Abstract
BackgroundCholesteatoma is regarded as a critical health issue in the world. Nevertheless, congenital cholesteatoma (CC) is an unusual problem, and bilateral CC is extremely rare.Case presentationThe present work reports a young boy with conductive hearing loss because of CC. The left side was operated using a canal-wall-down mastoidectomy method for immense destruction of the middle ear structures and mastoid air cells, and endoscopic ear surgery was performed on the second side.ConclusionAlthough CC is very rare, it should be considered in the differential diagnosis of hearing loss in children. Moreover, it should be carefully followed up because it can probably occur on the second side.
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