Auditory perception in individuals with Friedreich’s Ataxia

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

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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 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

Item Type:	Article (Journal)
Additional Information:	4466/66025
Uncontrolled Keywords:	Friedreich’s ataxia, Auditory perception, Temporal processing, Speech perception
Subjects:	R Medicine > RF Otorhinolaryngology
Kulliyyahs/Centres/Divisions/Institutes (Can select more than one option. Press CONTROL button):	Kulliyyah of Allied Health Sciences Kulliyyah of Allied Health Sciences > Department of Audiology and Speech-Language Pathology
Depositing User:	DR. SAIFUL ADLI JAMALUDDIN
Date Deposited:	28 Sep 2018 16:06
Last Modified:	28 Sep 2018 16:06
URI:	http://irep.iium.edu.my/id/eprint/66025

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