Bilateral congenital cholesteatomas associated with ossicular anomalies: a case report

A case of bilateral congenital cholesteatomas, which occurred in a 15-year-old male was reported. His family had been aware of his hearing impairment since he was 3 years old. He had experienced neither otalgia nor otorrhea. Tympanograms were A type bilaterally, and an audiogram showed conductive hearing loss of about 60 dB on both sides. High resolution CT of a horizontal section revealed soft tissue density occupying the space medial to the body of the incus in the right ear, while the middle ear space appeared to be completely clear on the left side. The membranous cholesteatomas on both sides were accompanied by ossicular anomalies with hypoplasia of the long process of incus and the superstructure of the stapes. This case constitutes the seventh case report of bilateral congenital cholesteatomas in the literature. The literature is reviewed and the pathogenesis of congenital cholesteatomas associated with ossicular anomalies is discussed.