title: [Congenital middle ear cholesteatoma].
abstract: OBJECTIVE
  To investigate the location, clinical symptom, image features, treatment and prognosis of the congenital middle ear cholesteatoma.
  
  
  METHODS
  Retrospective review of 9 patients treated at Department of Otolaryngology Head and Neck Surgery, the affiliated Hospital of the first Strasbourg University, France.
  
  
  RESULTS
  The mean age was 6.1 years. Including 7 boys and 2 girls with hearing loss, and 7 of them suffered conductive hearing loss. Typical cholesteatomas were found behind posterior tympanic membrane according the otoscopic examinations in 6 cases. CT were completed in 8 of 9 cases, spheroid or ovoid masses were found in the tympanic cavities, most of them located in the posterior region. All of the patients underwent the surgical treatment, 5 of 9 cases had the ossicular erosion. Four of 9 cases had the residual diseases. All of the cases were followed-up at the mean period of 5.3 years, the preoperative hearing threshold is 28 dB compared with 26 dB of postoperative hearing threshold.
  
  
  CONCLUSIONS
  Congenital middle ear cholesteatoma is a rare and hiding lesion. The symptoms are more typical in children than that in adults, which is unilateral conductive hearing loss. Likewise,the preoperative CT scanning is important. The rate of residual lesions and prognosis of congenital cholesteatoma is mainly depended on the extension and the methods of the surgery, revision surgery as well.
authors.affiliation: Department of Otorhinolaryngology, the First Affiliated Hospital, Chongqing Medical University, China. qianyi119@gmail.com
authors.forename: Yi
authors.initials: Y
authors.lastname: Qian
authors.forename: Guohua
authors.initials: G
authors.lastname: Hu
mesh_terms: D001309:Auditory Threshold; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000151:congenital* / Q000175:diagnosis*; D005260:Female; D006314:Hearing Loss, Conductive / Q000209:etiology; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies