title: Hearing screening in children with skeletal dysplasia.
abstract: OBJECTIVE
  To determine the prevalence of hearing loss and abnormal tympanometry in children with skeletal dysplasia.
  
  
  METHODS
  Clinical screening program.
  
  
  METHODS
  National convention of the Little People of America.
  
  
  METHODS
  Convenience sample of volunteers aged 18 years or younger with skeletal dysplasias.
  
  
  METHODS
  Hearing screening with behavioral testing and/or otoacoustic emissions, otoscopy, and tympanometry.
  
  
  METHODS
  A failed hearing screen was defined as hearing 35 dB HL (hearing level) or greater at 1 or more tested frequencies or by a "fail" otoacoustic emissions response. Types B and C tympanograms were considered abnormal.
  
  
  RESULTS
  A total of 58 children (aged ≤18 years) with skeletal dysplasia enrolled, and 56 completed hearing screening. Forty-one children had normal hearing (71%); 9 failed in 1 ear (16%); and 6 failed in both ears (10%). Forty-four children had achondroplasia, and 31 had normal hearing in both ears (71%); 8 failed hearing screening in 1 ear (18%), and 3 in both ears (7%). Tympanometry was performed in 45 children, with normal tympanograms found in 21 (47%), bilateral abnormal tympanograms in 15 (33%), and unilateral abnormal tympanograms in 9 (20%). Fourteen children with achondroplasia had normal tympanograms (42%); 11 had bilateral abnormal tympanograms (33%); and 8 had unilateral abnormal tympanograms (24%). For those children without functioning tympanostomy tubes, there was a 9.5 times greater odds of hearing loss if there was abnormal tympanometry (P = .03).
  
  
  CONCLUSIONS
  Hearing loss and middle-ear disease are both highly prevalent in children with skeletal dysplasias. Abnormal tympanometry is highly associated with the presence of hearing loss, as expected in children with eustachian tube dysfunction. Hearing screening with medical intervention is recommended for these children.
authors.affiliation: Johns Hopkins Outpatient Center, 601 N Caroline St, Baltimore, MD 21287-0910, USA. dtunkel@jhmi.edu
authors.forename: David E
authors.initials: DE
authors.lastname: Tunkel
authors.forename: Richard
authors.initials: R
authors.lastname: Kerbavaz
authors.forename: Beth
authors.initials: B
authors.lastname: Smith
authors.forename: Danielle
authors.initials: D
authors.lastname: Rose-Hardison
authors.forename: Yewande
authors.initials: Y
authors.lastname: Alade
authors.forename: Julie
authors.initials: J
authors.lastname: Hoover-Fong
mesh_terms: D000130:Achondroplasia / Q000175:diagnosis; D000158:Acoustic Impedance Tests; D000293:Adolescent; D000465:Algorithms; D001301:Audiometry, Pure-Tone; D001848:Bone Diseases, Developmental / Q000175:diagnosis* / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004392:Dwarfism / Q000175:diagnosis; D005260:Female; D006314:Hearing Loss, Conductive / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D008403:Mass Screening; D017084:Otoacoustic Emissions, Spontaneous; D038781:Otoscopy