Growth consisting of keratinising squamous epithelium in the middle ear and/or mastoid process; non-cancerous but destructive and expanding

Aetiology

• Common, can occur in any age group
• Can be acquired or congenital
  • Acquired (most common) - caused by chronic otitis media or perforated tympanic membrane
  • Congenital - proliferation of embryonic rest
• History of frequent ear surgery is a key risk factor

Pathophysiology

• The keratin becomes trapped and builds
• As it expands, it erodes surrounding bone
• Histology: squamous epithelium with abundant keratin production, associated inflammation

Clinical features

• May be asymptomatic in its early stages
• Unilateral discharge - persistent or recurrent discharge, often foul-smelling
• Associated conductive hearing loss may also occur
• Rarely with progression of the disease, vertigo, sensorineural hearing loss, facial nerve palsy, meningitis, or intracranial abscess may develop

Investigations

• Otoscopy:
  • Retraction visible before development of cholesteatoma
  • Examination show a defect in the tympanic membrane full of cheesy, white material

Retraction

Cholesteatoma

Management

• Mastoid surgery to remove the sac of debris, reconstruction