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Title: Voice in Friedreich Ataxia
Austin Authors: Vogel, Adam P;Wardrop, Mayumi I;Folker, Joanne E;Synofzik, Matthis;Corben, Louise A;Delatycki, Martin B ;Awan, Shaheen N
Affiliation: Austin Health, Heidelberg, Victoria, Australia
Centre for Neuroscience of Speech, The University of Melbourne, Melbourne, Victoria, Australia
Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University of Tübingen, Germany
German Research Center for Neurodegenerative Diseases (DZNE), University of Tübingen, Germany
School of Health and Rehabilitation Sciences, The University of Queensland, Brisbane, Queensland, Australia
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Melbourne, Victoria, Australia
School of Psychological Sciences, Monash University, Melbourne, Victoria, Australia Monash Health, Melbourne, Victoria, Australia
Clinical Genetics, Austin Health, Heidelberg, Victoria, Australia
Department of Audiology & Speech Pathology, Bloomsburg University of Pennsylvania, Bloomsburg, Pennsylvania
Issue Date: Mar-2017
Date: 2016-08-05
Publication information: Journal of Voice 2017; 31(2): 243
Abstract: BACKGROUND: Friedreich Ataxia (FRDA) is the most common hereditary ataxia, with dysarthria as one of its key clinical signs. OBJECTIVE: To describe the voice profile of individuals with FRDA to inform outcome marker development and goals of speech therapy. METHODS: Thirty-six individuals with FRDA and 30 age-matched controls provided sustained vowel and connected speech samples. Speech and voice samples were analyzed acoustically using the Analysis of Dysphonia in Speech and Voice program and perceptually using the Consensus Auditory-Perceptual Evaluation of Voice form. Correlations between dysphonia and overall dysarthria severity, demographic, clinical, and genetic information were explored. RESULTS: Individuals with FRDA presented with mild dysphonia characterized by hoarseness (combined roughness and breathiness), increased strain, and altered pitch variability (increased in vowel productions; slightly decreased on reading samples). Acoustically, individuals with FRDA had significantly higher scores on the Cepstral Spectral Index of Dysphonia during vowel production. A combination of perceptual and acoustic measures of dysphonia used in this study was quite effective in categorizing the FRDA versus control participants, with >80% overall accuracy. CONCLUSIONS: Although dysphonia severity in FRDA did not correlate significantly with overall disease severity, speaking rate and syllabic duration significantly correlated with age at disease onset and disease duration, and also have an effect on listener perception of dysphonia. The relationship between dysphonia and dysarthria in FRDA suggests that reducing overall dysphonia severity via therapeutic techniques that improve phonatory stability and increase speaking rate is a viable target for speech therapy.
DOI: 10.1016/j.jvoice.2016.04.015
Journal: Journal of Voice
PubMed URL:
Type: Journal Article
Subjects: Acoustics
Clinical markers
Hereditary ataxia
Appears in Collections:Journal articles

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