo Diode (PD). The strength of the re- negative with the frequency increase.
flected light is correlated with the measured pressure fluc-
tuations. Before the voltage signal from the PD will be re- Conclusions: A sensible clinical methodology is proposed.
corded by an acquisition system, it should be augmented It consists in measurements of intracochlear pressure of
by an operational amplifier. perilymph before and after stapedotomy. Results relying
on the technique presented above are to be used to ful-
Conclusions: The process of making the sensor is demand- fil the preclinical data inevitable in the process of assess-
ing, however self-made sensors are needed as their sensitiv- ing the effectiveness of a new chamber stapes prosthesis.
ity and specificity are high and their functioning is known.
Is the chamber stapes prosthesis safe for
How to measure pressure in the perilymph patients?
fluid?
Girejko G.1, Mrówka M.2, Skarżyński P.H.2,
Girejko G.1, Kwacz M.2 Kwacz M.1
1
IMiIB, Warsaw University of Technology, Warsaw, Poland 1
IMiF, Warsaw University of Technology, Warsaw, Poland
2
IMiF, Warsaw University of Technology, Warsaw, Poland 2
Institute of Physiology and Pathology of Hearing, Warsaw,
Poland
Aim: The most efficient parameter defining the effec-
tiveness of middle ear prosthehis is pressure generated Aim: New chamber stapes prosthesis (ChSP) is a medi-
in perylimph. The aim of this study is to formulate the cal device intended for use in the stapedotomy surgery.
methodology of performing pressure measurements us- The ChSP has an innovative design and is composed of
ing optoelectronical sensor. three elements: the chamber, the membrane, and the plate.
Although the simulation and experimental studies have
Material and Methods: In order to carry out the experi- shown the effectiveness of the ChSP functioning, its safe-
ment several devices and tools presented in Figure 1 are ty of use has not yet been demonstrated. The aim of this
needed. Temporal bones should be fresh, ear canal shall be work is to perform the preliminary risk analysis (RA) and
shorten to 1 cm and extended facial recess approach car- to assess the risk-benefit ratio.
ried out. 1) In order to carry out the experiment, a sound
of particular intensity (90 dB SPL) will be calibrated, gener- Material and Methods: The RA was performed according
ated and controlled. The acoustic frequency will vary from to ISO 14971. The hazards were determined and risk for
400 Hz up to 10 000 Hz. 2) Temporal bone preparation: each hazardous situation (HS) was estimated in qualitative
94 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
manner based on expert opinion. The risk was defined as flexible membrane (2, FM) that closes the wider base of
the combination of the probability (P) of occurrence and the chamber, and a rigid plate (3) placed on the FM. The
the severity (S) for each harm. The P levels were: P1-high chamber and the rigid plate were made from acryloni-
(likely to happen), P2-medium (can happen but not fre- trile-butadiene-styrene (ABS-M30i) using ProJet 3600
quently), P3-low (unlikely to happen). The S levels were: Printer. The FM was self-made from UV-light curing ad-
S1-significant (death or loss of function or structure), S2- hesive NOA 68 (Norland Optics, USA) according to tech-
moderate (reversible or minor injury), S3-negligible (will nique described by Olson (JASA, 1998). The FM had a
not cause injury or will injure slightly). The benefit aris- thickness of ~25 um. The ChSP prototype (Fig.1B) was
ing from the ChSP was estimated in comparison with the filled with deionized water, covered with the FM and im-
piston prosthesis.Results.Each of hazards can progress to planted in a human cadaveric temporal bone specimen.
the HS if foreseeable sequences of events (SE) occur. Re- Fig. 1. The ChSP prototype (A, B) and experimental setup
lationship between hazards (Hz), SEs, HS and the harm (C, D). B – temporal bone, S – acoustic signal, M – mi-
(Hm) that can occur are as follows:Hz1: Acoustic or me- crophoneA scanning laser-Doppler vibrometer (PSV400,
chanical energy. SE:1. Sudden loud sound, noise expo- Polytec, Germany) was used to measure vibrations of the
sure or violent head injury; 2.Prosthesis membrane dam- round window membrane (RWM) before and after the
age. HS: ChSP cannot transmit sound. Hm: Conductive or ChSP-stapedotomy. An acoustic signal (90dB SPL, 0.8-
sensori-neural hearing loss (CHL/SNHL) or mixed hear- 8 kHz) was introduced to the external ear canal via an
ing loss (MHL), reoperation. HZ2: Biological. SE:1.Sudden ER-2 loudspeaker (Etymotic Research, USA). The 90-dB
loud sound or violent head injury, 2.Unsealing of cham- SPL near the tympanic membrane was controlled by an
ber. HS:Bacteria or viruses enter to inner ear, fluid leakage. ER-7C microphone (Fig.1C).The RWM vibration in a
Hm:Bacterial or viral infection, SNHL, pain, reoperation. normal ear was measured at 155 scan points. Then, the
Hz3:Biocompatibility. SE:1.Incorrect chemical composi- stapes footplate (SF) was immobilized, the stapedial mus-
tion of fluid filled the chamber, 2.Fluid merges with per- cle was cut, the stapes suprastructure was removed and
ilymph. HS:Toxicological reaction. Hm: SHL, dizziness. a 0.6-mm hole was drilled in the SF. Into this hole, the
Hz4:Function. SE:1.Incorrect fatigue strength of mem- thin tube of the ChSP (4 in Fig.1A) was inserted and the
brane or plate, 2. Fatigue failure. HS:Long-term reduced chamber was fixed to the oval window niche. The long
or impossible sound transfer. Hm: Long-term CHL. The process of the incus was fixed between the sidewalls of
estimated risks associated with the biological and biocom- the rigid plate (Fig.1D). Then, the RWM vibration in the
patibility hazards are on the unacceptable S1 level and risk post-stapedotomy ear was measured.Based on the meas-
reduction is required. The benefit arising from the ChSP ured displacement amplitudes, the magnitude of fluid
in comparison with the piston includes: (1) the chamber volume displacement (VD) at the RWM was calculated.
