Cupulolithiasis

Harold F. Schuknecht, MD, Boston

This paper is dedicated to Julius Lempert peared a strong rotatory nystagmus to the
whose ingenuity, skill, and enthusiasm set otol¬ right. The attack lasted about thirty seconds
ogy in motion in this century. It was a privilege and was accompanied by violent vertigo and
and honor for me to have come momentarily nausea. If, immediately after the cessation of
under the tutelage of this superb otologie sur¬ the symptoms, the head was again turned to
geon and teacher. the right, no attack occurred and in order to
evoke a new attack in this way, the patient had
to lie for some time on her back or on her left
THE TERM cupulolithiasis is presented side.
for the first time to designate a vestibular Barany and others originally attributed
disorder which previously has been identi- this disorder to lesions in the semicircular
fied by several names including postural ver- canals but, because the dizziness was precip¬
tigo, positional vertigo, and positional verti- itated not by head movement but by head
go of the benign paroxysmal type. Recent position, they came to believe that the con¬
pathological studies support the concept dition was due to a disorder of the otoliths.
that the disorder is caused by a deposit, Dix and Hallpike2 in 1952 made a notable
presumably composed of mineral, on the contribution to our understanding of posi¬
cupula of the posterior semicircular canal tional vertigo. In a study of 100 patients
which renders this organ sensitive to gravi- with this symptom they found a high inci¬
tational force and, therefore, subject to stim- dence of ear disorders (eg, otitis media,
ulation with changes in head position. The acoustic trauma). They noted that the verti¬
clinical features of cupulolithiasis are dis- ginous attacks occurred when the abnormal
tinctive and serve to differentiate it from ear was placed undermost in the testing
positional vertigo caused by lesions of the procedure. Their observations supported
central nervous system. The diagnosis can Bárány's opinion that this type of vertigo is
be made by inducing the characteristic ves- of inner ear origin.
tibular manifestations by provocative posi- In 1956 Lindsay and Hemenway3 de¬
tional testing. scribed for the first time a symptom complex
B\l=a'\r\l=a'\ny1 first described the disorder as he which they observed in seven patients. It
observed it in a 27-year-old woman and he was characterized by a sudden, severe, and
wrote as follows: prolonged vertiginous episode which subsid¬
The attacks only appeared when she lay on ed after several weeks and was followed by
her right side. When she did this, there ap- positional vertigo. All were in the fifth, sixth
or seventh decade of life and the positional
Submitted for the Julius Lempert memorial issue
of the ARCHIVES. vertigo persisted for weeks to years. All had
From the Harvard Medical School and the Mas- impaired response to caloric testing and
sachusetts Eye and Ear Infirmary, Boston.
some had hearing loss. These functional al¬
Reprint requests to Massachusetts Eye and Ear
Infirmary, 243 Charles St, Boston 02114. terations existed in the ear located under-

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 most with the head in the provocative posi¬ Many individuals experience a mild sensation
tion. of nausea during and immediately following the
attack and a few have nausea and vomiting. In
Subsequently, I4 presented some additional rare cases nausea and vomiting may persist
clinical observations, reported the results of
for several hours and these patients often are
some pertinent animal experiments, and in¬
reluctant to undergo provocative testing.
terpreted the pathological findings in three The sensation of vertigo is always of short
sets of previously reported temporal bones
duration, usually about five to ten seconds,
from patients with this disorder. One of the which helps to distinguish this condition from
sets of temporal bones resides in the collec¬ other vestibular disorders. Because of the in¬
tion of John Lindsay in Chicago and the tensity of the vertiginous sensation the patients
other two in the collection of C.S. Hallpike commonly assign a longer duration to the at¬
in London. It was also noted that ablation tack and careful questioning may be required
of vestibular function in the involved ear— to elicit a reliable history. At the onset of the
that is, the ear located undermost with the attack most patients quickly move from the
head in the provocative position—gave com¬ provocative head position and attribute to this
the quick termination of the attack; others,
plete relief of positional vertigo in four pa¬ however, will relate that if the head is main¬
tients (three of Citron and Hallpike and one tained in the provocative position the vertigo
of mine). The evidence from all sources will subside in a few seconds.
appeared to support the idea that positional In many cases the disorder is self-limiting
vertigo of the benign paroxysmal type, as it and subsides within a few weeks or months; in
was then termed, is caused by substances of others there are remissions and recurrences
high specific gravity, possibly otoconia, act¬ over time spans of weeks to years and in still
ing upon the cúpula of the posterior semicir¬ others it is persistent. Some patients report
cular canal. that the attack is produced only occasionally by
The purpose of the present report is to the provocative position, a feature which is
review the previous evidence once again and confirmed by inconsistent responses to the test
to present new data from animal experi¬ procedure.
ments as well as the pathological findings in In an attempt to determine a possible etio¬
two additional sets of human temporal logic factor, the historical record should include
information relative to recent head injury, ear
bones, all of which may be interpreted to symptoms (tinnitus, hearing loss, recent or past
support the thesis that this particular type otorrhea, ear surgery), or a recent severe pro¬
of positional vertigo is a clinical entity suit¬ longed vestibular upset.
ably termed cupulolithiasis. Although the sex distribution has not been
studied, it is Barber's5 impression and also
Symptoms mine that most patients with the disorder are
females.
The principal complaintof the patient is the
occurrence of sudden attacks of vertigo precipi¬ Test Procedure
tated by certain head positions. Usually the
patient volunteers that the attack can be in¬ After acquiring the history, the diagnosis of
duced by rolling over in bed either to one side cupulolithiasis usually can be promptly con¬
or the other but not to both sides. Some state
firmed by the positional test procedure which is
that the attack is provoked by a sudden move¬ performed as follows:
ment of the head to the right or left, or when The patient is seated upon an examining
extending the neck, as in looking upward, and, table with the head turned to one side and the
more rarely, upon stooping over. Characteristi¬
gaze fixed on the examiner's forehead. The
cally, there is a severe subjective sensation of examiner then grasps the head in his hands and
disequilibrium which many patients find in¬ briskly places the patient into the supine
tensely disagreeable. Those with good motor head-hanging position. The head should be
function rarely fall because the onset, although placed below the level of the table and 30 to 40
sudden, is not apoplectiform, so that there is degrees to one side. After the position has been
time to grasp for support during the short assumed there is a quiescent latent period of
period of disequilibrium. Aged patients experi¬ one to six seconds (average of three or four
encing loss of muscle strength and control are seconds). If the vertigo is induced the patient
more in danger of falling. usually demonstrates a sensation of distress

