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/deteriorate more rapidly in pregnancy
• Familial link

Clinical features

• Gradual onset hearing loss

Investigations

• Audiometry: conductive or mixed hearing loss
  • A classic feature is Carhart's notch at 2KHz

Management

• Hearing amplification through hearing aids, which can be beneficial in managing the hearing loss associated with otosclerosis
• Surgical replacement of the stapes bone through a stapedectomy, which can restore hearing in many cases