Cholesteatoma with intact tympanic membrane: a report of 41 cases.

Aural cholesteatoma may present itself as an unexplained hearing impairment without either perforation or otorrhea and without radiologic evidence of bone destruction. The etiology is probably congenital. In our cholesteatoma cases, 3.7% occur in individuals with an intact tympanic membrane. Most of these patients have had the disease 5 years or less and 50% are under 20 years of age. Complications of the disease are uncommon in this group. All but 1 of our 41 cases were managed with the intact canal wall technique. Most were reexplored (planned second-stage) and residual disease was encountered in 32%. One should keep the possibility of a congentital cholesteatoma in mind wheneger encountering a unilateral serous otitis media or an unexplained unilateral conductive hearing impairment, regardless of the patient's age.