Auditory dysfunction in patients with Huntington's disease
Language English Country Netherlands Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
PubMed
28826025
DOI
10.1016/j.clinph.2017.07.403
PII: S1388-2457(17)30890-8
Knihovny.cz E-resources
- Keywords
- Auditory pathology, Central hearing loss, Cognition, Huntington’s disease,
- MeSH
- Audiometry, Speech methods MeSH
- Hearing Loss, Central diagnosis etiology physiopathology MeSH
- Adult MeSH
- Huntington Disease complications diagnosis physiopathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Speech Perception physiology MeSH
- Aged MeSH
- Evoked Potentials, Auditory, Brain Stem physiology MeSH
- Auditory Threshold physiology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
OBJECTIVE: Huntington's disease (HD) is an autosomal, dominantly inherited, neurodegenerative disease. The main clinical features are motor impairment, progressive cognitive deterioration and behavioral changes. The aim of our study was to find out whether patients with HD suffer from disorders of the auditory system. METHODS: A group of 17 genetically verified patients (11 males, 6 females) with various stages of HD (examined by UHDRS - motor part and total functional capacity, MMSE for cognitive functions) underwent an audiological examination (high frequency pure tone audiometry, otoacoustic emissions, speech audiometry, speech audiometry in babble noise, auditory brainstem responses). Additionally, 5 patients underwent a more extensive audiological examination, focused on central auditory processing. The results were compared with a group of age-matched healthy volunteers. RESULTS: Our results show that HD patients have physiologic hearing thresholds, otoacoustic emissions and auditory brainstem responses; however, they display a significant decrease in speech understanding, especially under demanding conditions (speech in noise) compared to age-matched controls. Additional auditory tests also show deficits in sound source localization, based on temporal and intensity cues. We also observed a statistically significant correlation between the perception of speech in noise, and motoric and cognitive functions. However, a correlation between genetic predisposition (number of triplets) and function of inner ear was not found. CONCLUSIONS: We conclude that HD negatively influences the function of the central part of the auditory system at cortical and subcortical levels, altering predominantly speech processing and sound source lateralization. SIGNIFICANCE: We have thoroughly characterized auditory pathology in patients with HD that suggests involvement of central auditory and cognitive areas.
References provided by Crossref.org
Functional Age-Related Changes Within the Human Auditory System Studied by Audiometric Examination
