Overview
Cholesteatoma describes squamous epithelium cells within the middle ear that grow and expand leading to local bone destruction. Cholesteatoma is benign, however, its expansion can lead to complications from problems with hearing to central nervous system involvement.
Epidemiology
- Around 9 per 100,000 people are affected
- Slightly more common in men than women
Risk Factors
- Otitis media
- Eustachian tube dysfunction
- Ear trauma/surgery
- Down’s syndrome and Turner’s syndrome – due to Eustachian tube dysfunction
Presentation
The classic presentation of cholesteatoma is a conductive hearing loss associated with foul-smelling discharge that does not resolve. Features overall may be:
- Conductive hearing loss – some patients may have normal hearing
- Unresolving malodourous ear discharge despite being given antibiotics
- Crusting/keratin in the attic (upper part of the middle ear) on otoscopy
Differential Diagnoses
Chronic suppurative otitis media
- There is perforation of the tympanic membrane on otoscopy
- There is no crusting/keratin in the attic of the ear
Investigations
All patients
- Pure tone audiogram:
- Usually shows conductive hearing loss
- CT petrous temporal bones:
- Can confirm the diagnosis
- MRI:
- Considered if soft tissue involvement suspected
Management
All patients
- 1st-line: surgical removal – usually done by tympanoplasty
Complications
- Progressive conductive hearing loss
- Cholesteatoma recurrence
- Vertigo – often due to fistulae forming between the labyrinth and cholesteatoma
- Central nervous system involvement e.g. facial nerve palsy, brain abscess
Prognosis
- Up to 10% of patients develop a cholesteatoma in the other ear
- 5-30% of cholesteatomas do not resolve fully with treatment
