Hereditary disorder in which new bony deposits occur within the stapes footplate and the cochlear, resulting in gradual onset hearing loss
Aetiology
- Characteristically seen in the 2nd-3rd decade
- More common in women
- Linked to high oestrogen - can be triggered/deteriorates more rapidly in pregancy
- Familial link
Clinical presentation
- Gradual onset hearing loss
Investigations
- Audiometry - conductive or mixed hearing loss
- A classic feature is Carhart's notch at 2KHz

Management
- Hearing aids
- Stapedectomy