cannot be lifted out of the vestibule nor move in too deep Results:In Figure 2, the VD magnitude at the RWM be-
into the vestibulum, (2) the cochlear stimulation is on the fore and after the ChSP-stapedotomy are shown. Fig. 2.
physiological level, (3) masking effect for high-frequen- Fluid VD at the RWM in the normal ear and the ChSP-
cy tinnitus can occur. stapedotomy ear. 90 dB SPL in the external ear canal.For
f > 1.0 kHz, the VD induced by the ChSP is signifi-
Conclusions: There are any clinical data to confirm com- cantly higher than the VD in the normal ear. The mag-
pliance with relevant essential requirements for safety (Di- nitude of the VD depends on both the stiffness of the
rective 93/42/EEC) when using the ChSP. Therefore, to FM (e.g. its thickness and material parameters) and the
validate that the ChSP meets acceptable risk-benefit cri- surface area of the chamber wider base.
teria, clinical testing is required.
Conclusions:The ChSP prototype works correctly and ef-
Chamber stapes prosthesis – preclinical fectively transmits sound for frequencies 0.8-8 kHz. Fur-
study of sound transmission. ther studies are necessary to design the biocompatible FM.
Kwacz M.1, Pudlik M.2, Mrowka M.3, Gambin W.1 Coexistence of congenital stapes ankylosis
with middle ear malformations in children.
1
IMiF, Warsaw University of Technology, Warsaw, Poland
2
IRE, Warsaw University of Technology, Warsaw, Poland Młotkowska-Klimek P., Skarżyński H.
3
World Hearing Center, Insitute of Physiology and Pathology of
Hearing, Warsaw/Kajetany, Poland Department of Oto-Rhino-Laryngology, Institute of Physiology
and Pathology of Hearing, Warsaw/Kajetany, Poland
Aim: New chamber stapes prosthesis (ChSP) is intend-
ed for patients with stapes otosclerosis. Before being in- Aim: The aim of the study was the analysis of intraoper-
troduced to clinical practice, a number of preclinical and ative findings in the middle ear and hearing results in pa-
clinical tests are required. The ChSP functioning has been tients after stapedotomy performed in treatment of con-
numerically simulated (Kwacz, Hear Res, 2014) and it genital hearing loss.
was shown that the prosthesis effectively transmits vibra-
tion for frequencies 0.4-10 kHz. The aim of this study is Material and Methods: Study group comprised 36 patients
to assess the sound transmission in ear implanted with a operated before 18 years of age, with congenital conductive
ChSP prototype. or mixed hearing loss. All underwent stapedotomy. Anom-
alies found in the tympanic cavity have been classified ac-
Materials and Methods: The ChSP prototype (Fig.1A) cording to the Teunissen and Cremers classification. Pre-
consists of a conical chamber (1) filled with fluid, a operative diagnostics included laryngological, audiological
and radiological examination. Postoperative diagnostics in- and Medline. Using the combination of keywords: “stapes
cluded otological and audiological assessment. surgery”, “child”, “juvenile” and “otosclerosis”, 188 articles
were found by PubMed, 31 by Web of Science and 46 by
Results: Conditions found intraoperatively in the middle Medline. As the literature on the topic is scanty and one
ear have been described and classified. Hearing results of the objectives was to analyse the development that oc-
have been assessed at 12 months after surgery. curred in the treatment of pediatric otosclerosis, we did
not limit the timeframe of our search. Based on the eli-
Conclusions: Stapes ankylosis may be coexistent with gibility criteria, 19 articles were found suitable for the fi-
different changes in the middle ear. Stapedotomy is a safe nal elaboration.
method of treatment of hearing loss in children with con-
genital middle ear defect and stapes ankylosis. CT imag- Results: About 400 cases of surgical treatment of pediatric
ing is an essential element of diagnostics before surgery otosclerosis have been described in the articles included
in order to exclude hypertension in the inner ear, but not in the review. The age of patients undergoing stapes sur-
always conclusive. gery ranged from 5 to 21 years with the preponderance
of girls in comparison to boys (in a ratio: 4-3/1). The au-
Progressive otosclerosis with bilateral thors of the analyzed papers emphasized rather low sen-
sensorineural hearing loss and vestibular sitivity of the computed tomography in the diagnosis of
impairment- case report. pediatric otosclerosis. Recent studies on stapedotomy au-
diological outcomes in children indicated that this proce-
Pietrasik K., Tacikowska G., Sosna M., dure allows for the air-bone gap closure in more than 92%
Skarżyński H. of operations. Due to the diversity of grafts and prostheses
used, it was impossible to establish their relationship with
World Hearing Center, Institute of Physiology and Pathology of postoperative results. Complications of stapes surgery were
Hearing, Warsaw/ Kajetany, Poland extremely rare and included in most cases transient diz-
ziness. There was also no relationship between the age of
Hearing loss, tinnitus and dizziness are common com- pediatric patients and postoperative closure of the air-bone
plains among patients with otosclerosis. Usually it takes gap and the frequency of postoperative complications.
years from early onset to deafness. The aim of the study
is to present a case of progressive otosclerosis with epi- Conclusions: Stapes surgery for pediatric otosclerosis is a
sodes of sudden hearing loss and loss of balance within safe procedure resulting in closing air-bone gap in long-
2 years.43-years old woman with bilateral sensorineural and short-term observation. The patient’s age should not
hearing loss was treated for episodes of sudden loss with be a contraindication to the operation.
steroids and after 2 years received cochlear implant be-
cause of non-functional residual hearing. Her otoneuro- Stapedotomy in the only hearing ear with
logical problems included imbalance in motion especially persistent tinnitus.
