title: Delayed diagnosis and fate of congenital cholesteatoma (keratoma).
abstract: OBJECTIVE
  To analyze clinical presentation, modes of detection, growth pattern, operative findings, and results of surgery in children 3 years old or older who had extensive congenital cholesteatoma (keratoma).
  
  
  METHODS
  Survey, case series.
  
  
  METHODS
  Two academically affiliated medical centers: a children's hospital and an eye, ear, and throat hospital, both located in major metropolitan cities.
  
  
  METHODS
  Twenty-five children selected according to specified criteria.
  
  
  METHODS
  Tympanomastoid surgery, ie, canal wall up and canal wall down, some with ossicular reconstructive surgery.
  
  
  METHODS
  Audiologic assessment (speech reception threshold) and recurrence (recidivism) of cholesteatoma.
  
  
  RESULTS
  Incidence of recidivism, 52%. Hearing maintained within the range of normal to mild hearing impairment postoperatively in 91% of the patients for whom complete data are available.
  
  
  CONCLUSIONS
  Congenital cholesteatoma may grow for years without causing signs or symptoms and, having grown without early detection, can extend to involve the epitympanum and mastoid antrum, cause ossicular erosion, and even extend to the middle cranial fossa. To adequately remove a congenital cholesteatoma that has gone undetected for many years, exposure of the anterior epitympanum is often necessary and removal of both the body of the incus and the head of the malleus often is required. Since congenital cholesteatoma usually develops in a child with a well-pneumatized mastoid that would create a large mastoid bowl if exteriorized, the otologic surgeon is likely to hesitate in using the canal wall down mastoidectomy technique. Alternatives to the canal wall down mastoidectomy technique, which can achieve reasonably good hearing results and avoid creation of a large mastoid bowl, include planned two-stage canal wall up surgery or canal preservation with primary reconstruction and close follow-up with otomicroscopy and serial computed tomographic scans.
authors.affiliation: Department of Otolaryngology, Children's National Medical Center, Washington, DC 20010, USA.
authors.forename: K M
authors.initials: KM
authors.lastname: Grundfast
authors.forename: G S
authors.initials: GS
authors.lastname: Ahuja
authors.forename: S C
authors.initials: SC
authors.lastname: Parisier
authors.forename: S M
authors.initials: SM
authors.lastname: Culver
mesh_terms: D000293:Adolescent; D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D018424:Cholesteatoma, Middle Ear / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D006309:Hearing; D006801:Humans; D012008:Recurrence; D013060:Speech