![Sample our Health and Social Care journals, sign in here to start your FREE access for 14 days]({"type":"image" , "src" : "/sda/5938/TOC-Subject-Sample-2021-Health-Social-Care.jpg"})
Abstract
Perceptual speech research in Friedreich ataxia (FRDA) has identified altered nasality as a key component of the dysarthria profile, however the incidence and severity of abnormal nasality remains unknown. Utilizing objective and perceptual methods, data on the relationship between resonance, disease duration, severity, age of onset and genetic profiles were collated. Thirty-seven participants with FRDA and 24 healthy controls provided contemporaneous speech samples for perceptual analysis, and single word samples for acoustic analysis. A subset of participants (eight participants with FRDA and eight controls) underwent nasometry assessment. Twenty-seven participants with FRDA presented with hypernasality and five with hyponasality on perceptual assessment. Acoustic analysis revealed participants with FRDA had greater nasality than controls (p < 0.05). Perceptual ratings of hypernasality correlated with GAA2 repeat length (ρ = 0.37, p = 0.03). Findings highlight the variability of nasality in FRDA, potentially reflecting variation in the neuropathological profile. Data also suggest the influence of genetic profiles on nasality.
Acknowledgements
The authors wish to thank the participants involved in the study; Dr. Michael Fahey for administering the FARS; Audrey Price for assisting in some assessments; and the Friedreich Ataxia Clinic, Monash Medical Centre, Victoria, Australia, and the Friedreich’s Ataxia Network, Queensland, Australia, for recruitment.
Declaration of interest
The authors report no declarations of interest. APV and LAC hold National Health and Medical Research Council (NHMRC) Early Career Research Fellowships (ID1012302 & ID1037002 respectively).
Supplementary material available online
Supplementary Tables A and B