Overview
Otosclerosis is an autosomal dominant condition in which there is an increased rate of bony turnover leading to fixation of the stapes and the oval window (fenestra ovalis) of the cochlea. It leads to slow and progressive conductive hearing loss.
Epidemiology
- The age of onset is usually 20s or 30s
- More commonly diagnosed in women despite not being sex-linked
Risk Factors
- Family history
- Female sex
Presentation
Most patients have progressive conductive hearing loss and tinnitus. Features are:
- Progressive conductive hearing loss and tinnitus that are usually bilateral
- Family history
- Otoscopy is usually normal
- Lower tones such as deep voices are usually most affected
Investigations
All patients
- Pure-tone audiometry:
- Shows conductive hearing loss, predominantly in lower tones
- CT scans can help with the diagnosis
Management
- 1st-line: hearing aids and consideration of stapedectomy
Complications
- Significant hearing loss
- Total deafness is not common
Prognosis
- Surgical management carries a good prognosis
- If untreated, hearing progressively gets worse
- In 80-90% of people, hearing is improved or even restored after surgery
