title: Does canal wall down mastoidectomy benefit syndromic children with congenital aural stenosis?
abstract: OBJECTIVE
  To determine whether a canal wall down mastoidectomy can provide long-term benefit for children with aural stenosis.
  
  
  METHODS
  Retrospective case series of children with congenital aural stenosis having undergone a canal wall down mastoidectomy over a twelve-year period at a tertiary children's hospital.
  
  
  RESULTS
  Data from thirteen children who underwent a total of twenty canal wall down mastoidectomies for aural stenosis were reviewed. The mean age at surgery was 7.1 years (range, 3.3-12.3 years). All patients had genetic syndromes including Trisomy 21 (n = 7), Trisomy 21 and Pierre Robin sequence (n = 1), Angelmann (n = 1), Cri-du-chat (n = 1), Branchio-oto-renal syndrome (n = 1), Spina bifida (n = 1) and Nager syndrome (n = 1). Seven (54%) children underwent bilateral canal wall down mastoidectomies. All thirteen ears that could not be visualized preoperatively had improved ease of office examination following surgery. Only one patient required revision surgery and all canals were patent at the last clinic visit. The mean follow-up was 4.9 years. There were no cases of facial nerve injury or cerebrospinal fluid leak.
  
  
  CONCLUSIONS
  Syndromic children with congenital aural stenosis with poorly pneumatized mastoids may benefit from canal wall down mastoidectomy to improve ease of office examinations.
authors.affiliation: Division of Otolaryngology - Head and Neck Surgery, University of Utah School of Medicine, Salt Lake City, UT, USA.
authors.forename: Phayvanh P
authors.initials: PP
authors.lastname: Sjogren
authors.affiliation: Division of Otolaryngology - Head and Neck Surgery, University of Utah School of Medicine, Salt Lake City, UT, USA.
authors.forename: Richard K
authors.initials: RK
authors.lastname: Gurgel
authors.affiliation: Division of Otolaryngology - Head and Neck Surgery, University of Utah School of Medicine, Salt Lake City, UT, USA; Pediatric Otolaryngology, Primary Children's Hospital, Salt Lake City, UT, USA. Electronic address: Albert.Park@imail.org.
authors.forename: Albert H
authors.initials: AH
authors.lastname: Park
mesh_terms: D017204:Angelman Syndrome / Q000150:complications; D019280:Branchio-Oto-Renal Syndrome / Q000150:complications; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic / Q000150:complications / Q000151:congenital / Q000601:surgery; D003410:Cri-du-Chat Syndrome / Q000150:complications; D004314:Down Syndrome / Q000150:complications; D004424:Ear Canal / Q000002:abnormalities / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008342:Mandibulofacial Dysostosis / Q000150:complications; D008416:Mastoid / Q000601:surgery*; D010855:Pierre Robin Syndrome / Q000150:complications; D012086:Reoperation; D012189:Retrospective Studies; D016135:Spinal Dysraphism / Q000150:complications; D016896:Treatment Outcome; D014432:Tympanic Membrane / Q000601:surgery
keywords: Canal wall down; Congenital aural stenosis; External auditory canal; Mastoidectomy; Otorrhea; Syndromic