Bilateral congenital cholesteatomas

Congenital cholesteatoma is a rare entity. Bilateral involvement is rarer still. We present the sixth case of bilateral congenital cholesteatoma and briefly review the theories regarding the origin of congenital cholesteatoma. The lesion in the left ear was seen on physical examination, however the lesion in the right ear was detected only on a computed tomography (CT) scan, which was obtained to assess the extent of the disease on the left side. Although usually asymptomatic, these cholesteatomas can enlarge and lead to complications. Management of congenital cholesteatoma by various surgical approaches is discussed.