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Cholesteatoma

Cholesteatoma is keratin-producing squamous epithelium in the wrong place — the middle ear and mastoid — that grows while destroying bone through enzymes and cytokines; treatment is surgical only, and follow-up afterwards (a "second-look" operation or diffusion-weighted MRI) is part of the treatment.

Why it matters in the exam

The exam asks about pathogenesis and types. Congenital cholesteatoma is a white mass behind an intact eardrum, usually in the anterosuperior quadrant, in a child without a history of infections or surgery. Primary acquired cholesteatoma arises from a retraction pocket, usually in the pars flaccida, into Prussak space — bounded laterally by the pars flaccida, medially by the neck of the malleus, and below by the short process of the malleus. Secondary acquired cholesteatoma arises from epithelial migration through a marginal perforation or from implantation during surgery. The theories: invagination, migration, metaplasia and basal cell hyperplasia.

Bone destruction comes from osteoclasts activated by cytokines such as RANKL, TNF-α and IL-1. The bone most often destroyed first is the long process of the incus, whose blood supply is poor. Complications: fistula of the lateral semicircular canal (vertigo with pressure on the ear — fistula test), facial nerve palsy, sensorineural hearing loss and intracranial complications. The typical complaint: chronic foul-smelling ear discharge that does not respond to drops, with conductive hearing loss.

CT of the temporal bone shows the extent of destruction and the structures at risk; non-echo-planar diffusion-weighted MRI (non-EPI DWI) detects residual or recurrent cholesteatoma. In a canal wall up operation the posterior canal wall is kept, so a second-look operation after 6 to 12 months or diffusion-weighted MRI is needed; a canal wall down operation leaves an open cavity that needs regular cleaning for life, but recurrence is lower.

Key points

Summary table

TypeClueTreatment and follow-up
CongenitalWhite mass behind an intact eardrum, anterosuperior quadrantSurgical removal
Primary acquiredRetraction pocket in the pars flaccida, Prussak spaceMastoidectomy and eardrum repair
Secondary acquiredMarginal perforation or after surgerySurgical removal
Canal wall upCanal wall keptSecond look after 6 to 12 months or non-EPI DWI MRI
Canal wall downOpen cavityRegular cleaning for life, less recurrence

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Sources

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Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.