in darkness and oscillopsia. Magnetic resonance imaging
revealed intensive otosclerotical process of the inner ear. Skarżyński H.1,2, Plichta L.1,2, Wawszczyk S.1,2,
Authors present audiological, otoneurological and radio- Skarżyński P.H.2,3,4
logical findings of the patient. In progressive otosclerosis
with residual hearing and bilateral vestibular impairment, 1
epartment of Oto-Rhino-Laryngology, Institute of Physiology
D
cochlear implantation and vestibular rehabilitation were and Pathology of Hearing, Warsaw/Kajetany, Poland
the optimal treatment solution. 2
World Hearing Center, Institute of Physiology and Pathology of
Hearing, Warsaw/Kajetany, Poland
Stapes surgery for paediatric otosclerosis – a 3
Institute of Sensory Organs, Kajetany, Poland
literature review. 4
Department of Heart Failure and Cardiac Rehabilitation,
Second Faculty, Medical University of Warsaw
Rajchel J.J.1, Skarżyński H.1, Dziendziel B.1,
Boruta M.1, Skarżyński P.H.1,2,3 The treatment of the only hearing ear is always a chal-
lenge for the otosurgeon and even more so for the pa-
1
orld Hearing Center, Institute of Physiology and Pathology of
W tient, especially if previous surgery in the other ear had
Hearing, Warsaw/Kajetany, Poland been with complications. However, many years of expe-
2
Heart Failure and Cardiac Rehabilitation Department, Medical rience with such cases suggests that indications for sta-
University of Warsaw, Warsaw, Poland pedotomy in conductive hearing loss should be the same
3
Institute of Sensory Organs, Kajetany, Poland whether we making a decision about surgery in the only
hearing ear or in both ears. The aim of this study was to
Aim: The objective of the study was to synthesize pub- present a case of a patient with only one hearing ear with
lished data on stapes surgery for pediatric otosclerosis, additional atypical intraoperative finding and to demon-
regarding course of the diagnostic process, the usefulness strate the such findings do not warrant resigning from
of pre-operative imaging techniques, the characteristics of performing a stapedotomy.
patients undergoing surgery, the course of the operation,
its results and possible complications. The patient was a 62 years-old woman admitted to the
clinic due to hearing problems and tinnitus, who had
Material and Methods: A literature search was performed undergone surgeries in both ears 19 and 22 years ear-
using the following databases: PubMed, Web of Science lier. The audiometry showed total deafness in the left
96 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
ear and profound mixed hearing loss in the right ear. Hearing Aid Benefit (ABHAB) and Assessment of Quali-
Hearing thresholds in the right ear in the past 22 years ty of Life (AQoL-8D). The protocol of the study includes
from the surgery were deteriorating gradually and pres- completing the questionnaires before surgery and after 3
ently the air conduction was at the level of 60 dB, and and 6 months. Additionally, pure-tone audiometry is con-
bone conduction at 25-30 dB with tenacious tinnitus. ducted to evaluate the hearing benefit.
It has been decided to perform the stapedotomy in the
right ear under general anesthesia. An intrameatal inci- Results: There are a few scientific publications on the prev-
sion was performed in the narrow external auditory ca- alence and severity of tinnitus in patient undergoing sta-
nal. The external ear canal was observed to be widened pes surgery. Nowadays, there is still a tendency to report
during a previous surgery that failed to improve patient’s only postoperative hearing outcomes based on pure-tone
hearing. Intraoperatively surgeons had observed discon- audiometry. The preliminary study carried out in our de-
nection of the stapes suprastructure from the plate and partment showed that tinnitus affects a substantial group
very narrow oval window. The divulsion of the stapes oc- of stapes surgery candidates. This finding prompted us to
curred most probably during a previous operation which create the comprehensive protocol of assessing tinnitus in
involved stapes mobilization. The stapes suprastructure relation to both subjective and objective hearing benefit
was removed and a 0.5 mm diameter hole was drilled in and quality of life.
the stapes plate. A titanium prosthesis KURZ Skarżyński
Piston 0.4 mm was applied. Conclusion: The study allows the development of an orig-
inal protocol to evaluate the original protocol to evalu-
After removal of the ear canal packing full closure of the ate severity of tinnitus in the course of otosclerosis and
air-bone gap was achieved with improvement of the bone their impact on the subjective assessment of hearing and
conduction curve by 5-10 dB. The patient reported im- quality of life.
mediate diminishment of the tinnitus and after 2 months
tinnitus was completely resolved. Audiologic outcomes after stapedotomy in
Ehlers – Danlos Syndrome – pediatric cases
The only hearing ear cannot be nowadays a contraindica- report.
tion for surgical treatment. In each of such cases it is es-
sential to assure that patient has full knowledge and un- Skarżyński P.H.1,2,3, Dziendziel B.1,
derstanding of possible complications. Surgeon must have Wawszczyk S.1, Boruta M. 1, Skarżyński H.1
adequate experience in such procedures. In our patient’s
case the surgery had been mandatory due to lowering 1
orld Hearing Center, Institute of Physiology and Pathology of
W
of the bone conduction curve and burdensome tinnitus. Hearing, Warsaw/Kajetany, Poland
2
Heart Failure and Cardiac Rehabilitation Department, Medical
Tinnitus evaluation method in adult University of Warsaw, Warsaw, Poland
patients with otosclerosis. 3
Institute of Sensory Organs, Kajetany, Poland
Skarżyński P.H.1,2,3, Dziendziel B.1, Świerniak W.1, Aim: Ehlers – Danlos syndromes (EDS) is a rare inherited
Bienkowska K.1, Skarżyński H.1 group of connective tissue disorder characterized by joint
hypermobility and delicate, hyperelastic skin. The aim of
1
orld Hearing Center, Institute of Physiology and Pathology of
W the study was to assess audiologic outcomes in two ado-
Hearing, Warsaw/Kajetany, Poland lescents with EDS after stapedotomy.
