Cholesteatoma

NB Cholesteatoma is NOT a neoplasm, but a destructive invasion of normal squamous epithelium that is trapped within a bony structure (most commonly the skull base) causing erosion of the bone.

Aetiology

Cholesteatoma can be congenital, primary acquired (tympanic membrane retraction- not common) or secondary acquired (after a tympanic membrane perforation e.g. following AOM as a complication of CSOM).

Presentation

Classically with painless otorrhea and/or recurrent bouts of AOM that are difficult to treat.  Hearing loss may be present and severe as the growth fills the middle ear space and damages the ossicles.  Foul smelling discharge can occur with the build up of debris and desquamated epithelium.

Unfortunately, patients frequently present with vertigo, facial nerve palsy or headache, which may indicate a labyrinthine fistula or intracranial invasion to affect CN VII, CN VIII or the brain itself- causing a brain abscess or meningitis.

On examination, a perforated tympanic membrane is almost always present and the canal may be filled with mucupurulent debris and/or granulation tissue.

Investigations

CT scanning is the main investigation, as it can visualise the lesion and detail the degree of invasion/involvement more often than not; and will help plan surgery.

Management

Surgical removal and mastoidectomy is often required.

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