Congenital Cholesteatoma of the Ear

Abstract

Congenital cholesteatoma of the ear is a capricious and challenging problem for the otologist. We present 21 cases managed at the Mayo Clinic from 1961 to 1983. In all but one unusual case, the cholesteatoma developed behind an intact tympanic membrane and there was no history of aural infections. Preoperative symptoms and signs were predominantly conductive deafness with either a normal tympanic membrane or an opaque, whitish appearance to the drum. Disease was located in the middle ear or in the middle ear and mastoid, except in one patient who had cholesteatoma extending into the petrous apex. Surgical management depended on the extent of the lesion, and recurrences were unusual, suggesting that congenital cholesteatoma may not be as aggressive as acquired cholesteatoma.

Adult-Onset Labyrinthine Fistula as a Sign of Undiagnosed Bilateral Congenital Cholesteatoma

“…To date, only a few studies have reported labyrinthine fistula in congenital cholesteatoma. The reported frequency of labyrinthine fistula in congenital cholesteatoma (0% to 5%) [5,7,12,13] is less than the frequency of acquired cholesteatoma (7%) [1]. However, Darrouzet et al reported a relatively higher rate (8.8%) of labyrinthine fistula in congenital cholesteatoma [2].…”

“…House reported that the incidence of congenital cholesteatoma is 3.7% [5], and incidence of bilateral congenital cholesteatoma has been estimated to be less than 3% of all congenital cholesteatomas [4]. Although both acquired and congenital cholesteatoma can lead to several complications, e.g., ossicular erosion, facial palsy, intracranial infection, and labyrinthine fistula, these complications tend to present less often in congenital cholesteatoma than in acquired cholesteatoma [7]. There are no reports of labyrinthine fistula resulting from bilateral congenital cholesteatoma in the international literature.…”

Congenital cholesteatoma and malformations of the facial nerve: Rare manifestations of the BOR syndrome

“…Most cholesteatomas are acquired in the setting of recurrent otitis media. Primary or congenital cholesteatomas (CC) are rare, representing approximately 2-5% of all cholesteatomas in three large series [Leong and Portmann, 1992;McDonald et al, 1984;House and Sheehy, 1980]. Diagnostic criteria for congenital cholesteatoma include the presence of a squamous epithelial mass behind an intact tympanic membrane in the absence of a significant history of infection, eustachian tube dysfunction, or tympanic perforation [Derlacki and Clemis, 1965].…”

Usefulness of Computed Tomography Hounsfield Unit Measurement for Diagnosis of Congenital Cholesteatoma

“…Congenital cholesteatoma appears as a whitish mass in the middle ear with an intact tympanic membrane in children, and it is a rare disease accounting for approximately 2% of all cholesteatoma cases (1).…”