May 1983

Congenital Middle Ear Cholesteatoma

Author Affiliations

Children's Hospital National Medical Center Washington, DC

Am J Dis Child. 1983;137(5):501-502. doi:10.1001/archpedi.1983.02140310079022

A congenital aural cholesteatoma is a cystic epithelial remnant of embryonal origin found medial to the intact tympanic membrane. This entity may be identified in young children who have not had perforated eardrums and who have had no history of otitis media. The keratoma may be located within the temporal bone, in the region of the petrous apex, in the middle ear cleft (mesotympanic cholesteatoma), or enclosed within the layers of the tympanic membrane (intramembranous cholesteatoma). These rare pseudotumors can simulate persistent otitis media with effusion and, if unrecognized, they can grow, become infected, and in time cause extensive destruction of the middle ear ossicles and mastoid. We describe two infants who had congenital mesotympanic cholesteatoma during routine well-baby examinations. The initial clinical impression in both cases was that the entity was loculated exudate from acute otitis media with effusion.

Report of Cases.—Case 1.—At the three-month routine checkup