title: [Congenital cholesteatoma of the middle ear: a case series different from cases in the literature].
abstract: We present 44 cases of congenital cholesteatoma of the middle ear. Twenty-one patients had a cholesteatoma located in the posterosuperior mesotympanum. This finding was in complete contrast to the commonly reported anterosuperior location, seen in only 2 cases in our study. The remaining 21 patients had a cholesteatoma involving either the entire mesotympanum and/or epitympanum. The posteriorly located congenital cholesteatoma might represent a completely different entity and originate from epithelial cell debris trapped in the posterior mesotympanum during development of the temporal bone. All but one patient were treated with a closed tympanoplasty. Eight patients underwent single stage surgery. A preplanned second stage procedure was performed in 33 patients, while 3 are presently awaiting the second stage. Residual disease was seen in 19 patients (57%) who had undergone second stage surgery. No patient has had recurrent disease this far. Thirty-eight patients (85%) had a preoperative air bone gap of 30 dB or more. Of the 33 patients evaluated for hearing results, 16 (48%) had a postoperative gap within 10 dB.
authors.forename: M
authors.initials: M
authors.lastname: Landolfi
authors.forename: M
authors.initials: M
authors.lastname: Falcioni
authors.forename: A
authors.initials: A
authors.lastname: Taibah
authors.forename: A
authors.initials: A
authors.lastname: Russo
authors.forename: G
authors.initials: G
authors.lastname: De Donato
authors.forename: F
authors.initials: F
authors.lastname: Mancini
authors.forename: M
authors.initials: M
authors.lastname: Sanna
mesh_terms: D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000151:congenital* / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D013997:Time Factors; D014433:Tympanoplasty
vernacular_title: Il colesteatoma congenito dell'orecchio medio: una casistica differente dalla letteratura.