Bilateral occurrence of congenital middle ear cholesteatoma

Congenital cholesteatoma of the middle ear is a relatively rare entity that is becoming more frequently encountered in the pediatric age group. A high index of suspicion is needed to recognize this entity at an early stage. More challenging is the bilateral occurrence of the lesion that, although still rare, should always be remembered when examining children with congenital cholesteatoma, not only on the initial visit but also on subsequent follow–up. We report the eleventh case of bilateral congenital middle ear cholesteatoma and stress the importance of long–term follow–up in these cases.