Congenital cholesteatoma in a 3-year-old

A congenital cholesteatoma is defined as a cholesteatoma that sits behind an intact tympanic membrane and is not associated with a history of otorrhea, otologic intervention, or perforation.1 The average age at diagnosis is 4.5 years; cases have been detected as early as infancy2 and as late as 62 years.3 More than 80% are found, at least partly, in the anterosuperior quadrant of the middle ear cleft, as occurred in our patient.4 The remainder can occur anywhere in the temporal bone, and they can even extend intradurally.