Audiologic and vestibular findings in wolfram syndrome

Roanne K. Karzon, Timothy Hullar

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

OBJECTIVES:: Assessment of auditory and vestibular function in Wolfram Syndrome (WS) patients, using a standardized protocol. DESIGN:: 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.

Original languageEnglish (US)
Pages (from-to)809-812
Number of pages4
JournalEar and Hearing
Volume34
Issue number6
DOIs
StatePublished - Nov 2013
Externally publishedYes

Fingerprint

Wolfram Syndrome
Hearing Loss
Speech Intelligibility
Noise
Acoustic Impedance Tests
Hearing
Ear
Cohort Studies
Prospective Studies
Equipment and Supplies

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Speech and Hearing

Cite this

Audiologic and vestibular findings in wolfram syndrome. / Karzon, Roanne K.; Hullar, Timothy.

In: Ear and Hearing, Vol. 34, No. 6, 11.2013, p. 809-812.

Research output: Contribution to journalArticle

Karzon, Roanne K. ; Hullar, Timothy. / Audiologic and vestibular findings in wolfram syndrome. In: Ear and Hearing. 2013 ; Vol. 34, No. 6. pp. 809-812.
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