title: Congenital cholesteatoma of the middle ear in children.
abstract: Congenital cholesteatoma, once considered to be a rarity, has shown an exponential increase in its incidence as evidenced by numerous clinical studies over the past decade. No explanation has been offered for this phenomenon. A review of the literature indicates that the definition of "congenital cholesteatoma" has strayed from the original one formulated by Derlacki and Clemis, which included a cholesteatoma medial to an intact tympanic membrane, without prior history of aural infections. Allowing patients with recurrent otitis media into studies may have contributed to a "false positive" rise in the incidence of this condition. This article reviews 19 cases of cholesteatomas behind intact tympanic membranes, treated at the Montreal Children's Hospital over a 10-year period. They represent 22% of the entire cholesteatoma population. There appears to be two groups of patients, each with a distinct clinical presentation that correlates well with the surgical outcome. Whether this implies different pathogenetic mechanisms remains unclear.
authors.affiliation: Department of Otolaryngology, McGill University, Montreal, Quebec, Canada.
authors.forename: J M
authors.initials: JM
authors.lastname: Chen
authors.forename: M D
authors.initials: MD
authors.lastname: Schloss
authors.forename: J J
authors.initials: JJ
authors.lastname: Manoukian
authors.forename: R S
authors.initials: RS
authors.lastname: Shapiro
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000150:complications / Q000151:congenital* / Q000601:surgery; D004427:Ear Diseases / Q000150:complications / Q000151:congenital / Q000601:surgery; D004432:Ear, Middle; D005260:Female; D006314:Hearing Loss, Conductive / Q000209:etiology / Q000601:surgery; D006801:Humans; D008297:Male; D010033:Otitis Media / Q000150:complications; D012189:Retrospective Studies