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 variously manifested by closing the eyes, grasp¬ of vestibular disorder following head in¬
ing the examiner, crying out in alarm, and jury.2611 Barber5 found this type of posi¬
making active efforts to sit up again. If the tional vertigo in 47% of 47 patients with
nystagmus is to be observed the patient must longitudinal fracture of the temporal bone
be instructed before the test to keep the eyes
and in 20.8% of 77 head injuries of compa¬
open. With the eyes in the position of foreward rable severity but without skull fracture.
gaze the nystagmus is predominantly rotatory.
In the right-ear-down position the rotation is Other types of trauma to the vestibular
counterclockwise. If the reaction occurs in the system which may be incurred from a head
left-ear-down position the nystagmus is oppo¬ blow are: (1) transverse fracture of the tem¬
site to that of the right-ear-down position. With poral bone, and (2) injury to the vestibular
the eyes directed toward the uppermost ear the neural pathways of the brain. Transverse
nystagmus is vertical and upward beating. The fracture results in disruption of the membra¬
nystagmus increases in rapid crescendo for nous labyrinth and severe loss of auditory
three to ten seconds and then rapidly subsides and vestibular function. Injury to the vestib¬
as the patient's distress is relieved. The patient
ular pathways in the brain may incite posi¬
is brought back into the sitting position with
the head still grasped by the examiner's hands. tional vertigo but the nystagmus often is
Almost immediately there is vertigo and nys¬ direction-changing, and lacks the onset de¬
tagmus in reverse to that elicited in the pre¬ lay, rotatory movement, limited duration,
vious position, but less severe and for a shorter fatigability, and severe subjective sensation
period of time. Each time the patient is placed which are so characteristic of the cupulolithi¬
into the provocative supine position the reac¬ asis syndrome.
tion is less severe, and often it cannot be It has been suggested previously411 that
elicited more than two or three times. A rest the possible mechanism for the development
period is required before the phenomenon can of positional vertigo following head injury is
be produced again. In the complete test pro¬
cedure the patient is placed in turn into both disruption of the utricular otolithic mem¬
right and left head-hanging positions. brane and release of otoconia into the endo¬
lymph of the pars superior. Theoretically
Etiologic Factors the otoconia, being free to respond to gravi¬
tational force, would settle into the ampulla
Spontaneous Degenerative Change, Ves¬ of the posterior canal and with change of
tibular Labyrinth.—Many patients suffering head position could forcibly displace the
from this disorder have no history of antece¬ cúpula. Postmortem studies of the temporal
dent head injury, ear disorder, neurological bone in head-injured patients thus far have
disease, or ear surgery. They are usually in failed to reveal dislodged otoconia, probably
the fifth, sixth, or seventh decades of life because they are absorbed by the décalci¬
and usually demonstrate no unusual degen¬ fication process during histological prepara¬
erative changes of aging. It is possible that tion. Vyslonzil,12 utilizing a dissection pro¬
the disorder in these patients is an expres¬ cedure, without prior décalcification, found
sion of spontaneous degenerative change in otoconia in the posterior semicircular canals
the vestibular labyrinth, consisting specif¬ of patients receiving ototoxic doses of strep¬
ically of the generation of substances of tomycin. Presumably, the reason these pa¬
high specific gravity (possibly loose otocon¬ tients did not demonstrate positional vertigo
ia) in the endolymph of the pars superior was that the function of the posterior canal
(utricle and canals). The symptoms often crista had been destroyed by the drug.
subside spontaneously after a few weeks or Otitis Media.—Dix and Hallpike2 were
months, but may recur, and may persist the first to observe that many patients with
indefinitely. Most patients learn to live with positional vertigo of the benign paroxysmal
the disorder but consider it an annoyance type have evidence of previous or existing
and wish to be free of it. A few experience ear infection. In a study of 100 cases of
disability of sufficient magnitude to impose positional vertigo evidence of otitis media
restrictions on social and occupational activ¬ was found in 26 of which 11 were bilateral
ities. and 15 were unilateral. All 15 patients with
Labyrinthine Concussion.—Positional ver¬ unilateral evidence of otitis media elicited
tigo probably is the most common type the positional nystagmus with the diseased

