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Abstract

Twenty-three adult patients with type 2 Usher's syndrome were evaluated for changes in pure tone average (PTA), discrete frequency thresholds, and speech discrimination scores. These patients were evaluated over a period of 5 years on the average (range, 2 to 9 years). Analyses of ear data showed a <10-dB change on PTA and discrete frequency thresholds for most ears. Of the ears that showed a threshold change ≥ 10 dB, statistical significance was reached only at 4,000 Hz (p < .01), where 11 ears representing ten patients dropped 10 to 15 dB. Speech discrimination data showed a ≥ 16% change in score for 18 ears (12 patients). Sixteen of those ears (ten patients) had a 16% to 52% decrease in score (p < .01), and the remaining two ears (two patients) showed a 20% and 30% improvement in score. These findings document little or no change in pure tone sensitivity for type 2 Usher's syndrome patients, but demonstrate the importance of monitoring their speech discrimination performance.

Keywords

retinitis pigmentosa sensorineural hearing loss Usher's syndrome

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Evaluation of Pure Tone and Speech Discrimination Changes in Usher's Syndrome

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