O To Sclerosis
O To Sclerosis
O To Sclerosis
OUTLINE
Introduction.
Aetiology.
Types of Otosclerosis.
Signs/symptoms.
Differentials.
Management
History.
Examination.
Investigation.
Treatment –Surgical/Medical.
Summary/Conclusion.
INTRODUCTION
Also called otospongiosis.
Is a primary disease of the bony labyrinth.
One or more foci of irregularly laid spongy
bone replace part of the normally dense
enchondrial layer of otic capsule.
Often involves the stapes region leading to
fixation of the stapes.
However it may also involve other areas of the
bony labyrinth leading to sensorineural
hearing loss on NO symptoms.
AETIOLOGY
Exact cause is not known.
Associated Factors;
Anatomical basis;
The bony labyrinth is composed of enchondrial bone
which is subject to slight changes in life.
Fissula ante fenestram lying in front of the oval
window is the site of predilection for stapedial
Otosclerosis.
Heredity.
50% of patients have positive family history of similar
illness.
Genetically it is an autosomal dorminant trait disease
with incomplete penetrance and variable expressibility.
Race.
The disease is commoner among the negroes.
Sex.
Females are affected twice more than males.
Age at onset.
Occurs between the ages of 20 – 30years.
Rare before 10years and after 40years.
Infection.
Related to RNA measles virus.
Effects of other factors.
Hearing loss due to Otosclerosis may be worsened by;
Pregnancy.
Menopause.
Following major ear surgery.
Following RTA.
THE TRAID OF OSTEOGENESIS IMPERFECTA, OTOSCLEROSIS AND
TYPES OF OTSCLEROSIS
STAPEDIAL OTOSCLEROSIS.
Causes fixation of the stapes with resultant conductive
hearing loss.
Lesion are just in front of the oval window in the area
called ‘fisulla ante fenestram’ (area /site of
predilection).
Lesions may also start;
Behind the margin of the stapes (posterior).
Around the margin of the stapes (circumferential)
In the footplate but the annular ligament is free
(biscuit type).
Or may completely obliterate the oval window niche
(obliterative type).
COCHLEAR OTOSCLEROSIS.
Involves the region of the round window or
other areas of the otic capsule.
Causes sensorineural hearing loss due to
release of toxic substances.
HISTOLOGIC OTOSCLEROSIS.
Remains asymptomatic.
Causes either conductive or sensorineural
hearing loss.
SIGNS
Tympanic membrane is normal and mobile.
Sometimes there is a reddish hue seen on the promontory
on otoscopy – Schwartz sign.
Eustachian tube is normal.
Tuning Fork test – Rinne test is negative while Weber
lateralizes to the ear with greater conductive hearing loss.
Pure tone audiometry;
Shows los of air conduction more for lower frequencies.
Bone conduction is normal.
In some cases there is a dip in bone conduction curve
which is maximum at 2000Hz and is called the cahart’s
notch.
The notch disappears after successful surgery.
SYMPTOMS
• Hearing loss – painless and progressive.
• Paracusis willisi – an otoslcreotic patient
hears better in noisy than quiet
environments.
• Tinnitus – commoner in cochlear Otosclerosis.
• Vertigo.
• Delayed speech.
DIFFERENTIAL DIAGNOSIS
Otosclerosis should be differentiated from;
Serous otitis media.
Adhesive otitis media.
Tympanosclerosis.
Attic fixation of the head of the malleus.
Ossicular chain discontinuity.
Congenital stapes fixation – congenital
syphilis.
MANAGEMENT
History.