Congenital Aural Atresia Associated with Cholesteatoma / 대한이비인후과학회지

Congenital aural atresia (CAA) with variable degrees of microtia are combined with many other otologic or facial anomalies. Among them, cholesteatoma developed behind atretic plate is rarely reported. Since Von Luders had first reported about this condition at 1912, several authors had described CAA associated with cholesteatoma. In their reports, atretic canal cannot prevent the development of cholesteatoma. We reviewed 4 cases of CAA associated with cholesteatoma with medical records and preoperative temporal bone CTs retrospectively. All the cases were unilaterally presented and corrected by canaloplsty. Keratin materials were demonstrated at the medial side of atretic ear canal.