Journal article
Auditory processing deficits in children with Friedreich ataxia
G Rance, L Corben, M Delatycki
Journal of Child Neurology | SAGE PUBLICATIONS INC | Published : 2012
Abstract
Friedreich ataxia is a neurodegenerative disease with an average age of onset of 10 years. The authors sought to investigate the presence and functional consequences of auditory neuropathy in a group of affected children and to evaluate the ability of personal FM-listening systems to improve perception. Nineteen school-aged individuals with Friedreich ataxia and a cohort of matched control subjects underwent a battery of auditory function tests. Sound detection was relatively normal, but auditory temporal processing and speech understanding in noise were severely impaired, with children with Friedreich ataxia typically able to access less than 40% of the information available to controls. Us..
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Awarded by National Institutes of Health
Funding Acknowledgements
The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was supported by a project grant from the Friedreich Ataxia Research Alliance. GR was supported by the Wagstaff Research Fellowship in Otolaryngology. Supported by grants from the National Institute of Neurological Disorders and Stroke (5R13NS040925-15), the National Institutes of Health Office of Rare Disease Research, the Child Neurology Society, and the National Ataxia Foundation. Supported by grants from the National Institutes of Health (2R13NS040925-14 Revised), the National Institutes of Health Office of Rare Diseases Research, the Child Neurology Society, and the National Ataxia Foundation.