Impact on Voice
Huntington's disease presents with changes in cognition (thinking), motor control (movement), and mood/behavior (psychological); it should be noted that even during the end stages of the disease, people with HD typically have intact receptive ability (they can understand what is being said to and around them). This section primarily focuses on how changes in Huntington's disease relate to voice.
The National Institutes of Health reports that for some people with Huntington’s Disease, the initial symptoms may be clumsiness and small, unintentional movements of the feet, fingers, face, or trunk. While the course of the disease ranges from 10-30 years, NIH notes that early onset of symptoms is correlated with swifter course of disease.
While slight “fidgety” movements, clumsiness, and trouble with balance can signal the onset of Huntington’s Disease, as the disease progresses the chorea and other symptoms become more pronounced.
According to the American Speech-Language Hearing Association (ASHA), the nature and severity of communicative symptoms will vary from person to person. Frequently, the areas of difficulty will remain the same but increase in overall severity as the disease progresses; sometimes abilities will come and go from moment to moment. Voice problems may be related to hyperkinetic dysarthria (speech difficulty related to issues with muscle strength, speed, and coordination of lips, tongue, throat and jaw). Features of reduced voice quality may include the following:
- Impaired respiration for speech
- Strained/strangled quality
- Hoarse/harsh sounding
- Inappropriate volume
- Intermittent hypernasality
- Difficulty coordinating phonation (voicing) and respiration (breathing)
According to the Movement Disorder Society, voicing in Huntington's disease may be interrupted with an inconsistent fundamental frequency. There may be an increase in mean shimmer (fluctuating loudness) and jitter (fluctuating pitch), as well as a reduction in harmonic-to-noise ratio. Sustained phonation is also difficult for those with HD, as involuntary opening and closing of the vocal folds may occur.
The Huntington's Disease Society of America notes that three stages of HD are commonly recognized, though some professionals specify five.
In the early stage, the family and person with Huntington's disease may notice subtle differences in mood, motor, and cognitive aspects. However, the affected person is typically able to retain their job through this phase. In the mid stage, issues with voluntary motor control will make speech and other activities of daily living more challenging; often the person with HD will be unable to continue work and will file for disability at this point. In late stage Huntington's Disease, the person may lose the ability to speak, but is still able to understand others. The chorea may be severe at this point, causing the affected person to need assistance with all activities of daily living.
Ho, Cull, Drake, Barker, Tabrizi, and Lee (2010) conducted a study with 41 participants who had Huntington’s disease, concerning voice and quality of life. They discovered that people with HD feel the effects of voice and speech-related changes as early as stage 1 (of 5), and additionally the study reinforced previous data that there were significant changes in voice-related quality of life between stages 4 and 5.
It is important that staff and caregivers realize that a person with HD who can no longer speak clearly (or at all) can still understand. The person with HD may feel isolated, depressed, and helpless if others refrain from speaking to them.
Compiled 11/2012. Mary Patteson.