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 ear placed undermost during the test pro¬ positioned with the right undermost.
ear
cedure. The caloric responses normal. Histo¬
were
Ear Surgery.—Positional vertigo of the logical study was reported to show a normal
paroxysmal type occurs as an uncommon left labyrinth and, on the right, normal semi¬
complication of stapes surgery and usually circular canals but severe degenerative
can be related to traumatic manipulations changes in the utricle, saccule, and cochlea.
involving the structures within the vestibule. The sensory epithelium of the utricle ap¬
The probable pathogenesis of postoperative peared disorganized, the underlying stroma
positional vertigo in these cases is rupture of was thick and infiltrated with cells, and the
the utricular otolithic membrane associated otolithic membrane was absent.
with release of otoconia. Usually the symp¬ Lindsay and Hemenway3 described the
tom subsides within a few weeks but, rarely, findings in the temporal bones of the patient
may persist for months or years. The com¬ who experienced a complex of symptoms
plication is uncommon following other types which they attributed to occlusion of a ves¬
of temporal bone surgery (fenestration, mas¬ sel supplying the vestibular labyrinth. At
toidectomy, tympanoplasty) and does not the age of 65 this patient experienced the
occur after labyrinthectomy. sudden onset of severe vertigo associated
Occlusion of Anterior Vestibular Artery. with vomiting which gradually subsided
—Lindsay and Hemenway3 described in over a period of one month. Subsequently,
1956 a symptom complex observed in pa¬ she had vertiginous episodes whenever she
tients in the latter decades of life which they assumed the supine position with the head
suspected was caused by occlusion of a ves¬ turned to the right. The vertigo was readily
sel supplying the vestibular labyrinth. The demonstrated by positional testing. Caloric
first manifestation of the disorder was the response was absent in the right ear. The
sudden onset of severe vertigo without deaf¬ positional vertigo continued until her death
ness or signs of central nervous system dis¬ 13 years later. Histological study was re¬
ease, followed by gradual recovery during ported to show degeneration of the superior
the subsequent few weeks. The clinical pat¬ division of the vestibular nerve in the right
tern was consistent with that known to ear only (Fig 1).
result from sudden destruction of one laby¬ In 1957 Cawthorne and Hallpike13 report¬
rinth. A second manifestation of the syn¬ ed the histological findings in a 59-year-old
drome was the development of positional woman who for two years had not been able
vertigo, commencing after the original attack to lie on her right side for fear of precipitat¬
had subsided, and persisting for weeks or ing an attack of vertigo. Caloric response
years. They presented the pathological was decreased in the right ear and position¬
findings in the temporal bones from a pa¬ al testing produced the attack in the supine
tient with this syndrome which were consis¬ right-ear-down position. Histological study
tent with occlusion of the anterior vestibular was reported to show moderate degeneration
artery. This artery supplies the utricular of the utricular macula and nerve and the
macula and the cristae of the horizontal and crista of the horizontal semicircular canal.
superior semicircular canals. Presumably de¬ The remaining structures of the right ear
generation of the utricle and its otolithic and the entire left ear were reported as
membrane following devascularization of being normal. It was the authors' opinion
this structure would release otoconia which that the findings were consistent with ather-
would then be free to settle upon the cúpula omatous occlusion of the arterial supply to
of the posterior semicircular canal. the right utricle and horizontal canal crista.
After examining the histological prepara¬
Pathological Findings tions of these three previously reported
cases, I concur with the general interpreta¬
Dix and Hallpike2 reported the findings tions of the authors. The diseased ear in
in the temporal bones of a 40-year-old wom¬ each case shows degeneration of the macule
an who had severe right sensorineural deaf¬ of the utricle and cristae of the horizontal
ness and positional vertigo of 20 years' du¬ and superior canals and the superior vestib¬
ration. The attacks occurred with the head ular nerve. In each ear the crista and nerve

