Objective: The purpose of this study is to describe and analyse the clinical features of congenital cholesteatomas associated with congenital ossicular anomalies.

Method: The clinical data of eight non-syndromic patients who were diagnosed congenital cholesteatomas associated with congenital ossicular anomalies were reviewed retrospectively. The clinical data included sex, age, symptoms, signs, audiological results, temporal bone computed tomography scan, intraoperative findings, management, pathological findings and follow-up results.

Results: In three cases, external ear anomalies were accompanied. In one case, an atretic plate replaced a normal tympanic membrane. In seven cases, the cholesteatoma was in the posterio-superior tympanum. Only in one case, the cholesteatoma was in the anterior-superior tympanum. The ossicular anomalies were mainly incus and/or stapes anomalies. In seven cases, the ossicular chain was reconstructed after removal of the cholesteatoma. In the other case, reconstruction of the ossicular chain had been given up because of the absence of the oval window. In three cases, the hearing ability had improved. In two cases, hearing ability did not change. All these five cases had no evidence of recurrent or residual cholesteatoma. The other three cases had been lost contact.

Conclusion: Congenital cholesteatomas associated with congenital ossicular anomalies is rare. The cause seems to be developmental abnormalities of the first and the second branchial arches.