Congenital cholesteatomas in the tympanic membrane

The etiology of congenital middle ear (ME) cholesteatomas is unclear. One etiologic possibility of ME cholesteatoma may be progression of a congenital tympanic membrane (TM) cholesteatoma. We recently have encountered three cases of congenital tympanic membrane cholesteatoma. Each child, ages 1, 3, and 14 years, presented with cholesteatoma of the tympanic membrane extending into the middle ear. These children have not had previous otologic surgery including myringotomy, nor had they had repeated middle ear infections, perforation, or trauma. Neither the 3-year-old nor 14-year-old child complained of hearing loss. Audiograms demonstrated only a mild conductive loss. Each child underwent excision with tympanoplasty. Although the middle ear component of the cholesteatoma was always more extensive than the pearl seen, the point of attachment was the TM and not the middle ear. This demonstrates one possible source for congenital cholesteatomas.