title: Dysarthria in Friedreich's ataxia: a perceptual analysis.
abstract: The aims of this study were to: (1) evaluate the perceptual speech dimensions, speech intelligibility and dysarthria severity of a group of individuals diagnosed with Friedreich's ataxia (FRDA); (2) determine the presence of subgroups within FRDA dysarthria; (3) investigate the relationship between the speech outcome and the clinical factors of disease progression. The study included 38 individuals (21 female, 17 male) with a confirmed diagnosis of FRDA. A group of 20 non-neurologically impaired individuals served as controls. Perceptual analysis, investigating 30 different dimensions of speech, was conducted on a speech sample obtained from each participant. In addition, the Assessment of Intelligibility of Dysarthria Speech was administered. All FRDA participants presented with dysarthria with severities ranging from mild to moderate. Cluster analysis revealed 3 subgroups, the first presenting with mild dysarthric symptoms, the second with increased velopharyngeal involvement and the third characterized by increased laryngeal dysfunction. Dysarthria severity showed a significant correlation to disease duration but to no other clinical measure. The findings support the notion of subgroups in FRDA dysarthria, representing distinct impairments of the speech mechanism and perhaps reflective of differing evolutions beyond the cerebellum.
authors.affiliation: School of Health and Rehabilitation Science, The University of Queensland, Brisbane, Qld., Australia. j.folker@uq.edu.au
authors.forename: Joanne
authors.initials: J
authors.lastname: Folker
authors.forename: Bruce
authors.initials: B
authors.lastname: Murdoch
authors.forename: Louise
authors.initials: L
authors.lastname: Cahill
authors.forename: Martin
authors.initials: M
authors.lastname: Delatycki
authors.forename: Louise
authors.initials: L
authors.lastname: Corben
authors.forename: Adam
authors.initials: A
authors.lastname: Vogel
mesh_terms: D000328:Adult; D002531:Cerebellum / Q000503:physiopathology; D016000:Cluster Analysis; D018450:Disease Progression; D004401:Dysarthria / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications* / Q000235:genetics; D006801:Humans; D007830:Larynx / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D010609:Pharyngeal Muscles / Q000503:physiopathology; D012720:Severity of Illness Index; D013065:Speech Intelligibility; D013068:Speech Production Measurement; D018911:Trinucleotide Repeats; D014681:Velopharyngeal Insufficiency / Q000209:etiology; D014826:Vocal Cord Paralysis / Q000209:etiology / Q000503:physiopathology; D055815:Young Adult