2
Heart Failure and Cardiac Rehabilitation Department, Medical
University of Warsaw, Warsaw, Poland Material: The study involves two pediatric patients with
3
Institute of Sensory Organs, Kajetany, Poland history of increasing hearing loss in both ears. They
both showed clinical features of the EDS syndrome. The
Aim: Subjective tinnitus is a common complaint among 14-year-old boy was observed bilateral conductive hear-
patients with otosclerosis. It can exacerbate the negative ing loss, progressing since the year 8. The patient under-
impact of hearing loss on people’s daily life. Hence, dis- went mobilization in the left ear and two years later - sta-
tinguishing patients with bothersome and nonbothersome pedotomy in the right ear. The second case concerns a
tinnitus is a diagnostic challenge with a possible impact 13-year-old girl who suffered from bilateral mixed hear-
on postoperative benefits. The aim of the study is to pre- ing loss, progressing from early childhood. In addition, the
sent a protocol for evaluating severity of tinnitus in adult girl had severe tinnitus and periodic dizziness. The patient
patients with otosclerosis. The secondary objective is to underwent a bilateral, sequential stapedotomy.
assess the hearing benefit and quality of life in patients
with and without tinnitus. Methods: Pure tone air- and bone – conduction audiom-
etry were used to evaluate hearing outcomes. The pure
Material: Patients over 18 years of age are included in – tone average was calculated for air and bone conduc-
the study. The eligibility criteria are occurrence of tinni- tion using the frequencies: 0.5, 1.0, 2.0 and 4.0 kHz. For
tus and first-time scheduled for the operation in the in- these frequencies was calculated also air – bone gape be-
vestigated ear. fore and after surgery.
Methods: The participants are asked to fill in the follow- Results: In the boy, the average air conduction thresh-
ing questionnaires: Tinnitus Functional Index (TFI), Tin- old decreased by 13 dB after mobilization surgery and
nitus and Hearing Survey (THS), Abbreviated Profile of 23.8 dB after stapedotomy. In the girl who underwent
bilateral stapedotomy, the avereage air conduction thresh- The results of surgery to obtain satisfactory postoperative
old decreased by 30.3 dB in the left ear and 27.5 dB in the hearing is also depended on the experience of a surgeon.
right ear. In the 2-year observation, the stability of hear-
ing thresholds was observed. Ossiculoplasty in single sided conductive
hearing loss with suspected otosclerosis.
Conclusion: Few studies have been published regarding
the outcomes surgery of patients with EDS with the ma- Skarżyński H.1,2, Bartosik J.1 , Kaczyńska B.2 ,
jority of EDS literature focusing of surgical complication. Skarżyńska M.B.2,3
To our knowledge, these are the first pediatric patients
with EDS, who underwent stapedotomy. The obtained 1
epartment of Oto-Rhino-Laryngology, Institute of Physiology
D
good results of hearing improvement, which are stable in and Pathology of Hearing, Warsaw/Kajetany, Poland
the 2-year observation, confirmed the validity and effec- 2
World Hearing Center, Institute of Physiology and Pathology of
tiveness of performed stapes surgery. Hearing, Warsaw/Kajetany, Poland
3
Institute of Sensory Organs, Kajetany, Poland
Long-term hearing results after stapes 4
Department of Heart Failure and Cardiac Rehabilitation,
surgery- observation of 5 and 10 years Second Faculty, Medical University of Warsaw, Poland
follow-up.
Introduction: Single sided conductive hearing loss, lack
Skarżyński H.1, Kutyba J.1, Ratajczak A.1, of stapedius muscle reflex in both ears, type A tympanom-
Gocel M.1, Gos E.1, Skarżyński P.H.1,2,3,4 etry, no changes observed in otoscopic examination sug-
gest usually the diagnosis of otosclerosis. In such cases the
1
orld Hearing Center, Institute of Physiology and Pathology of
W preoperative evaluation should involve decision that intra-
Hearing, Warsaw/Kajetany, Poland operative assessment is necessary for the surgeon to make
2
Heart Failure and Cardiac Rehabilitation Department, Medical a final decision about treatment.
University of Warsaw, Warsaw, Poland
3
Institute of Sensory Organs, Warsaw/Kajetany, Poland Material and Methods: The analysis is based on the case
4
International Center of Hearing and Speech Medincus, study of 38-years old patient, who in the diagnostic pro-
Kajetany / Nadarzyn cess had been repeatedly informed that her hearing loss
is caused by otosclerosis.
Aim: Otosclerosis is an abnormal growth of bone near the
middle ear which can lead to conductive, mixed or even Results: Patient has been qualified for surgical treatment
sensorineural hearing loss. According to literature one of – exploratory tympanotomy with possible stapedotomy
the most effective treatment of this disease is stapes sur- or other type of reconstruction of middle ear conductive
gery. The aim of this study was to evaluate the results of apparatus. Intraoperatively was observed the destruction
stapes surgery after 1, 5 and 10 years follow-up, accord- of the long process of incus, with its fragments in a strap
ing to pure-tone audiometry results. of tendon tissue between the body of incus and the head
of stapes. This is why the CT images made before sur-
Material: From over 21 000 stapes surgeries (stapedoto- gery had shown that the long process is thinned but pre-
mies, restapedotomies and stapedotomy revisions), which served. The long process of incus has been reconstructed
constitute the World Hearing Center material, 100 patients with glass-ionomer cement. Very good hearing results have
operated in 2004-2006 were randomly selected. This group been achieved with closure of the air-bone gap.
of patient underwent stapedotomy in process of otoscle-
rosis. The observed group is consisted on men and wom- Conclusions: Presented stages of the diagnostic process
en aged 18-60. and intraoperative findings demonstrate then it is nec-
essary to carefully formulate the inferences, even initial,
Methods: Preoperative and postoperative results of pure- with regard to the final diagnosis and implemented oto-
tone audiometry for air and bone conduction were ana- surgical treatment.
lyzed to evaluate hearing outcomes. The mean hearing
thresholds for air and bone conduction were calculated Certain causes of surgical treatment failure
using the frequencies: 0.5, 1.0, 2.0 and 4.0 kHz. To evalu- of otosclerosis - case report.
ate improvement after stapes surgery mean preoperative
and postoperative air-bone gap (ABG) was calculated. Skarżyński H., Plichta Ł.
