Cholesteatoma
Cholesteatoma is an abnormal growth of keratinising squamous epithelium within the middle ear and/or mastoid air spaces, which may progressively destroy adjacent structures.
This updated UKMLA guide to cholesteatoma is based on NICE CKS, which covers causes, risk factors, symptoms, complications, diagnosis and management.
Causes and Risk Factors
The pathophysiology of cholesteatoma is not completely understood. Cholesteatoma is usually acquired but can rarely be congenital.
Risk factors include:
- Males
- ENT surgeries (e.g. tympanoplasty)
- History of middle ear disease (e.g. otitis media with effusion, recurrent otitis media)
- Anomalies associated with Eustachian tube dysfunction (e.g. cleft palate, Turner syndrome, Down syndrome, craniofacial abnormalities)
Clinical Features
Cholesteatoma may be asymptomatic or present with mild or intermittent symptoms in its early stages
Classically presents as recurrent / persistent unilateral, foul-smelling, purulent ear discharge
- Typically scanty
- Often mistaken for recurrent otitis media / externa – but does NOT resolve with antibiotics
Typical otoscopic findings:
- A round, white or yellowish mass behind the tympanic membrane
- Presence of a retraction pocket of the tympanic membrane
- Accumulation of keratin debris / crust (white / yellow) within the pocket +/- surrounding pus
- Possible tympanic membrane perforation adjacent to the crust
- Possible visible surrounding bone erosion
Congenital cholesteatoma (rare) may appear as a spherical white mass behind an intact tympanic membrane, and typically has no ear discharge or predisposing features.
Complications
Untreated cholesteatoma may progressively erode adjacent structures, leading to:
- Conductive hearing loss (commonly due to ossicular erosion)
- Recurrent/chronic ear infections
- Facial nerve palsy
- Mastoiditis
- Vertigo (inner ear involvement)
- Sensorineural hearing loss (less common – suggests inner ear involvement)
Intracranial complications are rare but serious:
- Meningitis
- Intracranial abscess
- Cavernous sinus thrombosis
Assessment and Management
All suspected cases should be referred to ENT
- CT temporal bone is necessary to assess the extent of disease
- Definitive management: surgical resection (mastoidectomy may be needed for extensive disease)