title: Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia.
abstract: 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.
authors.affiliation: Department of Otolaryngology, The University of Melbourne, Parkville, Victoria 3010, Australia. grance@unimelb.edu.au
authors.forename: G
authors.initials: G
authors.lastname: Rance
authors.forename: L A
authors.initials: LA
authors.lastname: Corben
authors.forename: E
authors.initials: E
authors.lastname: Du Bourg
authors.forename: A
authors.initials: A
authors.lastname: King
authors.forename: M B
authors.initials: MB
authors.lastname: Delatycki
mesh_terms: 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