Accumulation of keratinizing squamous epithelium in the middle ear or mastoid — locally destructive, classic for chronic foul otorrhea and conductive loss.
Also known as: cholesteatoma, acquired cholesteatoma, congenital cholesteatoma, primary acquired cholesteatoma
Overview
A non-neoplastic, locally destructive accumulation of keratinizing stratified squamous epithelium within the middle ear, attic, or mastoid. Despite the name, it is not a tumor and contains no cholesterol. Classified as congenital (intact tympanic membrane, no prior infection) or acquired (most common; arising from retraction pockets or perforations).
Epidemiology
Acquired form is most common in adults aged 30-50, with a male predominance. Congenital form classically presents in children aged 4-6 as a white retrotympanic mass behind an intact tympanic membrane. Strongly associated with chronic eustachian tube dysfunction.
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Prior tympanic membrane perforation or tympanostomy tube history
Cleft palate and craniofacial anomalies
Trauma to the tympanic membrane
Pathophysiology
Chronic negative middle ear pressure produces a retraction pocket of the pars flaccida (attic) or pars tensa. Squamous epithelium becomes trapped, accumulates keratin, and expands. The keratin matrix produces enzymes (collagenase, osteoclast-activating cytokines) that resorb adjacent bone — leading to ossicular erosion, labyrinthine fistula, and intracranial extension. Secondary bacterial infection (often Pseudomonas, Staphylococcus, anaerobes) causes the characteristic foul otorrhea.
Clinical presentation
Symptoms
Chronic, foul-smelling otorrhea that may persist despite multiple courses of antibiotics
Conductive hearing loss
Aural fullness; occasionally otalgia
Vertigo or imbalance suggests labyrinthine fistula
Facial weakness suggests erosion into the facial canal
Signs / physical exam
Otoscopy: retraction pocket of the pars flaccida (attic) filled with white keratin debris is the classic finding
Marginal or attic perforation with pearly-white debris extruding through the defect
Granulation tissue or polyp obscuring the middle ear
Positive fistula test (vertigo and nystagmus induced by pneumatic otoscopy or tragal pressure) if labyrinthine fistula present
Conductive hearing loss on tuning fork tests
Classic findings
Chronic foul otorrhea unresponsive to antibiotics, conductive hearing loss, and pearly-white debris in an attic retraction pocket.
Differential diagnosis
Chronic suppurative otitis media without cholesteatoma — Persistent perforation with mucoid otorrhea but no keratin debris or retraction pocket; CT shows no mass
Otitis externa — Tenderness on tragal pressure, ear canal edema and discharge, normal middle ear
Tympanosclerosis — Chalky white plaques within the tympanic membrane or middle ear; conductive loss without erosion
Glomus tumor (paraganglioma) — Pulsatile tinnitus, vascular red mass behind tympanic membrane, audible bruit; MRI with contrast
Middle ear adenoma or carcinoma — Rare; persistent mass that fails to resolve; biopsy
Granulation tissue from chronic infection — Friable bleeding tissue in the middle ear; resolves with treatment of infection
Diagnostic workup
Diagnostic criteria
Clinical diagnosis supported by otoscopy and imaging: keratin debris in a retraction pocket or perforation, with CT evidence of soft tissue mass and bony erosion.
Labs
Culture of otorrhea — typically polymicrobial, often Pseudomonas aeruginosa and Staphylococcus aureus
No routine systemic labs
Imaging
High-resolution CT of the temporal bones — defines extent, ossicular and otic capsule erosion, scutum blunting, tegmen integrity, facial canal status; preoperative imaging of choice
MRI with non-echoplanar diffusion-weighted imaging — distinguishes cholesteatoma (bright on DWI) from granulation tissue or fluid; used for postoperative surveillance to detect residual or recurrent disease
Pure tone audiogram to document baseline hearing and air-bone gap
Diagnostic algorithm
flowchart TD
A[Chronic foul otorrhea<br/>+ hearing loss] --> B[Otoscopy]
B --> C{Attic retraction<br/>or perforation<br/>with keratin debris?}
C -->|Yes| D[Suspect cholesteatoma]
D --> E[CT temporal bone<br/>+ audiogram]
E --> F[Topical antibiotic/steroid<br/>aural toilet]
F --> G[Tympanomastoidectomy]
G --> H[Second-look surgery<br/>or DWI-MRI<br/>at 9-12 months]
C -->|No| I[Treat as CSOM<br/>or otitis externa]
Workup and management of suspected cholesteatoma.
Treatment
First-line
Surgical removal is definitive — tympanomastoidectomy (canal wall up or canal wall down) with goals of complete disease eradication, dry safe ear, and hearing preservation when possible
Aural toilet and topical antibiotic-steroid drops (e.g., ciprofloxacin-dexamethasone) to control acute infection prior to surgery
Ossicular reconstruction at the time of primary surgery or as a staged second-look procedure
Second-line / adjunct
Canal wall down mastoidectomy preferred for extensive disease, only-hearing ear, recurrent disease, or labyrinthine fistula
Second-look surgery or follow-up MRI with DWI 9-12 months postoperatively to detect residual disease
Hearing aid or implantable device if hearing cannot be restored surgically
Complications
Ossicular erosion with conductive or mixed hearing loss
Labyrinthine fistula with sensorineural hearing loss and vertigo
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