Results: Basing on the long-term results after stapes sur- World Hearing Center, Institute of Physiology and Pathology of
gery, it was found that in the majority of selected group Hearing, Warsaw/ Kajetany, Poland
of patients air-bone gap closed to within 10 dB. The ex-
tensive results of long-term observation after stapes sur- The course of treatment of a 55 years old woman hospi-
gery are going to be presented during the 4th International talized due to progressive, bilateral, mixed hearing loss
Symposium on Otosclerosis and Stapes Surgery in Cracow. caused by otosclerosis is discussed. Prior to admission, the
patient had undergone: stapes mobilization, then right ear
Conclusions: The stapes surgery in process of otosclero- stapedotomy and restapedotomy and finally left ear sta-
sis is a save and successful treatment for long-term hear- pedotomy. Transitory improvement of hearing after sta-
ing improvement. It is shown that many patients had air- pes mobilization with following deterioration with lower-
bone gap reduction and in turn significant hearing gain. ing both air and bone conduction thresholds in the right
98 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
ear were the indication for stapedotomy. After the oper- the survey responded: Prof. W. Hause, prof. E. Offeciers,
ation improvement of hearing was achieved but air bone prof. M. Tos., prof. U. Fisch, prof. J.M. Sterkers, prof. J.
gap from 35 to 15 dB persisted. Due to this fact after al- Helms, prof. T. McDonald, prof. T. Balkany, prof. E. Myers,
most a year restapedotomy resulted in full closure of the prof. E. Lehnhard, who presented conservative approach.
air bone gap. Finally because of hearing loss in the left ear The proposed method of treatment involved convention-
stapedotomy on this side was performed. Revision opera- al hearing aids for as long as possible. Only half of survey
tions in otosclerosis demand sufficient experience due to responders considered that exceptional situations may be
a variety of possible causes of failure. It is especially dif- an indication for surgery.
ficult when the first operation was performed by anoth-
er surgeon in another center. Stating significant air bone In the last 16 years, the author performed this procedure
gap is an indication for reoperation by the most experi- in 613 only hearing ears . The contralateral ears were deaf
enced otosurgeon in the team. The most common cause because of advanced, long-term otosclerotic lesions and
of failure of stapedotomy is occurrence of concretion af- damage of the inner ear, or damaged by the previous sta-
ter subsequent operations. pedotomy. All patients were informed on conditions of
their hearing, possible chances and on rare but possible,
Promontorium drilling for better not intended complications. Full cooperation of patients
visualization of oval window - case report and patients’ consent were the basis for decision on stape-
dotomy of the only hearing ear. Our results in this group
Skarżyński H., Plichta Ł. of patients indicate hearing efficiency preserved in 99.2%
in the only hearing ear.
World Hearing Center, Institute of Physiology and Pathology of
Hearing, Warsaw/ Kajetany, Poland These exceptionally good results obtained in a significant
number of patients with otosclerosis and patients with the
We present a case of a 62 years old woman with progres- only hearing ear, that in treatment of such cases we should
sive, bilateral hearing loss lasting for 30 years due to oto- use the typical criteria. The surgeon should make a con-
sclerosis. We describe one of the difficulties encountered scious and confident decision on the operation. Lack of
during stapedotomy and remedial measures necessary decision will not, however, stop the destructive processes
for successful operation. During operation the otosur- in the inner ear, but it may prevent future effective ampli-
geon found a narrow oval window niche with overhang- fication or cochlear implantation.
ing promontory. It was decided to drill a fragment of the
promontory for better visualization of the niche and to Authors underline that the decision on surgery for oto-
perform successful stapedotomy. The operation enabled sclerosis in the only hearing ear should be taken with care
closure of the air-bone gap without sensorineural hearing and attention paid to fast elevating thresholds, increase of
deterioration. The patient is awaiting the operation of the tinnitus and vestibular changes seen on CT images. Sur-
other ear.Stapedotomy gives an outstanding opportunity gery in the only hearing ear is as risky as any other stapes
to improve hearing in otosclerosis, however certain situa- surgery. Stable hearing after surgery in the only hearing
tions require nonstandard measures. In the described case ear confirms the effectiveness of proposed treatment, but
drilling a fragment of promontory enabled successful ac- does not exclude the possibility of complications.
complishment of the operation.
Impact of stapes surgery on tinnitus in
Stapedotomy in the only hearing ear. patient with osteogenesis imperfecta – a
case study.
Skarżyński P.H.1,2,3, Skarżyński H.1
Skarżyński P.H.1,3,4 Osińska K.1,2 Dziendziel B.1,
1
orld Hearing Center, Institute of Physiology and Pathology of
W Rajchel J.1, Skarżyńska M.B.1, 4
Hearing, Warsaw/Kajetany, Poland
2
Heart Failure and Cardiac Rehabilitation Department, Medical 1
orld Hearing Center, Institute of Physiology and Pathology of
W
University of Warsaw, Warsaw, Poland Hearing, Warsaw/Kajetany, Poland
3
Institute of Sensory Organs, Kajetany, Poland 2
Oto-Rhino-Laryngology Surgery Clinic, Institute of Physiology
and Pathology of Hearing, Warsaw/Kajetany, Poland
Over 25 year experience in surgical treatment of otosclero- 3
Heart Failure and Cardiac Rehabilitation Department, Medical
sis, during which the second author has performed more University of Warsaw, Warsaw, Poland
than 20,000 operations, allow authors formulating the 4
Institute of Sensory Organs, Warsaw/Kajetany, Poland
opinion with regard to decisions about treatment of the
only hearing ear. This subject has been presented for dis- Osteogenesis imperfecta (OI) is the most common heredi-
cussion for the first time at the national conference in Ka- tary disorder of connective tissue. It is associated with de-
towice in Poland in 1998. The author presented then a re- fects of the osteoarticular system, dentinogenesis imper-
search based on a survey carried out among outstanding fecta and progressive hearing loss. The aim of study was
international surgeons. Author’s opinion at that time was to retrospectively evaluate tinnitus and hearing of patient
very careful and the only proposed indication for such in- with Osteogenesis Imperfecta after stapes surgery. A 60 –
tervention was sudden deterioration of hearing with a fast year old woman has undergone bilateral, sequential stapes
process of vestibule obliteration, confirmed by computed surgery. She was asked to complete the Tinnitus Functional
tomography and audiometric results indicating elevated Index (TFI) to assess the annoyance tinnitus and Abbrevi-
hearing thresholds preventing effective amplification. In ated Profile of Hearing Aid Benefit (ABHAB) to subjective
assess hearing. Questionnaires consisted of two sets. The (air conduction and bone conduction thresholds). The
first set was about preoperative state, the second about results were analyzed preoperatively, at 1, 5 and 10 years
postoperative state. The audiological findings of this pa- postoperatively. The mean preoperative and postopera-
tient have been described earlier. A patient reported pro- tive air-bone gap (ABG) was calculated as the difference
gressive hearing loss and constantly tinnitus over 35 years between the air conduction (AC) and bone conduction
before first operation. Preoperative TFI results showed, (BC) thresholds. The average hearing gain was calculated
that tinnitus was the biggest problem in subscales: intru- as the difference between the preoperative and the post-
sive, sense of control, sleep and relaxation. She reported operative ABG.
