title: Audiological profile of children and young adults with syndromic and complex craniosynostosis.
abstract: OBJECTIVE
  To determine syndrome-specific type, severity, and prevalence of hearing loss to facilitate follow-up and treatment.
  
  
  METHODS
  Tertiary pediatric hospital craniofacial clinic survey study. If insufficient or no data were available for a child, he or she was referred to an audiologist for pure-tone audiometry.
  
  
  METHODS
  Academic research facility.
  
  
  METHODS
  Information was gathered regarding 132 children and young adults with craniosynostosis.
  
  
  METHODS
  The primary outcome was hearing assessment of children and young adults with various types of craniosynostosis. A secondary outcome was inference regarding the incidence of otitis media among children and young adults with craniosynostosis.
  
  
  RESULTS
  We found mild or moderate hearing loss in 44.0% of patients with Apert syndrome, in 28.5% with Crouzon syndrome, in 62.1% with Muenke syndrome, in 28.6% with Saethre-Chotzen syndrome, and in 6.7% with complex craniosynostosis. Hearing loss was conductive in most patients with Apert, Crouzon, and Saethre-Chotzen syndromes and it was predominantly sensorineural in patients with Muenke syndrome. Sensorineural hearing loss at lower frequencies was found only in patients with Muenke syndrome.
  
  
  CONCLUSIONS
  Most patients with syndromic and complex craniosynostosis have recurrent otitis media with effusion, causing episodes of conductive hearing loss throughout their lives. Sensorineural hearing loss can occur in all 4 syndromes studied but is the primary cause of hearing loss in children and young adults with Muenke syndrome. For patients with these syndromes, we recommend routine visits to the general practitioner or otolaryngologist, depending on national standards of care, to screen for otitis media with effusion throughout life. We also advise early screening for sensorineural hearing loss among children and young adults with these syndromes.
authors.affiliation: Department of Plastic, Reconstructive, and Hand Surgery, Erasmus Medical Center-Sophia, Rotterdam, the Netherlands. t.dejong@erasmusmc.nl
authors.forename: Tim
authors.initials: T
authors.lastname: de Jong
authors.forename: Martijn S
authors.initials: MS
authors.lastname: Toll
authors.forename: Henriëtte H W
authors.initials: HH
authors.lastname: de Gier
authors.forename: Irene M J
authors.initials: IM
authors.lastname: Mathijssen
mesh_terms: D000168:Acrocephalosyndactylia / Q000503:physiopathology; D000293:Adolescent; D001301:Audiometry, Pure-Tone; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000503:physiopathology; D003398:Craniosynostoses / Q000503:physiopathology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications; D006801:Humans; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000503:physiopathology; D010033:Otitis Media / Q000150:complications; D010034:Otitis Media with Effusion / Q000150:complications