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Speech perception ability in individuals with Friedreich ataxia

The aim of this study was to investigate auditory pathway function and speech perception ability in individuals with Friedreich ataxia (FRDA). Ten subjects confirmed by genetic testing as being homozygous for a GAA expansion in intron 1 of the FXN gene were included. While each of the subjects demonstrated normal, or near normal sound detection, 3 of the 10 showed electrophysiological evidence of auditory pathway disorder [presenting with the auditory neuropathy/dyssynchrony (AN/AD) result pattern], and 9 of the 10 showed abnormal speech understanding when tested with levels of background noise typical of everyday listening conditions.

Read More: Speech perception ability in individuals with Friedreich ataxia

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Jen Farmer

Jen Farmer

Executive Director

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