a complete disappearance of tinnitus after surgery. Preop-
erative audiometric tests showed bilateral mixed hearing Results: The results will be presented during the 4th In-
loss. After surgery, closure of the air – bone – gap in left ternational Symposium on Otosclerosis and Stapes Sur-
ear and significant reduction in right ear was observed. gery in Cracow.
The APHAB results confirmed the reported improvement
of hearing after stapes surgery. Osteogenesis imperfecta is Conclusion: According to literature stapes surgery should
characterized by disorders of bone metabolism, with hear- be offered to the elderly patients (especially when it is pos-
ing loss being a frequently reported factor. Patients with sible to recover social hearing) with the same indications
this disorder often have malformation of stapes, which as younger patients with otosclerosis even if audiomet-
may influence the postoperative hearing results. There are ric results are better in younger groups. Stapedotomy or
many publication which present exclusively postoperative stapedectomy in the elderly is indicated to avoid hearing
hearing result. Little is known about annoyance of tinnitus aid use or to facilitate hearing aid fitting, when the asso-
in this group. This case shows that there is an opportuni- ciation of otosclerosis and presbycusis makes hearing aid
ty to obtain improved hearing and also reduction of tin- unserviceable.
nitus. This effect was maintained in 5 year follow-up pe-
riod. As a result of sequential stapedotomy in both ears, Effectiveness of stapes surgery in the elderly
a patient reported subjectively improved hearing and to- – review of literature.
tal reduction of tinnitus.
Skarżyński P.H.1,2,3, Gocel M.1, Dziendziel B.1,
Effectiveness of stapes surgery in patients Skarżyński H.1
over 60 years old.
1
orld Hearing Center, Institute of Physiology and Pathology of
W
Skarżyński P.H. , Gocel M. , Bieńkowska K. ,
1,2,3 1 1
Hearing, Warsaw/Kajetany, Poland
Dziendziel B.1, Kutyba J.1, Ratajczak A.1, Gos E.1, 2
Heart Failure and Cardiac Rehabilitation Department, Medical
Skarżyński H.1 University of Warsaw, Warsaw, Poland
3
Institute of Sensory Organs, Kajetany, Poland
1
orld Hearing Center, Institute of Physiology and Pathology of
W
Hearing, Warsaw/Kajetany, Poland Aim: Otosclerosis is a complex and progressive disease of
2
Heart Failure and Cardiac Rehabilitation Department, Medical pathological bone remodeling that affects the otic capsule
University of Warsaw, Warsaw, Poland of the temporal bone, resulting in conductive and with
3
Institute of Sensory Organs, Kajetany, Poland time sensorineural or mixed hearing loss. Otosclerosis is
most common in women aged 15-40. However, as live ex-
Aim: Otosclerosis is a disease that leads to progressive, pectancy increases, there may be a tendency to observe
unilateral or bilateral hearing loss, firstly conductive, and more elderly patients.
with time mixed or sensorineural hearing loss (depend-
ing on the location of otosclerotic foci and the severity of The aim of this study is to measure effectiveness of sta-
the disease). Otosclerosis is the most common in group of pes surgery in the elderly according to literature review.
women aged 15-40. However, as live expectancy increas-
es, there may be a tendency to observe more elderly pa- Material and Methods: The review is based on electronic
tients who need stapes surgery. databases, containing scientific articles: PubMed, Web of
Science and Google Scholar. The publications have been
The aim of this study was to analyze hearing results af- found by using consecutive key words: „otosclerosis in
ter stapes surgery in otosclerotic in group of patients old- the elderly”, „stapedotomy in the elderly”, „age-related
er than 60 years. otosclerosis”, age-related stapedotomy”, „stapes surgery
in the elderly”. Eventually, 10 English-language publica-
Material and Methods: The object of these retrospective tions, in which at least one of the presented groups of
study were patients undergoing stapes surgery. From over patients were patients over 60 years of age were select-
21 000 stapes surgeries (stapedotomies, resapedotomies ed and analyzed.
and stapedotomy revisions), which constitute the World
Hearing Center material, 50 patients operated on in 2004- Results: According to the authors, decisive factor for suc-
2006 were randomly selected. In all cases otosclerosis were cessful surgery is closure of air-bone gap in 0-10 dB. This
diagnosed preoperatively. Age was an inclusive criterion. result was obtained in over 70% of older patients. Based
All patients at the time of surgery were over 60 years old. on a literature review, it was observed that in groups of
elderly patients preoperative bone conduction thresholds
The evaluation of effectiveness of stapes surgery in elderly were elevated (> 30 dB HL), which may be dictated not
patients was made on the grounds of pure-tone audiometry only by otosclerosis, but also by presbycusis hearing loss.