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 Fig 1.—Atrophy of the superior division of the vestibular nerve (N), utri¬
cle (U), and horizontal semicircular canal crista (C) in a patient with posi¬
tional vertigo of the paroxysmal type. Courtesy of Lindsay and Hemenway.

of the posterior canal appears to be intact. corded. There was no reaction in the supine
I have recently acquired the temporal right-ear-down position. Pure tone audiometrie
bones from two patients who experienced tests showed a mild bilateral sensorineural
typical positional vertigo of the paroxysmal hearing loss for frequencies above 2,000 cycles
per second (cps). Speech discrimination was
type. The histological findings in the two not tested.
sets of temporal bones are nearly identical
These symptoms continued unchanged and
and provide further evidence concerning the were noted in the medical record on several
unique pathophysiology of this disorder. occasions during the following three years, the
last mention being made when the patient was
Report of Cases 74 years of age. She died at the age of 77 of
CASE 1.—At the age of 69, the patient first massive cerebral hemorrhage, and both tempor¬
experienced paroxysmal attacks of vertigo pre¬ al bones were removed for histological study.
cipitated by stooping over, lying down, and, in Histological Study.—The temporal bones are
particular, when rolling onto the left side. They in an excellent state of histological preservation
did not occur when rolling onto the right side. and preparation. The middle ears and mastoids
She noted a tendency to stagger and fall for¬ are well pneumatized and appear normal. The
ward and to the left and occasionally had the organs of Corti show normal hair cell popula¬
sensation that she was being pulled backward. tions throughout. There is a diffuse uniform
On two occasions she fell to the floor at the loss of about 60% of the spiral ganglion cells in
onset of an attack. The episodes were of short both cochleae.
duration, could be precipitated at will, and In both ears the utricular and saccular macu¬
were not associated with auditory symptoms. lae have normal sensory epithelium and intact
Examination at the age of 71 revealed normal otolithic membranes and the vestibular nerves
tympanic membranes. Caloric tests using 5 cc appear normal. All cristae show intact sensory
of water at 80 F produced responses of 90 epithelium.
seconds on both sides which was considered to Attached to the posterior surface of the cúp¬
be normal. There was no spontaneous nystag¬ ula of the left posterior semicircular canal is a
mus. Tests for positional vertigo provoked a basophilic staining homogenous deposit measur¬
severe vertiginous episode of short duration ing 300/u in its greatest dimension (Fig 2 and
associated with clockwise rotatory nystagmus 3).
when she was placed in the supine left-ear- CASE 2.—At the age of 64, the patient began
down position. Details such as onset latency, having attacks of vertigo precipitated by
exact duration, and fatigability were not re- changes in head position. The duration of each

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 Fig 2.—Dense deposit (D) on the cúpula (C) of the posterior semicircular canal of the left ear.
Tests for positional vertigo provoked a severe vertiginous episode of short duration associated with
clockwise rotatory nystagmus when she was placed in the supine left-ear-down position (case No. 1).

Fig 4.—There is a dense deposit (D) on the cúpula

(C) of the posterior semicircular canal of the left ear.
Tests for positional vertigo provoked a clockwise ro¬
Fig 3.—Normal crista and cúpula of the right pos¬ tatory nystagmus and subjective sensation of vertigo
terior semicircular canal (case No. 1). in the supine left-ear-down position (case No. 2).

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 Fig 5.—Normal crista and cúpula of the right posterior semicircular canal (case No. 2).

Fig 6.—There is a thin layer of granular deposit (D) on the membranous wall
of the left posterior semicircular canal in its most inferior portion (case No. 2).

