Step 1 of 17
Clinical Suspicion & History
Recurrent foul otorrhoea · hearing loss · attic crust · retraction pocket · vertigo · facial weakness
Cholesteatoma is suspected from a focused history before the ear is even examined. Six clinical clusters trigger the pathway: recurrent foul-smelling otorrhoea refractory to antibiotics, progressive conductive hearing loss, an attic crust or visible retraction pocket on prior examination, otogenic vertigo or imbalance, peripheral facial weakness, or a history of multiple ear infections in childhood.
Beyond the symptoms themselves, the history must capture modifiers that change the diagnostic and surgical plan: duration of otorrhoea, hearing-loss tempo, prior ear surgery (revision disease behaves differently), trauma or myringotomy that could have implanted epithelium, sinonasal disease and eustachian-tube symptoms, and paediatric risk factors (cleft palate, craniofacial syndromes, Down syndrome).
- Duration and character of otorrhoea (chronic, foul, refractory)
- Hearing-loss progression — conductive vs mixed vs sensorineural
- Prior ear surgery (tympanoplasty, mastoidectomy, ventilation tubes)
- Trauma / myringotomy history (implantation risk)
- Vertigo / imbalance — fistula sign, labyrinthine erosion
- Facial weakness — facial nerve canal erosion
- Paediatric risk — cleft palate, Down syndrome, craniofacial anomalies
- Sinonasal / eustachian-tube dysfunction symptoms
★ High-yield pearls (chapter-wide)
- Six clinical clusters trigger the pathway — foul otorrhoea, hearing loss, attic crust, retraction, vertigo, facial weakness.
- Screen for complications before any elective workup — extracranial and intracranial.
- Otoendoscopy maps the hidden recesses microscopy cannot reach — sinus tympani, facial recess, anterior epitympanum, supratubal recess, hypotympanum, protympanum.
- Aetiology informs behaviour — congenital, primary acquired, secondary acquired, implantation.
- ET dysfunction is half the disease — address it explicitly, especially in paediatric and recurrent cases.
- HRCT temporal bone is the surgical map; non-EPI DWI MRI is the modern surveillance tool; contrast MRI is for complications.
- Limited attic / mesotympanic → endoscopic. Extensive mastoid → tympanomastoidectomy.
- CWU vs CWD — limited disease + good ET + reliable follow-up favours CWU; extensive disease, fistula, poor ET, or poor follow-up favours CWD.
- Labyrinthine fistula — small repair carefully; large preserve matrix and CWD.
- Paediatric disease is more aggressive — lower threshold for staging and surveillance.
- Stage surgery for piecemeal removal, doubtful clearance, or high adhesion risk.
- DWI MRI has replaced obligatory second-look in confident, reliable adult cases.
- Residual disease is surgical-technique driven; recurrent disease is ET-dysfunction driven.
- Final goals: safe ear, dry ear, complete eradication, hearing preserved or restored, facial nerve safe, intracranial complications prevented, lifelong surveillance.
Evidence base
6 sources- HIGH
Yung M et al. · J Int Adv Otol · 2017EAONO/JOS international consensusPMID 28059056
International expert consensus defining modern cholesteatoma terminology, classification, staging (STAM system), and reporting standards. Widely accepted framework for clinical diagnosis and disease categorisation. Expert consensus; Level V evidence. Vol 13(1):1-8.
- HIGH
Olszewska E et al. · Eur Arch Otorhinolaryngol · 2004Comprehensive pathogenesis reviewPMID 12835944
Comprehensive review synthesising the major theories of cholesteatoma pathogenesis — retraction-pocket formation, epithelial migration, basal-cell hyperplasia, inflammatory mechanisms. Foundational reference for disease evolution and clinical suspicion. Narrative review; Level V evidence. Vol 261(1):6-24.
- MOD
Sadé J, Fuchs C, Luntz M · Arch Otolaryngol Head Neck Surg · 1997Sadé retraction-pocket theoryPMID 9193217
Observational anatomical study supporting the pars flaccida retraction-pocket theory and its association with mastoid pneumatisation and eustachian-tube dysfunction in acquired cholesteatoma. Anatomical-physiological study; Level IV evidence. Vol 123(6):584-588.
- HIGH
Levenson MJ, Michaels L, Parisier SC · Laryngoscope · 1988Levenson-Michaels-Parisier congenital cholesteatoma seriesPMID 3412093
Classic clinicopathologic study describing congenital cholesteatoma presentation, retrotympanic white masses, intact tympanic membrane findings, and embryologic origin. Clinicopathologic observational study; Level IV evidence. Vol 98(9):949-955.
- HIGH
Nevoux J et al. · Eur Ann Otorhinolaryngol Head Neck Dis · 2010Paediatric cholesteatoma comprehensive reviewPMID 20860924
Comprehensive paediatric cholesteatoma review discussing aggressive clinical behaviour, delayed diagnosis, recurrence tendencies, and characteristic paediatric presentation patterns. Comprehensive review; Level V evidence. Vol 127(4):143-150.
- MODClassification of retraction pockets in the pars tensa and pars flaccida
Charachon R et al. · Adv Otorhinolaryngol · 1999Retraction-pocket classification
Clinical classification study describing progression patterns of pars flaccida and pars tensa retraction pockets and their role in cholesteatoma development. Clinical observational study; Level IV evidence. Vol 55:190-193.