100 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
Conclusion: Surgical methods of otosclerosis treatment Conclusion: Basing on results of pure-tone audiometry
are equally effective in the elderly as in other age groups. and the APHAB questionnaire, significant and long-term
However, in three publications, a different position was auditory benefits after stapedotomy were observed. There
postulated, paying attention to poorer postoperative re- are few scientific reports on the surgical treatment of con-
sults of people over 60 years of age compared to young- genital malformations in the course of concomitant rare
er patients. At the same time, the value of other aspects diseases. In many medical centers, the standard procedure
demonstrating the benefits of otosclerosis treatment in is compensation of hearing loss using classic hearing aids
the elderly, such as: inhibition of the disease process, or bone anchored hearing aids BAHA. The decision to use
but also older patients experience greater satisfaction a stapedotomy in case of congenital defects of the middle
especially in terms of daily activities and interperson- ear depends mainly on the degree of malformation. The
al relations. otosurgeon’s experience is another important factor deter-
mining the success of the operation.
Evaluation of auditory benefits in a patient
with Treacher Collins Syndrome after Review of possible postoperative
stapedotomy - a case report. otoneurological complications after
stapedotodomy.
Skarżyński P.H.1,2,3,4, Ratajczak A.1,2,
Dziendziel B.1, Skarżyński H.1 Sosna M., Tacikowska G., Uszyńska-Tuzinek M.,
Skarżyński PH., Skarżyński H.
1
orld Hearing Center, Institute of Physiology and Pathology of
W
Hearing, Warsaw/Kajetany, Poland World Hearing Center, Institute of Physiology and Pathology of
2
Heart Failure and Cardiac Rehabilitation Department, Medical Hearing, Warsaw/ Kajetany, Poland
University of Warsaw, Warsaw, Poland
3
Institute of Sensory Organs, Kajetany, Poland Aim of the study: Operation of otosclerosis, despite its
4
International Center of Hearing and Speech Medincus, effectiveness and popularity, may cause disturbance in
Kajetany, Poland vestibulum function. The aim of the study is to present
possible postoperative vestibular complications and their
Aim: The Treacher Collins Syndrome is characterized by mechanism.
numerous deformities within the facial skeleton. Congen-
ital malformations of the external and middle ear are also Materials and Methods: Review of the literature and ret-
observed in this group of patients which results in conduc- rospective analysis of the history of the patients’ after sta-
tive or mixed hearing loss. The aim of the study is to as- pedotomy procedure, operated in Otorhinolaryngosurgery
sess the auditory benefits of a patient with Treacher Col- Clinic in Institute of Physiology and Pathology of Hearing.
lins Syndrome after a stapedotomy.
Results: The postoperative vertigo after stapedotomy may
Material: The work concerns the 26-year-old woman with be divided in: short-lasting and persistent. The first is
Treacher Collins Syndrome with mixed hearing loss caused observed in 3-7% of the patients, the latter in less than
by bilateral congenital defects of the middle ear. The pa- 0,5%. The most frequent causes for short lasting verti-
tient did not accept sound amplification using bone an- go include: the contact of prosthesis or surgical instru-
chored hearing aids and classic hearing aids for air con- ments with membranaceous labirynthus of the sacculus
duction. At the age of 11 a stapedotomy in the ear with a and utricle, the change of the fluids pressure in vestibu-
larger hearing loss (right ear) was performed. lum after drilling the opening in the footplate of the sta-
pes, perylimph aspiration during the procedure, labir-
Methods: The results of pure-tone audiometry for air and ynthitis, enzymatic process conjoined with releasing the
bone conduction were analyzed to evaluate hearing out- toxic substances by the otosclerotic plaques. The causes of
comes. The mean hearing thresholds for air and bone con- persistent vertigo may be: replacement of the otolith with
duction were calculated using the frequencies: 0.5, 1.0, 2.0 consequent benign paroxysmal positional vertigo, persis-
and 4.0 kHz. To assess the subjective benefits of hearing tent perylimphatic fistula, too deep penetrating prothesis
after the surgery, the Abbreviated Profile of Hearing Aid with pressure on the membranaceous labirynthus of utri-
Benefit (APHAB) questionnaire was used. Its individu- cle and sacculus, vestibular damage, dislocation of the
al scales helped to estimate understanding of speech in prosthesis (too short prosthesis, necrosis of long process
different environmental conditions and reactions to sur- of the incus), adhesiones.
rounding sounds.
Conclusions: Vestibular disorders are not rare compli-
Results: Before the operation, the mean air conduction cations of stapedotomy procedure. In the majority of
threshold was 60 dB HL, and the size of air-bone gap was cases the symptoms are present only in postoperative
26 dB in the operated ear. After the stapedotomy, a sig- period and are the consequence of disturbance in in-
nificant improvement in hearing was achieved. Postoper- ner ear fluid homeostasis, resolves spontaneously and
ative air-bone gap shows the improvement of air and bone do not demand any further interventions. Sometimes,
thresholds obtained after stapedotomy versus preoperative the persistent vestibular symptoms are the indication
air bone gap. In the long 15-years observation hearing in for reoperation and repositioning maneouvers. Every
the operated ear has not exacerbated. The results of indi- patient, before signing the informed consent, should
vidual APHAB scales showed improvement in subjective be informed about the possible influence of stapedot-
hearing in different environmental conditions. omy on vestibular organ.
