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.
Rance
G
G
Department of Otolaryngology, The University of Melbourne, Parkville, Victoria 3010, Australia. grance@unimelb.edu.au
Corben
L A
LA
Du Bourg
E
E
King
A
A
Delatycki
M B
MB
D000293:Adolescent; D000328:Adult; D001308:Auditory Perceptual Disorders / Q000503:physiopathology / Q000628:therapy*; D002648:Child; D005260:Female; D005621:Friedreich Ataxia / Q000503:physiopathology / Q000628:therapy*; D006310:Hearing Aids; D006320:Hearing Tests; D006801:Humans; D008297:Male; D013067:Speech Perception; D055815:Young Adult