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Nasality in Friedreich ataxia.
Matthew L Poole1, Jessica S Wee, Joanne E Folker
1Speech Neuroscience Unit, The University of Melbourne , Parkville, VIC , Australia .
Clinical Linguistics & Phonetics
|September 11, 2014
Summary
Abnormal nasality is common in Friedreich
Area of Science:
- Neurology
- Speech-Language Pathology
Background:
- Altered nasality is a known feature of dysarthria in Friedreich ataxia (FRDA).
- The prevalence and severity of abnormal nasality in FRDA are not well understood.
Purpose of the Study:
- To investigate the incidence and severity of abnormal nasality in individuals with FRDA.
- To explore the relationship between nasality, disease characteristics, and genetic profiles in FRDA.
Main Methods:
- Combined perceptual and acoustic speech analyses were used.
- Nasometry was performed on a subset of participants.
- Thirty-seven individuals with FRDA and 24 healthy controls were included.
Main Results:
- Most participants with FRDA (27/37) exhibited hypernasality, while some (5/37) showed hyponasality.
- Acoustic analysis confirmed significantly greater nasality in FRDA participants compared to controls (p < 0.05).
- Hypernasality perception correlated with GAA2 repeat length (ρ = 0.37, p = 0.03).
Conclusions:
- Nasality in Friedreich ataxia is highly variable, possibly linked to neuropathological differences.
- Genetic factors, specifically GAA2 repeat length, may influence the degree of hypernasality observed in FRDA.
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