Congenital cholesteatoma in a case of congenital aural atresia.

OBJECTIVE: A congenital cholesteatoma arising in the setting of congenital aural atresia is described. This represents the first case reported in the literature.

STUDY DESIGN: Case report.

SETTING: Tertiary otologic referral center, University of Virginia Health System, Charlottesville, Virginia.

PATIENT: Eight-year-old patient with unilateral congenital aural atresia.

INTERVENTIONS: Preoperative computer tomographic scanning and surgical atresia repair with removal of cholesteatoma.

MAIN OUTCOME MEASURES: Recurrence of cholesteatoma; epithelialized ear canal; hearing improvement.

RESULTS: A congenital cholesteatoma located medial to the ossicular mass in a child with congenital aural atresia was removed, with no evidence of recurrent disease at a revision operation 1 year later. Hearing was improved to a speech reception threshold of 25 dB.

CONCLUSION: Congenital cholesteatoma can arise medial to the ossicular mass in congenital aural atresia. This finding lends support to the epidermoid rest theory for the cause of congenital cholesteatoma.