Definition
Primary metabolic bone disease of the otic capsule and ossicles, causing abnormal bone remodeling and fixation of the ossicles, leading to conductive hearing loss.
Epidemiology
- Mostly affects Caucasians
- Family history in 60%
- Bilateral in 80%
- Female to male ratio is 2:1
- Common in ages 20-40 years
Etiology
- Autosomal Dominant with Incomplete Penetrance
- Hormonal factors (e.g., pregnancy)
- Viral factors
Presentation
- Slowly progressive hearing loss
- Tinnitus
- OE: Schwartze sign
- Audiology: PTA, Tympanometry
Management
- Observation
- Trial of Amplification
- Medical Treatment
- Surgery: Stapedectomy
Stapedectomy operation:
Carharts notch
