Auditory dysfunction in patients with Huntington's disease

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064173%3A_____%2F17%3AN0000223" target="_blank" >RIV/00064173:_____/17:N0000223 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/68378041:_____/17:00478070 RIV/67985904:_____/17:00478070 RIV/00216208:11110/17:10365414 RIV/00216208:11120/17:43915730 a 3 dalších

Výsledek na webu

<a href="https://doi.org/10.1016/j.clinph.2017.07.403" target="_blank" >https://doi.org/10.1016/j.clinph.2017.07.403</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.clinph.2017.07.403" target="_blank" >10.1016/j.clinph.2017.07.403</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Auditory dysfunction in patients with Huntington's disease

Popis výsledku v původním jazyce

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. 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. 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. 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.

Název v anglickém jazyce

Auditory dysfunction in patients with Huntington's disease

Popis výsledku anglicky

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. 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. 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. 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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30210 - Clinical neurology

Projekt

—

Návaznosti

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Rok uplatnění

2017

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Clinical Neurophysiology

ISSN

1388-2457

e-ISSN

1872-8952

Svazek periodika

128

Číslo periodika v rámci svazku

10

Stát vydavatele periodika

IE - Irsko

Počet stran výsledku

8

Strana od-do

1946-1953

Kód UT WoS článku

000415787900017

EID výsledku v databázi Scopus

2-s2.0-85027509345