Cholesteatoma
Exam Tips
- In OSCEs, unilateral foul-smelling persistent otorrhoea plus progressive conductive hearing loss should trigger cholesteatoma until proven otherwise.
- A retraction pocket with keratin debris in the attic/posterosuperior drum is a classic otoscopic clue (see standard otoscopy figure of attic cholesteatoma).
- Differentiate residual (incomplete initial clearance) from recurrent (new retraction pocket after prior complete excision) disease.
- State red flags explicitly: facial weakness, vertigo, severe headache/fever, meningism, or neurological signs require urgent specialist/hospital assessment.
- Definitive management is surgical; medical treatment is supportive or for superimposed infection and must not delay ENT referral.
Definition
Cholesteatoma is an abnormal collection of keratinizing squamous epithelium within the middle ear and/or mastoid that behaves in a locally destructive way despite being histologically benign. It gradually accumulates keratin debris, can become chronically infected, and may erode ossicles, labyrinth, facial nerve canal, or skull base if not treated.
Pathophysiology
Most cases are acquired and arise from tympanic membrane retraction (pars flaccida or pars tensa) due to chronic Eustachian tube dysfunction and poor middle-ear ventilation. A retraction pocket with a narrow neck loses normal epithelial self-cleaning, trapping keratin that expands the sac; secondary acquired disease can also follow marginal perforation, infection, trauma, or iatrogenic implantation of squamous epithelium. Bone destruction is driven by pressure plus chronic inflammation (including osteoclast-activating mediators), causing ossicular erosion and potential spread to inner ear or intracranial spaces. Congenital cholesteatoma is rarer and reflects embryologically trapped epithelium behind an intact tympanic membrane.
Risk Factors
- Male sex (slight predominance)
- Recurrent otitis media and chronic middle-ear inflammation
- Eustachian tube dysfunction (including otitis media with effusion history)
- Previous ENT surgery (for example tympanoplasty) or ear trauma/blast injury
- Cleft palate and craniofacial anomalies
- Genetic syndromes linked to Eustachian dysfunction (for example Down syndrome, Turner syndrome)
Clinical Features
Symptoms
- Persistent or recurrent unilateral, scanty, foul-smelling otorrhoea not settling with standard treatment
- Progressive conductive hearing loss
- Tinnitus
- Otalgia (often suggests more advanced/inflamed disease)
- Vertigo or imbalance (possible labyrinthine involvement)
- Facial weakness (red flag for facial nerve involvement)
Signs
- Retraction pocket in attic or posterosuperior tympanic membrane with white/yellow keratin debris
- Marginal or attic tympanic membrane perforation adjacent to crust/debris
- A white or yellowish retro-tympanic mass
- Purulent debris in external canal; may obscure full tympanic membrane view
- Visible bony erosion in advanced disease
- Congenital pattern: spherical white mass behind intact tympanic membrane, often anterosuperiorly (see typical otoscopy figure of congenital cholesteatoma)
Investigations
Management
Lifestyle Modifications
- Urgent ENT assessment for suspected cholesteatoma; do not rely on repeated empirical antibiotic courses when discharge persists
- Keep ear dry (water precautions), avoid self-cleaning/instrumentation, and seek review promptly if worsening pain, vertigo, facial weakness, or headache/fever develop
- Safety-net for intracranial and mastoid complications, especially in children where disease may be more aggressive
Pharmacological Treatment
Analgesia
- Paracetamol 1 g orally every 4-6 hours when required (max 4 g/day in adults)
- Ibuprofen 200-400 mg orally three times daily with food when required (max 1.2 g/day OTC; higher only if prescribed)
Symptom control only; does not treat cholesteatoma. Avoid/limit NSAIDs in peptic ulcer disease, significant renal impairment, heart failure, anticoagulant use, or NSAID-sensitive asthma.
Treatment of concurrent acute ear infection while awaiting specialist review
- Amoxicillin 500 mg orally three times daily for 5 days (adult first-line when bacterial acute otitis media is judged likely and antibiotics are indicated)
- Clarithromycin 500 mg orally twice daily for 5 days if true penicillin allergy
Use only when there is a clear superimposed acute infection; persistent unilateral otorrhoea still requires ENT referral. Check allergy history and drug interactions (notably macrolides).
Topical therapy safety point
- Avoid aminoglycoside-containing ear drops (for example neomycin/gentamicin preparations) when tympanic membrane perforation is known or suspected unless specialist-directed
Risk of ototoxicity with middle-ear exposure; choose non-ototoxic options under ENT guidance.
Surgical / Interventional
- Definitive treatment is surgery (for example tympanomastoid surgery such as canal wall up or canal wall down mastoidectomy, with tympanoplasty as required)
- Aim is complete disease clearance, infection control, and hearing preservation/reconstruction where feasible (ossiculoplasty when appropriate)
- Planned long-term postoperative surveillance, often including interval clinical review and diffusion-weighted MRI to detect residual/recurrent disease
Complications
- Recurrent chronic ear infection
- Conductive hearing loss from ossicular erosion or mass effect
- Labyrinthine/perilymph fistula
- Vertigo from semicircular canal erosion
- Facial nerve palsy due to temporal bone/facial canal erosion
- Profound sensorineural hearing loss from inner-ear involvement
- Mastoiditis
- Meningitis
- Intracranial abscess (middle or posterior cranial fossa extension)
- Cavernous sinus thrombosis (rare)
Prognosis
Cholesteatoma is treatable but recurrence risk is significant, so prolonged follow-up is essential after surgery. Residual disease relates to incomplete clearance, while recurrent disease reflects new retraction-pocket formation; published series report substantial long-term recurrence (including high 10-year rates), with children generally showing more aggressive behavior and less favorable outcomes than adults.
Sources & References
🏥BMJ Best Practice(3)
✅NICE Guidelines(1)
- Cholesteatoma[overview]