Acoustic Analysis of Voice in Huntington's Disease Patients

doi:10.1016/j.jvoice.2009.08.007

Journal of Voice

Volume 25, Issue 2, March 2011, Pages 208-217

https://doi.org/10.1016/j.jvoice.2009.08.007 Get rights and content

Summary

Background/Objectives

Alterations of voice and speech are frequently observed in Huntington's disease (HD). The aim of this study was to assess the degree of voice and speech abnormalities in a group of patients with HD and to analyze possible correlations with degree of disease severity.

Patients/Methods

An observational study was carried out on diagnosed cases and controls. The voices of 20 patients were analyzed and compared with an age- and sex-matched control group. Variables analyzed included subjective voice exploration, analysis of aerodynamic efficiency, acoustic analysis measures, and laryngeal examination descriptions. Results obtained were correlated with degree of disease severity.

Results

Changes in the Voice Handicap Index and clinical characteristics of the voice were observed. Maximum phonation time was reduced. Acoustic analysis revealed changes that were analyzed. Uncontrolled adduction-abduction movements were observed by laryngeal examination. All results showed a positive correlation with degree of disease severity assessed by the Unified Huntington's Disease Rating Scale.

Conclusions

HD causes alterations in subjective voice features, aerodynamic and acoustic analysis measures that are correlated with disease severity.

Introduction

Huntington's disease (HD) is a progressive inherited neurological disorder manifested by involuntary movements, cognitive impairment, and psychiatric symptoms.¹ Although chorea is the most common involuntary movement in HD (90%), other movement disorders can occur, such as parkinsonism, dystonia, myoclonus, tics, and ataxia.² “Quick hyperkinetic dysarthria” of extrapyramidal system origin, there has been speculated that the speech characteristics of these patients reflect quick involuntary movements and variable tone in musculature important for speech production.³

It seems reasonable that such involuntary contractions of speech mechanism musculature would be particularly disturbing at levels of speech production requiring steady muscular function, such as phonation. Involuntary contractions, variable muscle tone, or tremor of laryngeal musculature could perceptibly affect the vibratory characteristics of the vocal folds.⁴ The voice of patients with HD has been described as being intermittently harsh, breathy, and strain strangled and as having irregular pitch fluctuations and arrest.5, 6, 7, 8 Such perceptual analyses have made important contributions to the diagnosis of HD in some patients and support the effect of this disease on phonatory aspects of speech production.9, 10

Petajan¹¹ has suggested that motor abnormalities may be present in nascent form in HD patients before the usual time of diagnosis at age 20–30 years. In a study of single motor unit (SMU) control, they identified abnormalities in 10 of 10 HD patients, and in 11 of 20 (55%) subjects considered to be at risk for developing HD. HD patients could not achieve SMU control and bursts of chorea were frequent. In individuals at risk, small irregular, ballistic activation of motor units termed microchorea and excessive recruitment of SMUs associated with voluntary activity were observed. It was proposed that microchorea and the inability to sustain SMU firing may be physiological markers for HD in subjects at risk but without clinical signs.

It is probable that the bursts of chorea observed in the interosseous muscles also occur in the laryngeal musculature. Such activity is one reasonable physiologic correlate for the perception of “irregular pitch fluctuations and voice arrest” observed in the phonation of HD patients. Similarly, it is possible that subtle motor abnormalities may occur in the laryngeal musculature of individuals at risk that could be reflected in acoustic measures of voice.12, 13

These alterations of voice could affect the quality of life of HD patients. The Voice Handicap Index (VHI) measures the influence of voice problems on a patient's quality of life.¹⁴ The VHI was used in this study to measure the patient's perception of voice handicap. The instability and uncontrolled movements produce abnormal changes in laryngeal muscle tone during sustained phonation, and the vocal effect results in vocal arrest and reduction of maximum phonation time (MPT). Acoustic analysis offers a method to study phonatory characteristics in these patients noninvasively and to provide objective information related to laryngeal pathophysiology of both clinical and academic interest.

Taking into account what is currently known on voice features of HD, it should be expected that this disease could cause impairment in VHI, in many of the parameters included in a standard acoustic analysis, in aerodynamic measures, and in the laryngeal examination. It should also be expected that the degree of severity of HD should influence these values. The aim of this study was to describe the degree of voice and speech abnormalities and the findings of laryngeal examination in a group of patients with a molecular genetics diagnosis of HD, compared with matched controls, and to assess the possible correlation between vocal impairment and disease severity.

The attainment of these objectives would provide information regarding the pathophysiology of voice in HD patients, which could be useful in clinical practice from the point of view of speech-therapy rehabilitation, a tool that we think fundamental in the command of these patients, allowing us to develop a strategy that compensates for the deficit produced by their phonatory dysfunction .

Section snippets

Patients and controls

The voices of 20 patients with a molecular genetics diagnosis of HD were analyzed. The World Medical Association's ethical guidelines on using humans in medical research were followed for all patients. The Informed Consent Form was approved by the Ethics Committee at the “Príncipe de Asturias” University Hospital in Alcalá de Henares (Madrid).

The HD patient group comprised 14 men and 6 women collected between 2003 and 2006. All reported a positive family history of HD. The mean age was 46 ± 11.2

Subjective voice exploration: VHI

The results of the VHI assessment in the three domains (Functional, Physical, and Emotional), and of global assessment of HD patients compared with the control group of similar age and sex, are shown in Table 2. The total score and the subtotal scores of the three domains of this scale were significantly higher in the HD patient group when compared with controls. There was also a statistically significant correlation in the HD patient group of the scores of this scale with disease severity

Discussion

HD is a hyperkinetic disease characterized by involuntary movements (mainly chorea, but also other movement disorders such as parkinsonism, dystonia, myoclonus, tics, and ataxia).1, 2 It would seem logical to think that this chaotic muscular activity could also affect the muscular mechanisms involved in phonation and speech, resulting in a tense, forced, rough voice with pitch and intensity fluctuations.5, 21, 22, 23 The stability and continuity of vowel sounds are altered by breaks and

Acknowledgments

The authors express their gratitude to the individuals who participated as subjects in this research and specially to the Huntington's disease Association. The contributions of Asunción Blanco are gratefully acknowledged.

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