title: Audiologic and vestibular findings in Wolfram syndrome.
abstract: OBJECTIVE
  Assessment of auditory and vestibular function in Wolfram Syndrome (WS) patients, using a standardized protocol.
  
  
  METHODS
  Prospective cohort study of 11 patients using otoscopic inspection, tympanometry, otoacoustic emissions, pure tones, speech in noise (SIN), the Speech Intelligibility Index, and rotational chair testing.
  
  
  RESULTS
  Mean SNHL diagnosis was 7.3 years with 55% prevalence. Four patients with a Speech Intelligibility Index less than 0.75 (better ear) routinely used bilateral amplification devices. Two patients with normal-hearing sensitivity exhibited abnormal SIN scores. The only patient with significant vestibular dysfunction also had a distinctive low-frequency component to her SNHL.
  
  
  CONCLUSIONS
  Hearing loss may occur earlier than previously suspected, and comprehensive testing including SIN testing may reveal deficits not apparent with pure-tone testing. Particular configurations of hearing loss may indicate a need for comprehensive vestibular assessment. Because SNHL can be the first symptom of WS, audiologists and otolaryngologists should be vigilant about referring patients with hearing loss for ophthalmologic examination.
authors.affiliation: 1Department of Audiology, 2Department of Otolaryngology-Head and Neck Surgery, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Roanne K
authors.initials: RK
authors.lastname: Karzon
authors.forename: Timothy E
authors.initials: TE
authors.lastname: Hullar
mesh_terms: D000293:Adolescent; D001299:Audiometry / Q000379:methods*; D002648:Child; D015331:Cohort Studies; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D017410:Practice Guidelines as Topic; D011446:Prospective Studies; D012720:Severity of Illness Index; D014722:Vestibule, Labyrinth / Q000503:physiopathology*; D014929:Wolfram Syndrome / Q000150:complications*; D055815:Young Adult