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 attack was but a few seconds and it was not enth decade of life and which persisted for
associated with auditory symptoms or loss of the remainder of their life spans—eight
consciousness. On one occasion she fell to the years for one and four years for the other.
floor, striking the occiput but did not lose One patient had fallen to the floor twice and
consciousness. The attacks were most severe
the other once but neither were injured.
when arising from bed, and she had to sit on
Both were mildly disabled but were able to
the edge of her bed for some seconds before she
could rise. Audiometrie tests revealed near-nor¬ live moderately comfortably with slight re¬
mal pure tone thresholds, speech reception lev¬ striction in activities. Neither patient had
els of 12 db, and discrimination scores of 92% experienced a head injury, ear infection, ear
in each ear. Caloric tests (Kobrak minimal and surgery,or symptoms suggestive of occlusion
Hallpike bithermal) were interpreted to be of the anterior vestibular artery, and the
normal. Electroencephalogram (ENG), skull etiology presumably was spontaneous degen¬
films, and brain scan were normal. Tests for erative change of the vestibular labyrinth.
positional vertigo with eyes in central position The significant pathological finding in
provoked clockwise rotatory nystagmus and each is a basophilic deposit, presumably of
subjective sensation of vertigo in the supine high specific gravity, located on the cúpula
left-ear-down position. The nystagmus had a
latency of six seconds, and a duration of seven of the posterior canal of the ear which was
seconds. With eyes closed and recorded on undermost when vertigo was induced on po¬
ENG the latency was 13 seconds and the dura¬ sitional testing. The origin of the deposit is
tion 33 seconds. Upon assuming the upright not obvious on histological study. It proba¬
position there was counterclockwise rotatory bly was not generated from the cúpula r
nystagmus and subjective vertigo. The nystag¬ substance as the cupulae appear identical to
mus had a latency of seven seconds and a the cupulae of the posterior canals of the
duration of four seconds. When recording on opposite ears which do not have this depos¬
ENG with eyes closed the latency was five it. The utricular otolithic membranes appear
seconds and the duration was 20 seconds. No normal as compared to the saccular otolithic
reaction occurred in the supine right-ear-down
membranes of the same ears and to the
position. The vertiginous symptoms continued otolithic membranes of the opposite ears;
unchanged for four years when at the age of 68
she died of pulmonary embolus, secondary to the integrity of the otoliths, however, can¬
congestive heart failure. Both temporal bones not be evaluated because they have been
were removed for histological study. resorbed by the décalcification process to
Histological Study.—The temporal bones are which they were subjected during histologi¬
well pneumatized and the mastoids, middle cal preparation.
ears, ossicles, and tympanic membranes appear It is possible that the cupular deposits
normal. There is mild preparation artifad con¬ represent calcium carbonate derived from
sisting of areas of slight fragmentation of the otoconia, perhaps altered to some extent,
bony structures, and scattered small tears in and imbedded in a type of matrix which is
the membranous structures. There is compres¬
sion artifact to the extent that Reissner's mem¬
stained by hematoxylin. It is known that in
some animal specimens prepared by intravi¬
brane is in contact with the organ of Corti in
some areas. The organs of Corti and spiral tal preparation the region of the otolithic
ganglia are atrophied in the basal 3 MM of the membrane occupied by otoconia is stained
cochleae but are normal elsewhere. The utricu¬ to show the outline of these crystals. It is
lar and saccular maculae and the sensory epi¬ also possible that the deposits represent
thelium of all cristae, as well as the vestibular products of degeneration from other sources.
nerves, appear normal in both ears. In a study of 550 specimens at the Massa¬
Attached to the cúpula of the left posterior chusetts Eye and Ear Infirmary Ruby (un¬
semicircular canal is a granular, basophilic published data) found similar deposits on
staining mass measuring 350/i in its greatest the cupulae of the posterior canals in 15 for
dimension (Fig 4 and 5). A thin layer of this an incidence of 207%. In another 53 there
material also is located on the membranous were small amorphous granular deposits on
wall of the posterior semicircular canal in its the cupulae of the posterior canal. No such
most inferior location (Fig 6).
deposits were found on the cupulae of the
Comment.—Both of these patients de¬ superior and horizontal canals. It is proba¬
veloped positional vertigo during the sev- ble that some of these deposits are the result

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 of postmortem degeneration of the utricular 7). The superior part of the macules of the
otolithic membrane. saccules were atrophied in two ears. It is
common for the superior part of the macule
Experimental Observations of the saccule to be supplied by a branch of
the anterior vestibular artery; the main sup¬
The observations from several experi¬ ply, however, is from a branch of the poste¬
ments have provided information which is rior vestibular artery. The posterior canal
pertinent to the proposed concept regarding cristae and inferior divisions of the vestibu¬
the pathogenesis of positional vertigo of the lar nerves were intact in all four.
paroxysmal type. The vestibular membranous labyrinth was
Cutting the Anterior Vestibular Artery in distorted and partially collapsed in all four,
the Cat.—In an experiment previously re¬ possibly because of decreased endolymph se¬
ported by me,4 the superior division of the cretion resulting from atrophy of the dark
vestibular nerve as well as the anterior ves¬ cell areas (Fig 8). This may explain why
tibular artery were sectioned. It is not tech¬ only one of four animals developed position¬
nically feasible to cut only the artery. The al vertigo, for the collapsed ampullary wall
operation was performed on the left ears of of the posterior canal would impair move¬
four cats by a surgical approach through the ment of the cúpula.
superior surface of the petrous pyramid. Postrotatory Stimulation of the Posterior
Thus, it was possible to cut these structures Semicircular Canal.—An experiment was
within the internal acoustic canal. performed to determine whether isolated
Each animal experienced a postoperative stimulation of a single posterior semicircular
vestibular upset consisting of horizontal nys¬ canal causes rotatory nystagmus on forward
tagmus to the right, ataxia, falling to the gaze.
left, and head tilt to the left. The acute signs Two operative procedures, consisting of
of vestibular upset subsided gradually over a a right labyrinthectomy followed in three
period of days, but postural abnormalities weeks by section of the superior division of
such as slight ataxia and head tilt persisted. the left vestibular nerve, were performed on
Positional vertigo having the character of a cat. This animal, therefore, retained ves¬
the benign paroxysmal type developed in tibular function of only the left posterior
one of the four cats. It was first elicited semicircular canal and saccule. A test for
three months after operation and persisted postrotatory nystagmus was performed six
until termination of the experiment at seven weeks later with the head positioned so that
months. A rotatory clockwise nystagmus oc¬ the left posterior canal was in the plane of
curred only in the supine position with the rotation. The nystagmus which resulted was
left ear undermost. The period of delay purely rotatory. With ampullofugal flow the
varied from one to five seconds and the nystagmus was clockwise and strong and
duration from 20 to 36 seconds. The inten¬ with ampullopetal flow it was counterclock¬
sity and duration of nystagmus diminished wise and weak.
rapidly on repeated testing but did not fa¬ Histological preparations of the temporal
tigue completely on repeated testing. bones revealed successful total ablation of
All four cats were killed seven months the right vestibular system and destruction
after operation and the temporal bones pre¬ of the sense organs of the left utricle and
pared for histologie study. Each left ear superior and horizontal canals as planned.
showed severe atrophy of the superior divi¬ There was partial collapse of the membra¬
sion of the vestibular nerve, the macule of nous labyrinth including the ampullary wall
the utricle, and the cristae of the superior of the posterior canal as in the previous
and horizontal canals, as well as the dark experiment, and it is surprising that postro¬
cell areas of these structures. The dark cell tatory nystagmus occurred in spite of this
areas of the utricle and ampullae contain condition.
cells which are cytologically compatible with Dependency of Nystagmus on Direction
secretory function.14 The utricular otolithic of Gaze.—Bárány1 first noted that when the
membranes were present but degenerated in eyes were directed toward the uppermost ear
two and were entirely missing in two (Fig in the provocative test position the nystag-

