title: Surgical aspects of cochlear implantation in syndromic children.
abstract: OBJECTIVE
  The objective of this study was to report surgical results and outcomes of cochlear implantation in a large series of children with syndromes from one centre.
  
  
  METHODS
  All syndromic children who underwent cochlear implantation at Great Ormond Street Hospital, from January 2000 to December 2010 were included in this study. The surgical technique was analysed and audiological outcomes were collected.
  
  
  RESULTS
  Over the 10-year period of this study, a total of 88 cochleas in 67 children with syndromes were implanted. The common syndromes implanted in this study were Ushers syndrome (23 patients, 33 cochleas), Wardenburgs syndrome (8 patients, 9 cochleas), Pendreds syndrome (4 patients, 4 cochleas), Jervell-Lange-Neilsen syndrome (3 patients, 4 cochleas), Enlarged vestibular aqueduct syndrome (4 patients, 7 cochleas), Cogans syndrome (3 patients, 4 cochleas), CHARGE (5 patients, 6 cochleas), and Branchio Oto Renal syndrome (3 patients, 4 cochleas). Pre-operative radiological inner ear anatomy was found to be abnormal in 28.4% (25/88) cochleas in this study group. Full insertion of the electrode was achieved in 93.1% (82/88) of cochleas, partial insertion in three cochleas, and insertion was abandoned in three cochleas. Early complications were seen in 6.8% (6/88) of implantations. All the 64/67 children who were implanted are still using the implant.
  
  
  CONCLUSIONS
  Cochlear implantation in syndromic children is challenging in both its audiological and surgical aspects. Good surgical results and good audiological and speech outcomes were achieved in this study, and subjective improvement in quality of life was achieved in these patients.
authors.affiliation: Department of Paediatric Otolaryngology, Great Ormond Street Hospital, London, UK. ybajaj@hotmail.co.uk
authors.forename: Yogesh
authors.initials: Y
authors.lastname: Bajaj
authors.forename: Nick
authors.initials: N
authors.lastname: Gibbins
authors.forename: Kathryn
authors.initials: K
authors.lastname: Fawkes
authors.forename: Ben
authors.initials: B
authors.lastname: Hartley
authors.forename: Chris
authors.initials: C
authors.lastname: Jephson
authors.forename: Nico
authors.initials: N
authors.lastname: Jonas
authors.forename: David
authors.initials: D
authors.lastname: Albert
authors.forename: Martin
authors.initials: M
authors.lastname: Bailey
authors.forename: Michelle
authors.initials: M
authors.lastname: Wyatt
authors.forename: Lesley
authors.initials: L
authors.lastname: Cochrane
mesh_terms: D000158:Acoustic Impedance Tests; D000293:Adolescent; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000379:methods*; D003638:Deafness / Q000235:genetics* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000209:etiology*; D011474:Prosthesis Design; D013069:Speech Reception Threshold Test; D013577:Syndrome