Abstract
PurposeAlthough speech motor changes are reported as a common sign of Huntington’s disease (HD), the most prominent signs of voice dysfunction remain unknown. The aim of the current study was to explore specific changes in phonatory function in subjects with HD.Method34 subjects with HD and 34 age- and sex-matched healthy controls were examined. Participants performed sustained vowel phonation for subsequent analyses of airflow insufficiency, aperiodicity, irregular vibrations of vocal folds, signal perturbations, increased noise, and articulation deficiency. In total, 272 phonations were collected and 12 voice parameters were extracted. Subsequently, a predictive model was built to find the most salient patterns of voice disorders in HD. The results were also correlated with disease severity according to the Unified HD Rating Scale (UHDRS) motor score.ResultsSubjects with HD showed deterioration in all investigated phonatory functions. Irregular pitch fluctuations, sudden phonation interruption, increased noise, and misplacement of articulators were found to be most significant patterns of phonatory dysfunction in HD (p<0.001). The combination of these four dysphonia aspects contributed to the best classification performance of 94.1% (sensitivity: 95.1%; specificity: 93.2%) in the separation of HD patients from healthy participants. Our results further indicated stronger associations between sudden phonation interruption and voluntary components of the UHDRS (r = −0.48, p<0.01) and between misplacement of articulators and involuntary components of the UHDRS (r = 0.52, p<0.01).ConclusionsOur configuration of phonatory features can detect subtle voice abnormalities in subjects with HD. As impairment of phonatory function in HD was found to parallel increasing motor involvement, a qualitative description of voice dysfunction may be helpful to gain better insight into the pathophysiology of the vocal mechanism.
Highlights
Huntington’s disease (HD) is defined as an autosomaldominant, progressive neuropsychiatric disorder caused by an expansion of the number of CAG repeats located on the short arm of chromosome 4 at 4p16.3 [1,2]
In the HD group, the MPT until the first voice break (MPTVB) negatively correlated with voluntary components of Unified HD Rating Scale (UHDRS) motor assessment score (r = 20.47, p,0.01), whereas there were no other correlations between acoustic parameters and voluntary components of motor performance
Considering individual involuntary components, the UHDRS dystonia subscore was positively correlated with detrended fluctuation analysis (DFA) (r = 0.40, p,0.05) and recurrence period density entropy (RPDE) (r = 0.38, p,0.05)
Summary
Huntington’s disease (HD) is defined as an autosomaldominant, progressive neuropsychiatric disorder caused by an expansion of the number of CAG repeats located on the short arm of chromosome 4 at 4p16.3 [1,2]. Patients with HD develop a motor speech disorder characterized as hyperkinetic dysarthria in the course of illness, which occurs mainly as a consequence of underlying choreatic movements [4]. The quality of speech performance in HD is negatively affected by the involuntary contractions of vocal muscles, especially if there is a requirement for steady function. To this extent, the vocal task of sustained vowel phonation is suitable because it demands stable coordination of the jaw, tongue, palate, and facial movements. To assess changes of speech in the course of HD progression, the measurement of steady vowel prolongation is irreplaceable, providing the possibility to observe fluctuations induced by involuntary movements
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