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 Fig 7.—Degeneration of the utricular macula (U) and section of the anterior vestibular artery. Otoconia have
its otolithic membrane and the crista of the horizontal separated from the gelatinous structure of the otolithic
semicircular canal (C) of the cat, one month following membrane. Inset shows high-power view of otoconia.

Fig 8.—Collapse of the ampullary wall of the anterior vestibular artery in the cat. Movement
posterior semicircular canal following cutting of of the cúpula (C) is impaired. The margin of the
the superior division of the vestibular nerve and crista neglecta (N) is seen at the left.

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 mus became purely vertical. Animal experi¬ force come into contact with the cúpula of
ments by Szentagothai15 have shown that the posterior semicircular canal. Presumably
ampullofugal flow in the posterior canal re¬ these particles may exist free in the endo¬
sults in contraction of the ipsilateral superi¬ lymph or may become attached to the cúpu¬
or oblique and contralateral inferior rectus la. With the head in the erect position the
muscles, and ampullopetal flow results in posterior canal ampulla is located inferiorly,
inhibition in these same muscles. Fluur16 whereas in the provocative test position
confirmed these relationships by directly (supine, head-hanging, ear-down) the pos¬
stimulating the ampullary nerves of cats. On terior canal assumes a superior position.
the basis of this information and the direc¬ The change in position from erect to su¬
tion of pull of the ipsilateral superior ob¬ pine-head hanging-ear down apparently re¬
lique and the contralateral inferior rectus sults in a severe ampullofugal displacement
muscles in different positions of gaze, of the cúpula of the posterior canal.
Harbert17 has demonstrated that the nystag¬ The delay of onset, which is usually sever¬
mus characteristic of positional vertigo of al seconds, may be due to the period of time
the paroxysmal type is the result of activity required to get the mass in motion; the
of the posterior semicircular canal. Further¬ severity of the sensation and nystagmus
more, he has postulated that the direction of may be due to the great magnitude of the
flow is ampullofugal in the posterior canal cupular displacement; the limited duration
being stimulated. of the vertiginous attack may be due to the
Intense Mechanical Stimulation.—It was return of the cúpula to a normal position
first shown by Wittmaack18 in 1909 and later after the particles have left it; the fatigabili-
by Hasegawa19 in 1931 and de Kleyn and ty may be due to dispersement of particles
Versteegh20 in 1933 that intense linear accel¬ in the endolymph of the pars superior occur¬
eration by centrifugation results in removal ring during repeated head positionings; and
of the otoconia and the gelatinous layer. the repeatability after rest may be due to
Parker et al21 demonstrated in an experiment the time required for the gravity sensitive
on guinea pigs that moderate loss of otocon¬ particles to again settle into the posterior
ia from the maculae may be observed follow¬ canal ampulla so that they can again act en
ing exposure to 12 to 25 g for 195 to 330 masse when the pars superior is turned over.
seconds. Moderate to severe displacement of
otoconia resulted from acceleration expo¬ Management
sures of approximately 50 g for one minute
and accelerations of 100 g for 30 seconds Establishing the Diagnosis.—It is com¬
and of over 100 g for 15 to 20 seconds mon for the patient suffering from positional
produced severe otoconia loss from all macu¬ vertigo of the paroxysmal type to have had
lae. They also demonstrated slight to moder¬ extensive diagnostic laboratory tests in a
ate otoconia displacements following impact well-meant search for the cause of the symp¬
decelerations of 240 to 314 g. Parker et al21 toms. The physician who is aware of the
stated that the results of their experiments existence of this disorder will, however, sus¬
"support the hypothesis that 'benign parox¬ pect its presence when recording the history
ysmal positional nystagmus' results from and can readily establish the diagnosis by
displacement of otoconia into the canal am¬ provocative positional testing. Some pa¬
pullae." tients are reluctant to submit to the test
experience because of the anticipated dis¬
Pathophysiologic Mechanism comfort which, in exceptional cases, includes
nausea and vomiting. Some will require one
The concept of cupulolithiasis provides a hour or more of rest before full recovery
reasonable explanation for most of the clini¬ occurs. All of the characteristic features of
cal features of positional vertigo of the par¬ the attack must be observed: that is, delay
oxysmal type. It assumes that substances of onset, rotatory nystagmus, limited dura¬
having a specific gravity greater than endo¬ tion, intense subjective sensation, reversal of
lymph and thus subject to movement with nystagmus on sitting up, and fatigability on
changes in the direction of gravitational repeat testing.

