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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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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.

Copyright © 2013 by Lippincott Williams & Wilkins

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