Auditory perception in individuals with friedreich's ataxia

G Rance; L Corben; E Barker; P Carew; D Chisari; M Rogers; R Dowell; S Jamaluddin; R Bryson; MB Delatycki

Journal article

Auditory perception in individuals with friedreich's ataxia

G Rance, L Corben, E Barker, P Carew, D Chisari, M Rogers, R Dowell, S Jamaluddin, R Bryson, MB Delatycki

Audiology and Neurotology | KARGER | Published : 2010

DOI: 10.1159/000255341

Abstract

Introduction: Friedreich's ataxia (FRDA) is an inherited ataxia with a range of progressive features including axonal degeneration of sensory nerves. The aim of this study was to investigate auditory perception in affected individuals. Methods: Fourteen subjects with genetically defined FRDA participated. Two control groups, one consisting of healthy, normally hearing individuals and another comprised of subjects with sensorineural hearing loss, were also assessed. Auditory processing was evaluated using structured tasks designed to reveal the listeners' ability to perceive temporal and spectral cues. Findings were then correlated with open-set speech understanding. Results: Nine of 14 indiv..

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University of Melbourne Researchers

Gary Rance Author

Louise Corben Author

Peter Carew Author

Donella Chisari Author

Grants

Funding Acknowledgements

[ "Funding: Wagstaff Research Fellowship in Otolaryngology (G. R., E. B.); National Health and Medical Research Council Practitioner Fellowship (M. B. D.); Friedreich's Ataxia Research Alliance (Australia) (M. B. D., L. C.); Friedreich's Ataxia Research Alliance (USA) (M. B. D., L. C.).", "We would like to express our appreciation to the participants in this study, who gave their time so willingly and who continue to support our research. Special thanks also to Dr. David Grayden for his technical support." ]