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Neuroscience. 2010 Dec 1;171(2):552-5. doi: 10.1016/j.neuroscience.2010.09.013. Epub 2010 Sep 17.

Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia.

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Department of Otolaryngology, The University of Melbourne, Parkville, Victoria 3010, Australia.


Friedreich ataxia (FRDA) is a neurodegenerative disease affecting motor and sensory systems. This study aimed to investigate the presence and perceptual consequences of auditory neuropathy (AN) in affected individuals and examine the use of personal-FM systems to ameliorate the resulting communication difficulties. Ten individuals with FRDA underwent a battery of auditory function tests and their results were compared with a cohort of matched controls. Friedreich ataxia subjects were then fit with personal FM-listening devices and evaluated over a 6 week period. Basic auditory processing was affected with each FRDA individual showing poorer temporal processing and figure/ground discrimination than their matched control. Speech perception in the presence of background noise was also impaired, with FRDA listeners typically able to access only around 50% of the information available to their normal peers. The use of personal FM-listening devices did however, dramatically improve their ability to hear and communicate in everyday listening situations.

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