II. Definition
- Ankylosis between Stapes footplate and surrounding bone of the inner ear
III. Epidemiology
- Most often presents in middle-aged white women
- Gender predominance: Twice as common in women
- Age at onset: 30-50 years old
-
Family History is present in 50% of cases
- Autosomal Dominant trait, with poor penetrance
IV. Pathophysiology
V. Symptoms
- Gradual onset, painless, bilateral Hearing Loss
- Tinnitus
VI. Signs
- Conductive Hearing Loss or mixed Hearing Loss
- Hearing Loss usually limited to 50 decibels
- Bilateral involvement is most common
- Disproportionately effects low frequencies most commonly
- Able to hear voices in noisy environments
- Normal Tympanic Membrane appearance
VII. Differential Diagnosis
VIII. Management
- Hearing Aid
-
Sodium Fluoride
- May halt or retard progression
- Surgery: Stapedotomy or Stapedectomy
- Restore transmission with middle ear prosthesis
- Improvement in 95% of patients