title: The prevalence of congenital hearing loss in neonates with Down syndrome.
abstract: OBJECTIVE
  To determine the prevalence of hearing loss in newborns with Down syndrome.
  
  
  METHODS
  We performed a cross-sectional, retrospective chart review of all infants with Down syndrome born at a university-affiliated hospital (n = 77) or transferred in to the associated pediatric hospital (n = 32) following birth at an outlying hospital between 1995 and 2010. We determined the rate of failure of newborn hearing screens, the proportion of infants lost to follow-up, and the rate of confirmed hearing loss, as well as the associations of risk factors for hearing loss with confirmed hearing loss.
  
  
  RESULTS
  Of the 109 patients with hearing screening data, 28 failed their newborn hearing screen. Twenty-seven infants were referred for audiologic evaluation, and 19 completed the evaluation. Fifteen of these 19 infants (79%) had confirmed hearing loss. The prevalence of congenital hearing loss in this sample of neonates with Down syndrome was 15%. Exposure to mechanical ventilation was the sole known risk factor associated with hearing loss. In this study, the loss to follow-up rate for infants with positive hearing screens was 32%.
  
  
  CONCLUSIONS
  Newborns with Down syndrome have a higher prevalence of congenital hearing loss compared with the total neonatal population (15% vs 0.25%). Continued monitoring of hearing is needed in children with Down syndrome.
authors.affiliation: Department of Neurology, Golisano Children's Hospital of Southwest Florida, Fort Myers, FL.
authors.forename: Adrienne S
authors.initials: AS
authors.lastname: Tedeschi
authors.affiliation: Division of Developmental-Behavioral Pediatrics and Psychology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH.
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Roizen
authors.affiliation: Division of Developmental-Behavioral Pediatrics and Psychology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH.
authors.forename: H Gerry
authors.initials: HG
authors.lastname: Taylor
authors.affiliation: Department of Otolaryngology Head & Neck Surgery, Case Western Reserve University School of Medicine, Cleveland, OH.
authors.forename: Gail
authors.initials: G
authors.lastname: Murray
authors.affiliation: Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH.
authors.forename: Christine A
authors.initials: CA
authors.lastname: Curtis
authors.affiliation: Department of Genetics and Genome Sciences, Case Western Reserve University School of Medicine, Cleveland, OH.
authors.forename: Aditi Shah
authors.initials: AS
authors.lastname: Parikh
mesh_terms: D002648:Child; D003430:Cross-Sectional Studies; D004314:Down Syndrome / Q000453:epidemiology*; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis / Q000453:epidemiology*; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015997:Neonatal Screening; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors