An Atypical Presentation of a Congenital Cholesteatoma in an Adult with Extensive Inner Ear Erosion

Abstract Congenital cholesteatoma (CC) in an adult in his sixth decade is a rare presentation. We present a case report of surgical management of a case of CC in an adult in his sixth decade with extensive erosion of the bony labyrinth and facial canal, reaching up to the internal auditory meatus without eroding the ossicles or extension into the mastoid.