Disregard of cholesteatoma in congenital aural stenosis

Congenital aural stenosis (CAS) is one type of congenital aural atresia (CAA) and is assumed to be a relatively mild type. Although CAS may be associated with cholesteatoma in the external ear canal, little attention has been paid to this association. We present two cases of CAS with middle ear infection due to destructive cholesteatoma. Both had been followed for microtia by a plastic surgeon. These patients had already exhibited cholesteatoma on CT examination, although it had been missed before the emergence of ear symptoms, otorrhea, and otalgia. We present our cases with successive changes in CT findings over time and discuss the problems of CAS with cholesteatoma.