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.


METHODS
Case report.


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


METHODS
Eight-year-old patient with unilateral congenital aural atresia.


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


METHODS
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.


CONCLUSIONS
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.
Caughey
Robert J
RJ
Department of Otolaryngology-Head and Neck Surgery, University of Virginia Health System, Charlottesville, Virginia 22908-0713, USA.
Jahrsdoerfer
Robert A
RA
Kesser
Bradley W
BW
D002648:Child; D018424:Cholesteatoma, Middle Ear / Q000150:complications / Q000151:congenital* / Q000601:surgery; D004424:Ear Canal / Q000002:abnormalities*; D005260:Female; D006801:Humans; D019933:Ossicular Replacement; D010146:Pain; D012086:Reoperation; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome