Cholesteatoma

Risk factors

• Chronic eustachian tube dysfunction
• Recurrent or chronic suppurative otitis media
• 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

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 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]

Treatment

Complications

• Ossicular erosion with conductive or mixed hearing loss
• Labyrinthine fistula with sensorineural hearing loss and vertigo
• Facial nerve paresis or paralysis
• Meningitis, epidural abscess, brain abscess, sigmoid sinus thrombosis
• Recurrent or residual disease after surgery (10-25%, higher in pediatric cases)

PANCE pearls

• Any chronic ear with foul otorrhea unresponsive to antibiotics is cholesteatoma until proven otherwise.
• Attic retraction pockets are the earliest visible sign; refer for otologic evaluation.
• DWI MRI is the surveillance modality of choice to detect recurrence without re-operating.
• A positive fistula test (vertigo with pneumatic otoscopy) is a red flag for labyrinthine erosion.
• Children have more aggressive disease with higher recurrence — meticulous follow-up is essential.

References

• AAO-HNS — American Academy of Otolaryngology-Head and Neck Surgery: Cholesteatoma clinical resources
• EAONO/JOS — EAONO/JOS Joint Consensus on the definitions, classification, and staging of middle ear cholesteatoma (Yung et al., J Int Adv Otol 2017)
• Cummings Otolaryngology — Cummings Otolaryngology - Head and Neck Surgery, chapter on chronic otitis media and cholesteatoma