Auditory perception in individuals with Friedreich’s Ataxia

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 FR...

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Main Authors:	G, Rance, L., Corben, E., Barker, P., Carew, D., Chisari, M., Rogers, R., Dowell, Jamaluddin, Saiful Adli, R., Bryson, M.B., Delatycki,
Format:	Article
Language:	English
Published:	Karger 2010
Subjects:	RF Otorhinolaryngology
Online Access:	http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/2/66025%20Auditory%20perception.pdf

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spelling	iium-660252018-09-28T08:06:39Z http://irep.iium.edu.my/66025/ Auditory perception in individuals with Friedreich’s Ataxia G, Rance L., Corben E., Barker P., Carew D., Chisari M., Rogers R., Dowell Jamaluddin, Saiful Adli R., Bryson M.B., Delatycki , , RF Otorhinolaryngology 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 individuals with FRDA showed evidence of auditory processing disorder. Gap and amplitude modulation detection levels in these subjects were significantly elevated, indicating impaired encoding of rapid signal changes. Electrophysiologic findings (auditory brainstem response, ABR) also reflected disrupted neural activity. Speech understanding was significantly affected in these listeners and the degree of disruption was related to temporal processing ability. Speech analyses indicated that timing cues (notably consonant voice onset time and vowel duration) were most affected. CONCLUSION: The results suggest that auditory pathway abnormality is a relatively common consequence of FRDA. Regular auditory evaluation should therefore be part of the management regime for all affected individuals. This assessment should include both ABR testing, which can provide insights into the degree to which auditory neural activity is disrupted, and some functional measure of hearing capacity such as speech perception assessment, which can quantify the disorder and provide a basis for intervention Karger 2010-05 Article PeerReviewed application/pdf en http://irep.iium.edu.my/66025/2/66025%20Auditory%20perception.pdf G, Rance and L., Corben and E., Barker and P., Carew and D., Chisari and M., Rogers and R., Dowell and Jamaluddin, Saiful Adli and R., Bryson and M.B., Delatycki and UNSPECIFIED and UNSPECIFIED (2010) Auditory perception in individuals with Friedreich’s Ataxia. Audiology and Neurotology, 15 (4). pp. 229-240. ISSN 1420-3030 E-ISSN 1421-9700 https://www.karger.com/Article/Abstract/255341 10.1159/000255341
repository_type	Digital Repository
institution_category	Local University
institution	International Islamic University Malaysia
building	IIUM Repository
collection	Online Access
language	English
topic	RF Otorhinolaryngology
spellingShingle	RF Otorhinolaryngology G, Rance L., Corben E., Barker P., Carew D., Chisari M., Rogers R., Dowell Jamaluddin, Saiful Adli R., Bryson M.B., Delatycki , , Auditory perception in individuals with Friedreich’s Ataxia
description	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 individuals with FRDA showed evidence of auditory processing disorder. Gap and amplitude modulation detection levels in these subjects were significantly elevated, indicating impaired encoding of rapid signal changes. Electrophysiologic findings (auditory brainstem response, ABR) also reflected disrupted neural activity. Speech understanding was significantly affected in these listeners and the degree of disruption was related to temporal processing ability. Speech analyses indicated that timing cues (notably consonant voice onset time and vowel duration) were most affected. CONCLUSION: The results suggest that auditory pathway abnormality is a relatively common consequence of FRDA. Regular auditory evaluation should therefore be part of the management regime for all affected individuals. This assessment should include both ABR testing, which can provide insights into the degree to which auditory neural activity is disrupted, and some functional measure of hearing capacity such as speech perception assessment, which can quantify the disorder and provide a basis for intervention
format	Article
author	G, Rance L., Corben E., Barker P., Carew D., Chisari M., Rogers R., Dowell Jamaluddin, Saiful Adli R., Bryson M.B., Delatycki , ,
author_facet	G, Rance L., Corben E., Barker P., Carew D., Chisari M., Rogers R., Dowell Jamaluddin, Saiful Adli R., Bryson M.B., Delatycki , ,
author_sort	G, Rance
title	Auditory perception in individuals with Friedreich’s Ataxia
title_short	Auditory perception in individuals with Friedreich’s Ataxia
title_full	Auditory perception in individuals with Friedreich’s Ataxia
title_fullStr	Auditory perception in individuals with Friedreich’s Ataxia
title_full_unstemmed	Auditory perception in individuals with Friedreich’s Ataxia
title_sort	auditory perception in individuals with friedreich’s ataxia
publisher	Karger
publishDate	2010
url	http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/ http://irep.iium.edu.my/66025/2/66025%20Auditory%20perception.pdf
first_indexed	2023-09-18T21:33:42Z
last_indexed	2023-09-18T21:33:42Z
_version_	1777412679782105088

Auditory perception in individuals with Friedreich’s Ataxia

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