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 Having observed the typical response to with a set of self-imposed restrictions, so
positional testing, the possible etiologic fac¬ that detailed counselling is unnecessary. A
tors should be considered. These have been further responsibility of the physician is to
listed previously as (1) degenerative change render a prognosis regarding the future
of the utricle; (2) labyrinthine concussion; course of the disorder. The prediction as to
(3) otitis media; (4) ear surgery; and (5) whether the disorder is self-limited, recur¬
occlusion of the anterior vestibular artery. rent, or persistent depends somewhat on the
The examination should include routine etiology. Experience has shown that when
audiometrie and vestibular fundion tests. positional vertigo follows head injury or ear
Ordinarily it is not necessary to acquire surgery it tends to subside within a few
Bekesy audiometrie tracings, electronystag¬ weeks or months, but when due to ear infec¬
mographic recordings, or roentgen films of tion, degeneration of aging, or to vascular
the skull. The reasoning used in attempting occlusion it tends to be more persistent. The
to define an etiology, based on our present precise symptoms and course probably are
state of knowledge, is simply this: if there is determined in a large degree by the physical
no evidence of related head trauma, ear characteristics of the cupular deposit—for
infection, surgical procedure, or the syn¬ example, the mass, specific gravity, solubili¬
drome characteristic of occlusion of the an¬ ty, and whether it is free or fixed to the
terior vestibular artery, the etiology is as¬ cúpula. Thus, the prognosis regarding dura¬
sumed to be spontaneous degeneration of tion should be guarded.
the utricle. Ablating Vestibular Function.—In rare
Counselling the Patient.—Patients are of¬ cases the disability may be sufficiently se¬
ten alarmed by the attacks, fear the worst, vere to justify consideration of some form of
and are not relieved of their anxieties until ablation of the vestibular labyrinth. Al¬
a reasonable explanation is offered. The im¬
though theoretically it should be adequate
mediate, deliberate, and methodical execu¬ to ablate function of only the posterior semi¬
tion of the provocative test is usually circular canal, this has not yet become tech¬
effective in gaining their confidence. The nically feasible. The following pertinent case
question as to how much information re¬ histories demonstrate the consistent success
garding the etiology and mechanism is to be of labyrinth ablation in the management of
offered is an individual consideration with severe positional vertigo of the paroxysmal
each patient. Above all, the patient must be type. In each case total ablation was per¬
reassured that the disorder, regardless of formed in the ear which was undermost in
how disturbing it may be, is essentially be¬ the provocative position. All of these pa¬
nign. tients experienced severe sensorineural hear¬
The most rational and most simple man¬ ing loss in the involved ear so that total
agement of the disorder is avoidance of the inner ear ablation was permissible.
provocative position, and most patients ac¬ Case 3.—Reported by Citron and Hallpike
complish this with little restriction of nor¬ (1956)22.—A woman, aged 50, experienced a
mal everyday activities. The extent to which sudden, prolonged vestibular upset and severe
activities must be restricted is determined left-sided sensorineural-type hearing loss in
by the characteristics of the attack, such as 1949, followed by positional vertigo with the
suddenness of onset, severity of disequili¬ left ear undermost. Caloric responses were ab¬
sent on the left side. A left-sided labyrinth
brium, associated nausea and vomiting, and destruction was performed in 1953 by a surgical
frequency of occurrence. Climbing, swim¬ procedure through the horizontal canal. The
ming, and athletic activities such as skiing patient was completely relieved of positional
may be contraindicated. The operation of vertigo. (Follow-up time was not given.)
mechanical equipment such as driving an Case 4.—Reported by Citron and Hallpike
automobile or tending industrial machinery (1956)22.—A woman, aged 60, had a left-sided
may have to be curtailed. The extent of radical mastoidectomy for chronic otorrhea in
occupational handicap will be determined 1953 and afterward experienced positional ver¬
by the requirements for motor activity, in tigo in assuming the supine position with the
particular for head movement. Most pa¬ left ear undermost. Caloric responses were ac¬
tients will have adjusted to the disorder tive but there was severe sensorineural hearing

