title: The management of children with Down syndrome and profound hearing loss.
abstract: BACKGROUND
  Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests.
  
  
  METHODS
  We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme.
  
  
  RESULTS
  Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI.
  
  
  CONCLUSIONS
  The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.
authors.forename: E
authors.initials: E
authors.lastname: Phelan
authors.forename: R
authors.initials: R
authors.lastname: Pal
authors.forename: L
authors.initials: L
authors.lastname: Henderson
authors.forename: K M J
authors.initials: KM
authors.lastname: Green
authors.forename: I A
authors.initials: IA
authors.lastname: Bruce
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000523:psychology*; D003054:Cochlear Implants / Q000523:psychology*; D003072:Cognition Disorders; D004314:Down Syndrome / Q000150:complications* / Q000523:psychology; D005260:Female; D006314:Hearing Loss, Conductive / Q000150:complications / Q000523:psychology / Q000601:surgery*; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000523:psychology / Q000601:surgery*; D006320:Hearing Tests / Q000523:psychology; D006801:Humans; D007223:Infant; D018579:Patient Selection; D011788:Quality of Life; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Cochlear implantation; Down syndrome; Sensorineural hearing loss