A RARE CASE OF A CONGENITAL ANOMALY OF THE EXTERNAL EAR – REDUPLICATION OF THE EXTERNAL AUDITORY CANAL

Abstract

Congenital malformations of the hearing organ occur with a frequency of 1–2 per 500 (mild anomalies) to 1–2 per 10 000 (severe anomalies) newborns. Up to 15 % of them are heritable and up to 10 % are bilateral. Anomalies of the first branchial cleft from which the structures of the external ear predominantly develop, are quite rare malformations (less than 10 % of all gill anomalies), but can present a significant problem of differential diagnosis. Most commonly, these anomalies manifest clinically as periauricular cysts, fistulous or reduplication of the external auditory canal. There are both isolated congenital anomalies of any of the parts of the hearing organ and syndromal ones. This article describes our own observation of a congenital anomaly of the external auditory canal in a child (a type of reduplication of the external auditory canal) and secondary cholesteatoma on this background. This case is interesting because the patient had multiple congenital anomalies: branchial cysts on both sides for which she had been operated on earlier and reduplication of the external auditory canal, a much rarer pathology of the first branchial cleft. At the same time, the difference in time when these anomalies manifested and were detected was almost 10 years.