OTOSCLEROSIS

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.

 Examinations – general/ otologic.

 Investigation – see signs section/Radiological-

CT scan, MRI.

 Treatment either Medical or Surgical.

 MEDICAL TREATMENT
 NO MEDICAL TREATMENT IS CURATIVE FOR
OTOSCLEROSIS.

 Sodium Fluoride has been tried in the past.

 It hastens the maturity of active focus and

arrests further cochlear loss.
 SURGICAL TREATMENT.
 STAPEDECTOMY with prosthetic replacement
is the treatment of choice.

 Prosthesis used includes;

Teflon piston.
Stainless steel piston.
Platinum Teflon.
Titanium Teflon piston.
SELECTION OF PATIENTS FOR SURGERY

 Hearing threshold should be 30dB or worse.

 Average Air-Bone Gap (ABG) should be at least

15dB with negative Rinne test.

 Speech discrimination score should be 60% or

more.
 CONTRAINDICATION FOR SURGERY
 Only hearing ear.
 Associated Meniere’s disease.
 Young children.
 Professional athletics, high construction
workers, divers and frequent air travelers.
 Those that work in noisy environments.
 Ear infections e.g. otitis externa, tympanic
membrane perforation, exostosis.
 HEARING AUGUMENTATION
 HEARING AIDS can be used for those patients;
 That refuses surgery.
 That are unfit for surgery.
 That are elderly.
 COMPLICATIONS.
 Hearing loss.
 Loss of job.
 Epilepsy after surgery.
 Chronic vertigo.
 Persistent tinnitus.