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.