Article Text

C16 Hoarseness can be found in vocalisations of both human as well as genetically modified minipig model of huntington’s disease
  1. Jan Hlavnička1,
  2. Tereza Tykalová1,
  3. Monika Mačáková2,
  4. Monika Baxa2,
  5. Roman čmejla1,
  6. Jan Motlík2,
  7. Jiří Klempíř3,4,
  8. Jan Rusz1,3
  1. 1Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University in Prague, Prague, Czech Republic
  2. 2Institute of Animal Physiology and Genetics, Czech Academy of Sciences, Liběchov, Czech Republic
  3. 3Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic
  4. 4Institute of Anatomy, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic


Background Voice quality of patients with Huntington’s disease is commonly perceived as harsh, hoarse, or breathy. Digital signal analysis allows to quantify the degree of hoarseness objectively by harmonic-to-noise ratio. Such a simple automated acoustic measurement could provide cheap, non-invasive, and effective biomarker of Huntington’s disease.

Aims The aim of this study is to explore, whether the hoarseness is presented in human as well as in a genetically modified minipig animal model of Huntington’s disease.

Methods Data were digitally recorded using head-set microphone with linear frequency response. Sustained phonations of the vowel/a/and vowel/i/were recorded from 20 patients with Huntington’s disease (mean duration of disease 6.4 years, standard deviation 3.0 years) and 23 matched healthy controls. Vocalisations were acquired from 14 Huntington’s disease transgenic minipigs (mean age 45.9 months, standard deviation 14.5 months), and 12 matched healthy siblings. Harmonic-to-noise ratio was analysed only on voiced segments of phonations and grunt-like vocalisations.

Results Harmonic-to-noise ratio was significantly decreased between patients with Huntington’s disease (mean 19.3 dB, standard deviation 5.4 dB) when compared to healthy control speakers (mean 22.4 dB, standard deviation 3.1 dB) (p < 0.05) as well as between transgenic minipigs (mean 3.26 dB, standard deviation 1.9 dB) as compared to healthy siblings (mean 4.3 dB, standard deviation 1.3 dB) (p < 0.05).

Conclusions Our results demonstrate that hoarseness is presented in both human as well as animal pig model of Huntington’s disease. Although dissimilarities of vocal apparatus between human and minipig are obvious, our findings suggest that pathophysiology mechanisms of Huntington’s disease influence voice in both human and minipig model similarly. More complex vocal assessment may be beneficial for monitoring of Huntington’s disease onset in transgenic minipigs.

  • genetically modified minipig animal model for Huntington’s disease
  • dysphonia
  • harmonic-to noise-ratio

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