SUCCESSFUL TREATMENT OF AUDITORY PERCEPTUAL DISORDER IN INDIVIDUALS WITH Friedreich ATAXIA

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 (C) 2010 IBRO Published by Elsevier Ltd All rights reserved