title: [Long term results of congenital middle ear cholesteatoma in children].
abstract: OBJECTIVE
  To report the long term results of congenital cholesteatoma of the middle ear in children.
  
  
  METHODS
  Retrospective study of thirty two children with 33 congenital cholesteatoma (1 bilateral cholesteatoma) operated on by the same surgeon. The mean age was 6 years. The most common clinical presentation was unilateral hearing loss. A trans-canal approach was performed in 4 cases and an intact canal wall technique in 29 cases. A two staged surgery was necessary in 28 patients, whom a residual cholesteatoma was observed in 7 patients. A third stage was performed in 11 patients because of a residual cholesteatoma in 2 two cases and a functional failure in 9 cases.
  
  
  RESULTS
  At five years postoperative audiometry (air bone gap inferior or equal to 20 dB HL), a functional success was obtained in 50% of cases.
  
  
  CONCLUSIONS
  The diagnosis of congenital cholesteatoma must be evoked in every atypical otitis media with effusion or unilateral transmission hypoacusis. The intact canal wall technique in two stages is the most appropriate treatment, since congenital cholesteatoma in children appears aggressive in a well pneumatized mastoid. The choice for an intact canal wall technique is also justified by the ambition of a conservative surgery in the young child. The functional results are generally satisfactory in early diagnosed cases. Thus, ENT and pediatric physicians have a unique role in detecting congenital cholesteatoma in the early period of life, and in ensuring a long term follow-up.
authors.affiliation: Service d'ORL, CHU de Grenoble, BP 217, 38043 Grenoble Cedex 9.
authors.forename: A
authors.initials: A
authors.lastname: Benhammou
authors.forename: D-Q
authors.initials: DQ
authors.lastname: Nguyen
authors.forename: K
authors.initials: K
authors.lastname: El Makhloufi
authors.forename: R
authors.initials: R
authors.lastname: Charachon
authors.forename: E
authors.initials: E
authors.lastname: Reyt
authors.forename: S
authors.initials: S
authors.lastname: Schmerber
mesh_terms: D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D005500:Follow-Up Studies; D046088:Hearing Loss, Unilateral / Q000209:etiology; D006801:Humans; D007223:Infant; D012189:Retrospective Studies; D016896:Treatment Outcome; D014433:Tympanoplasty / Q000379:methods