A cholesteatoma is an abnormal, non-neoplastic accumulation of keratinizing stratified squamous epithelium and desquamated keratin debris within the middle ear, mastoid, or other pneumatized regions of the temporal bone. Although benign in the histological sense, it behaves locally aggressively because the trapped e…
A cholesteatoma is an abnormal, non-neoplastic accumulation of keratinizing stratified squamous epithelium and desquamated keratin debris within the middle ear, mastoid, or other pneumatized regions of the temporal bone. Although benign in the histological sense, it behaves locally aggressively because the trapped epithelial sac expands, erodes adjacent bone, and incites chronic inflammation. Cholesteatomas are classified as congenital, arising from embryonic epithelial rests behind an intact tympanic membrane, or acquired, the more common form, which develops from tympanic membrane retraction pockets, perforation, or epithelial migration, frequently in the setting of chronic otitis media and eustachian tube dysfunction. Progressive enlargement and the release of osteolytic mediators lead to destruction of the ossicular chain, the bony labyrinth, the facial nerve canal, and the tegmen, producing conductive or mixed hearing loss, recurrent foul otorrhea, vertigo, facial nerve weakness, and, in advanced cases, intracranial complications such as meningitis or abscess. Diagnosis relies on otoscopic and microscopic examination, audiometry, and imaging, with high-resolution computed tomography defining bony erosion and diffusion-weighted magnetic resonance imaging aiding detection and surveillance. The definitive treatment is surgical, typically tympanomastoidectomy to eradicate the disease and prevent recurrence, often combined with reconstruction of the tympanic membrane and ossicular chain to restore hearing.
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