Ear and Hearing

Skip Navigation LinksHome > November/December 2013 - Volume 34 - Issue 6 > Audiologic and Vestibular Findings in Wolfram Syndrome
Ear & Hearing:
doi: 10.1097/AUD.0b013e3182944db7
Brief Reports

Audiologic and Vestibular Findings in Wolfram Syndrome

Karzon, Roanne K.1,2; Hullar, Timothy E.2

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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.

© 2013 by Lippincott Williams & Wilkins


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