FFinalistSouthampton · 2027
ENT
AKT · ENT/Ear & hearinglow yield

Otosclerosis

Abnormal bone remodelling fixing the stapes footplate

Overview

A hereditary disorder of abnormal bony remodelling around the stapes footplate, fixing it in the oval window and causing a progressive CONDUCTIVE hearing loss in young adults. Often bilateral, worse in pregnancy, with a positive family history.

Recognise

  • Progressive bilateral conductive hearing loss in a young adult (20s–30s), often with a family history
  • Paradoxical improvement in noisy surroundings (paracusis of Willis); tinnitus
  • Worsens during pregnancy; a normal-looking tympanic membrane (occasionally a pink "Schwartze sign" flush)

Red flags

  • Mixed/sensorineural component (cochlear involvement) affects management; exclude other conductive causes

Differentials & how to tell them apart

Otitis media with effusioneffusion + flat tympanogram — otosclerosis has a normal drum and normal tympanometry
Cholesteatoma/ossicular discontinuitydischarge or trauma history, otoscopic abnormality
Presbycusisage-related SENSORINEURAL loss, not conductive

Investigations

Tuning-fork tests (Rinne NEGATIVE on the affected side = conductive; Weber lateralises to the worse ear) + pure-tone audiometry showing conductive loss with a "Carhart notch"; normal tympanometry. ENT referral.

Management

Hearing aid or stapes surgery (stapedotomy) for the conductive loss

  1. 1Confirm conductive loss (Rinne negative, audiometry). Offer a hearing aid as an effective non-surgical option.Gate: Otosclerosis is a CONDUCTIVE loss with a NORMAL drum and NORMAL tympanometry — that combination (normal otoscopy + conductive audiogram + young adult + family history) separates it from effusion or ossicular pathology and points to stapes fixation
  2. 2Surgical option: stapedectomy/stapedotomy (replace the fixed stapes with a prosthesis) for suitable patients; cochlear implant for advanced mixed loss.
Hearing aid — first-line non-surgical option — effective for the conductive loss
Stapedectomy/stapedotomy — surgical replacement of the fixed stapes — definitive for suitable patients
(Sodium fluoride) — historically used to slow progression — limited role

Key points

Young adult + progressive bilateral conductive loss + normal drum + family history (worse in pregnancy) = otosclerosis. Rinne negative with a normal otoscopy is the bedside clue.

Monitor & prognosis

Audiometry over time; surgical/hearing-aid outcomes.

Progressive but well managed by aids or surgery.

Source: ENT UK; StatPearls