title: Surgical treatment of middle ear cholesteatoma in children with Down syndrome.
abstract: OBJECTIVE
  To report our personal experience in the surgical treatment of cholesteatoma in children with Down syndrome.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Tertiary care otology and skull base centers.
  
  
  METHODS
  Nine patients with Down syndrome were surgically treated for cholesteatoma. Two patients had bilateral disease, resulting in a total of 11 ears surgically treated.
  
  
  METHODS
  A canal-wall-up mastoidectomy was performed in two ears; in eight of the ears, a canal-wall-down mastoidectomy was carried out and a modified Bondy procedure was performed in one ear.
  
  
  RESULTS
  Residual cholesteatoma was found in one ear after the canal-wall-up mastoidectomy and recurrent cholesteatoma developed in another ear, also after canal-wall-up mastoidectomy. The recurrence required conversion to canal-wall-down mastoidectomy. One patient developed a perforation of the neotympanic membrane that had to be revised.
  
  
  CONCLUSIONS
  Cholesteatoma in children with Down syndrome is a challenging entity for the otologic surgeon. Otolaryngologists should always suspect a cholesteatoma in each child with Down syndrome presenting warning symptoms such as otorrhea and hearing loss. If there is any doubt on inspection, further imaging studies (high-resolution computed tomography) are necessary. To the best of our knowledge, the current study is the first report to document the surgical treatment of cholesteatoma in subjects with Down syndrome.
authors.affiliation: Department of Otolaryngology, University of Parma, Parma, Italy. andreabacciu@yahoo.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Bacciu
authors.forename: Enrico
authors.initials: E
authors.lastname: Pasanisi
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Vincenti
authors.forename: Davide
authors.initials: D
authors.lastname: Giordano
authors.forename: Antonio
authors.initials: A
authors.lastname: Caruso
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Lauda
authors.forename: Salvatore
authors.initials: S
authors.lastname: Bacciu
mesh_terms: D000293:Adolescent; D002648:Child; D018424:Cholesteatoma, Middle Ear / Q000601:surgery*; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008416:Mastoid / Q000601:surgery*; D010033:Otitis Media / Q000150:complications; D013506:Otologic Surgical Procedures; D012008:Recurrence; D012189:Retrospective Studies; D016896:Treatment Outcome