Abstract
Congenital cholesteatoma is a rare entity and cholesteatoma surrounding the entire otic capsule and involving petrous apex is even rarer. The condition may remain hidden for a long duration destroying the temporal bone and might present as an intracranial space-occupying lesion, loss of hearing, facial nerve palsy without significant otoscopic findings. We report a case of thirty-three years old female who presented with facial palsy and hearing loss for 7 months without any history of ear discharge. Possibility of congenital cholesteatoma was sorted based on clinical and radiological features. Transotic approach was done for clearing the disease. Congenital cholesteatoma should be considered as a differential diagnosis in any patient with unexplained otological signs and symptoms. Radical excision is the mainstay of treatment. Keywords: Congenital cholesteatoma, Facial nerve palsy, Hearing loss, Petrous bone.
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