title: Atypical presentation of congenital cholesteatoma in an adult case with good hearing result.
abstract: BACKGROUND
  Congenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a "pearl" beneath the anterosuperior quadrant of the tympanic membrane.
  
  
  METHODS
  We report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years.
  
  
  CONCLUSIONS
  The case was surgically treated. Postoperative hearing result was satisfactory.
  
  
  CONCLUSIONS
  Congenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.
authors: Bursa Sevket Yilmaz Training and Research Hospital, Department of Otorhinolaryngology, 16800 Yıldırım, Bursa, Turkey.
authors: Fevzi
authors: F
authors: Solmaz
authors: Bursa Sevket Yilmaz Training and Research Hospital, Department of Otorhinolaryngology, 16800 Yıldırım, Bursa, Turkey.
authors: Davut
authors: D
authors: Akduman
authors: Bursa Sevket Yilmaz Training and Research Hospital, Department of Otorhinolaryngology, 16800 Yıldırım, Bursa, Turkey.
authors: Mehmet
authors: M
authors: Haksever
authors: Bursa Sevket Yilmaz Training and Research Hospital, Department of Otorhinolaryngology, 16800 Yıldırım, Bursa, Turkey.
authors: Ercan
authors: E
authors: Gündoğdu
authors: Bursa Sevket Yilmaz Training and Research Hospital, Department of Otorhinolaryngology, 16800 Yıldırım, Bursa, Turkey.
authors: Atilla
authors: A
authors: Mescioğlu
keywords: Atypical presentation; Congenital cholesteatoma; Hearing; Intact canal wall tympanomastoidectomy