Simplified three-dimensional basilar Results: Otosclerotic foci were visualized in 74% 68%, and
membrane model in FSI analysis. 59% of the cases by neurotologists, neuroradiologists and
radiologists, respectively. Radiographically, fenestral oto-
Tudruj S., Piechna J., Kamieniecki K., sclerosis was noted in 97% of the above cases with 14% of
Borkowski P. ears also having cochlear involvement. In all patients with
positive CT scan reads by neurotologists, otosclerosis was
Warsaw University of Technology, Warsaw, Poland confirmed intraoperatively. Out of all reviewed ears, bi-
lateral otosclerosis was noted in 67% of cases. The aver-
Aim: Numerical simulations of FSI (fluid structure inter- age cochlear aqueduct length, midline diameter and aper-
action) type are time-consuming. This is due to the need ture dimensions in otosclerotic ears were 12.19, 0.68 and
of using two solvers, which have to communicate one 2.60 mm, respectively. There was no noted statistical dif-
with each other during work. One of these solvers per- ference in these dimensions among otosclerotic and non-
forms calculations in the domain of a solid body (FEM otosclerotic ears.
solver), while the other in the fluid domain (CFD solv-
er). To speed up the calculation, it was proposed to use Conclusions: In our series, the rate of radiographic de-
one solver working in the fluid domain, with the possi- tection of otosclerosis widely differed based on the train-
bility of performing simple calculations for a solid body, ing background of the individual reviewing the scans. Hy-
that is, the basilar membrane. podense otosclerotic foci were primarily situated along the
anterior footplate. Metric evaluation of cochlear aqueduct
Material and Methods: ANSYS FLUENT is a solver de- dimensions revealed no statistically significant difference
signed to perform fluid dynamics calculations. A user- among otosclerotic and normal ears. The average dimen-
defined function, or UDF, is a function that you program sions were comparable to previously published data regard-
that can be dynamically loaded with the ANSYS FLUENT ing healthy ears. Hence, we can conclude that the struc-
solver to enhance the standard features of the code. Us- ture of cochlear aqueduct and potentially its patency are
ing this tool, a basilar membrane model was created and not effected by otosclerosis.
added to the solver. The basilar membrane is modeled as
a set of resonators. The movement of resonators is trans- MicroRNA Expression Profiling in
ferred directly to the fluid by means of appropriate bound- Perilymph Specimen of Otosclerotic
ary conditions. Thanks to this, the geometry of the model Patients.
does not change and there is no need to modify its mesh,
which is a time-consuming task. Wichova H., Shew M., Staecker H.
Results: The proposed solution resulted in a significant University of Kansas Medical Center, Lawrence, USA
acceleration of the calculation compared to the tradition-
al FSI model. Introduction: Otosclerosis is a relatively rare inflammato-
ry bone remodeling disorder of the temporal bone with a
Conclusions: The simplified FSI basilar membrane mod- complex and not fully understood etiology. A progression
el, based on the ANSYS FLUENT solver and the UDF of inflammatory bone resorption, most commonly along
mechanism, seems to be a good alternative to tradition- the fissula ante fenestram, results in progressive conduc-
al, time-consuming FSI models for simulating the inner tive hearing loss due to fixation of the stapes. Ultimate-
ear operation. ly, 70% of patients with conductive hearing loss undergo
stapedectomy or stapedotomy. (1) At our institution, we
High Resolution Computed Tomography have been serially collecting perilymph samples for micro-
of the Inner Ear: Effect of Otosclerosis on RNA (miRNA) analysis. MiRNA are 19-23 base pair sin-
Cochlear Aqueduct Dimensions. gle stranded RNA sequences that regulate mRNA transla-
tion and play a vital role in a myriad of cellular pathways
Wichova H., Alvi S., Lin J., Staecker H. and disease pathologies. (2) MiRNA regulate and define
expression of genes through guided degradation through
University of Kansas Medical Center, Lawrence, USA various mechanisms and have been implicated in several
neurodegenerative disorders through modulation of stress
Objectives: The aim of this study was to determine the and survival pathways. (3,4) Of clinical significance, miR-
radiographic detection of otosclerosis and compare to in- NA have been successfully identified in numerous body
traoperative findings, as well as to provide radiographic fluids including blood serum, saliva, urine, and CSF and
metric classification of the cochlear aqueduct. were found to serve as biomarkers for detecting and mon-
itoring numerous pathophysiological conditions. (5)
Methods: A retrospective chart review of seventy-sev-
en high resolution CT scans in patients with a diag- Aim: The aim of this project is to describe the miRNA
nosis of otosclerosis based on clinical and operative profile in the perilymph of patients with otosclerosis un-
findings. All scans were evaluated by neurotologists, dergoing surgical interventions.
neuroradiologists and general radiologists. The lo-
cation and extent of otosclerotic foci were recorded. Materials and Methods: The perilymph of 4 patients un-
Unified methods were then used to determine the di- dergoing stapedectomy with identified otosclerotic foci
mensions of the cochlear aqueduct in both affected was collected. Perilymph was collected using a sterile glass
and unaffected ears. capillary applied to the CO2 laser created oval window
102 © Journal of Hearing Science® · 2018 Vol. 8 · No. 1
4th International Symposium on Otosclerosis & Stapes Surgery
opening. MiRNA expression profiling was carried out us- otosclerosis and not noted in our comparative general
ing the Affymetrix GeneChip miRNA 4.0 Arrays. Data population were found. Correlation between these spe-
sets were then analyzed using Ingenuity Pathway Analy- cific miRNA and processed involved in osteoporosis were
sis (IPA) software. IPA is an online software tool for iden- further noted.
tifying significant molecular interaction networks and bi-
ological functions associated with a set of genes (miRNAs) Conclusion: Otosclerosis is a relatively rare disease mak-
based on information gathered in the Ingenuity Pathway ing the access to perilymph samples limited. Preliminary
Knowledge Base (IPKB). Furthermore, miRNA profiles data shows possible relationships which will need to be
from above mentioned patients were compared to miR- further investigated with a larger patient sample size. Ul-
NA perilymph expression norms established in our lab to timately, the goal is to identify a panel of molecules that
detect components specific to otosclerosis. serve as otosclerosis markers by defining miRNA that are
unique to perilymph and networks of miRNAs that inter-
Results: Collected samples on average yielded 5-2 ng of act with molecules believed to be involved in the patho-
total RNA per sample in a volume of 2-5 µl. Among all genesis of otosclerosis.
four specimen, miRNA specific to middle ear and cochlear