Occult contralateral congenital cholesteatoma: is the epidermoid formation theory enough?

Bilateral congenital cholesteatomas are rare entities. Nine cases have been previously described in the literature. Many different etiologies for the development of congenital cholesteatoma have been proposed. The case of a five-year-old boy with bilateral congenital cholesteatoma is discussed. A lesion of the left ear was apparent clinically. However, the right-sided lesion was silent and was demonstrated only by radiologic exam. Theories of pathogenesis are reviewed.