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Auditory dysfunction in patients with Huntington's disease.

Oliver Profant1, Jan Roth2, Zbyněk Bureš3

  • 1Department of Auditory Neuroscience, Institute of Experimental Medicine, The Czech Academy of Sciences, Prague, Czech Republic; Department of Otorhinolaryngology and Head and Neck Surgery, 1(st) Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czech Republic; Department of Otorhinolaryngology of University Hospital Královské Vinohrady and 3(rd) Faculty of Medicine, Charles University, Prague, Czech Republic.

Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology
|August 22, 2017
PubMed
Summary
This summary is machine-generated.

Huntington's disease (HD) patients show normal hearing but struggle with understanding speech, especially in noisy environments. This auditory processing deficit correlates with motor and cognitive decline in HD.

Keywords:
Auditory pathologyCentral hearing lossCognitionHuntington’s disease

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Area of Science:

  • Neuroscience
  • Audiology
  • Genetics

Background:

  • Huntington's disease (HD) is a progressive, autosomal dominant neurodegenerative disorder.
  • Key features include motor, cognitive, and behavioral impairments.
  • Auditory system involvement in HD is not well understood.

Purpose of the Study:

  • To investigate auditory system function in patients with genetically verified Huntington's disease.
  • To assess audiological parameters and central auditory processing in HD patients.

Main Methods:

  • 17 HD patients and age-matched controls underwent comprehensive audiological testing.
  • Tests included pure tone audiometry, otoacoustic emissions, speech audiometry (in noise), and auditory brainstem responses.
  • Advanced tests focused on central auditory processing and sound localization.

Main Results:

  • HD patients exhibited normal hearing thresholds, otoacoustic emissions, and auditory brainstem responses.
  • A significant decline in speech understanding, particularly in noise, was observed in HD patients.
  • Deficits in sound source localization and a correlation between speech-in-noise perception and motor/cognitive function were noted.

Conclusions:

  • Huntington's disease negatively impacts central auditory pathways (cortical and subcortical).
  • Speech processing and sound source lateralization are predominantly affected.
  • Findings suggest central auditory and cognitive areas are involved in HD pathology.