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 loss in the left ear. In 1955, a left labyrinth Comment
destruction was performed by subluxating the
stapes and injecting 95% alcohol solution into When usable hearing is present a tech¬
the vestibule. Positional vertigo could not be nique is required which will selectively
elicited following ablation. (Follow-up period ablate the vestibular labyrinth. Among the
was three months.)
methods which have been used with varying
Case 5.—Reported by Citron and Hallpike
(1962)23._A woman, at the age of 31, in 1937, degrees of success are the following: (1)
experienced a severe left-sided head injury and selective section of the vestibular nerve root;
was unconscious for three days. In 1956, at the
(2) ultrasound application; (3) cryosurgery;
age of 50, she developed positional vertigo.
and (4) streptomycin sulfate treatment.
When tested at the age of 51 she demonstrated
positional vertigo with the head in the supine Summary
position and the left ear undermost. A moder¬
ately severe left-sided sensorineural hearing Cupulolithiasis is a peripheral vestibular
loss was present and caloric responses were disorder characterized by positional vertigo
greatly reduced on the left side. The left eighth of the paroxysmal type. The diagnosis is
cranial nerve was sectioned with an approach
confirmed by the positional test procedure
through the posterior cranial fossa in January which provokes a vertiginous attack with
1961, following which positional vertigo could
not be elicited. (Follow-up period was not giv¬ characteristic nystagmus. The features of
en.) the attack which confirm the diagnosis of
Case 6.—Reported by Schuknecht (1962)4.— cupulolithiasis and differentiate it from posi¬
A woman at the age of 72, in 1952, experienced tional vertigo due to lesions of the central
sudden partial loss of hearing in the left ear. nervous system are (1) delay of onset; (2)
Five years later (1956) at the age of 77 she limited duration; (3) severe subjective sen¬
developed short, severe attacks of vertigo occur¬ sation; (4) rotatory nystagmus; and (5)
ring when the head was in certain positions, fatigability. Histological study of the tem¬
particularly in the supine position with the poral bones from two patients provide evi¬
head turned so that the right ear was under¬ dence that the disorder is caused by the
most. Two attacks were characterized by a deposition of a substance on the cúpula of
sudden sensation of falling backward and, on the posterior semicircular canal. It is pre¬
one occasion, she fell, hitting her head. She had sumed that this substance is of high specific
experienced a progressive loss of hearing in her gravity and renders this organ gravity-sensi¬
right ear for many years. tive and subject to stimulation with changes
Audiometrie tests revealed bilateral sensori¬ in head position. It seems probable that in
neural hearing loss, profound on the right and most cases the
deposit takes origin from the
moderate on the left. There was no sponta¬ otoconia of the utricle.
neous nystagmus. Caloric responses were equal
Etiologic factors ap¬
pear to be (1) spontaneous degeneration of
and normal—that is, 70-second responses to 5 the
utricular otolithic membrane; (2) laby¬
cc of water at 80 F, observing the nystagmus
through illuminated Frenzel glasses in a dark¬ ear
rinthine concussion; (3) otitis media; (4)
surgery; and (5) occlusion of the ante¬
ened room. Positional tests revealed severe nys¬
tagmus and vertigo in the right-ear-down posi¬ rior vestibular artery. It has been demon¬
tion, characterized by onset delay of five to ten
strated that the diseased ear is located un¬
seconds, with duration of six seconds and fatig- dermost in the provocative head position.
able on repeated testing. Nystagmus was of the This unique pathophysiological mechanism
combined counterclockwise rotatory and right for positional vertigo is supported by animal
horizontal type and was associated with a vio¬ experiments which show that (1) utricular
lent sensation of turning. degeneration releases otoconia into the en¬
The right labyrinth was destroyed by a surgi¬ dolymph; and (2) stimulation of the poste¬
cal procedure through the oval window. The rior semicircular canal creates a purely rota¬
postoperative vestibular upset was less severe tory nystagmus on forward gaze. For most
than anticipated. Postural tests on the fourth patients the most rational management is
postoperative day, and subsequently, failed to avoidance of the provocative position. When
elicit vertigo or nystagmus (Follow-up period the disorder seriously restricts the activities
was ten years.) of the patient, some form of labyrinth abla-

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 tion may be performed. Among the methods osurgical application to the vestibular laby¬
in current use are total labyrinthectomy, rinth, and vestibular ablation with strepto¬
vestibular nerve section, ultrasound or cry- mycin.
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