• Je něco špatně v tomto záznamu ?

Auditory dysfunction in patients with Huntington's disease

O. Profant, J. Roth, Z. Bureš, Z. Balogová, I. Lišková, J. Betka, J. Syka,

. 2017 ; 128 (10) : 1946-1953. [pub] 20170729

Jazyk angličtina Země Nizozemsko

Typ dokumentu časopisecké články, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc18016409

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.

Citace poskytuje Crossref.org

000      
00000naa a2200000 a 4500
001      
bmc18016409
003      
CZ-PrNML
005      
20180521113147.0
007      
ta
008      
180515s2017 ne f 000 0|eng||
009      
AR
024    7_
$a 10.1016/j.clinph.2017.07.403 $2 doi
035    __
$a (PubMed)28826025
040    __
$a ABA008 $b cze $d ABA008 $e AACR2
041    0_
$a eng
044    __
$a ne
100    1_
$a Profant, Oliver $u Department 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. Electronic address: profant@biomed.cas.cz.
245    10
$a Auditory dysfunction in patients with Huntington's disease / $c O. Profant, J. Roth, Z. Bureš, Z. Balogová, I. Lišková, J. Betka, J. Syka,
520    9_
$a 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.
650    _2
$a dospělí $7 D000328
650    _2
$a senioři $7 D000368
650    _2
$a audiometrie slovní $x metody $7 D001302
650    _2
$a sluchový práh $x fyziologie $7 D001309
650    _2
$a sluchové kmenové evokované potenciály $x fyziologie $7 D016057
650    _2
$a ženské pohlaví $7 D005260
650    _2
$a centrální nedoslýchavost $x diagnóza $x etiologie $x patofyziologie $7 D006313
650    _2
$a lidé $7 D006801
650    _2
$a Huntingtonova nemoc $x komplikace $x diagnóza $x patofyziologie $7 D006816
650    _2
$a mužské pohlaví $7 D008297
650    _2
$a lidé středního věku $7 D008875
650    _2
$a percepce řeči $x fyziologie $7 D013067
655    _2
$a časopisecké články $7 D016428
655    _2
$a práce podpořená grantem $7 D013485
700    1_
$a Roth, Jan $u Department of Neurology and Center of Clinical Neuroscience, 1(st) Faculty of Medicine, Charles University, General University Hospital, Prague, Czech Republic.
700    1_
$a Bureš, Zbyněk $u Department of Auditory Neuroscience, Institute of Experimental Medicine, The Czech Academy of Sciences, Prague, Czech Republic; Czech Institute of Informatics, Robotics and Cybernetics, Czech Technical University in Prague, Prague, Czech Republic.
700    1_
$a Balogová, Zuzana $u Department of Auditory Neuroscience, Institute of Experimental Medicine, The Czech Academy of Sciences, Prague, Czech Republic; Department of Otorhinolaryngology of University Hospital Královské Vinohrady and 3(rd) Faculty of Medicine, Charles University, Prague, Czech Republic.
700    1_
$a Lišková, Irena $u Department of Neurology and Center of Clinical Neuroscience, 1(st) Faculty of Medicine, Charles University, General University Hospital, Prague, Czech Republic; Institute of Animal Physiology and Genetics, The Czech Academy of Sciences, Liběchov, Czech Republic.
700    1_
$a Betka, Jan $u Department of Otorhinolaryngology and Head and Neck Surgery, 1(st) Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czech Republic.
700    1_
$a Syka, Josef $u Department of Auditory Neuroscience, Institute of Experimental Medicine, The Czech Academy of Sciences, Prague, Czech Republic.
773    0_
$w MED00005214 $t Clinical neurophysiology official journal of the International Federation of Clinical Neurophysiology $x 1872-8952 $g Roč. 128, č. 10 (2017), s. 1946-1953
856    41
$u https://pubmed.ncbi.nlm.nih.gov/28826025 $y Pubmed
910    __
$a ABA008 $b sig $c sign $y a $z 0
990    __
$a 20180515 $b ABA008
991    __
$a 20180521113329 $b ABA008
999    __
$a ok $b bmc $g 1300033 $s 1013249
BAS    __
$a 3
BAS    __
$a PreBMC
BMC    __
$a 2017 $b 128 $c 10 $d 1946-1953 $e 20170729 $i 1872-8952 $m Clinical neurophysiology $n Clin Neurophysiol $x MED00005214
LZP    __
$a Pubmed-20180515

Najít záznam

Citační ukazatele

    Možnosti archivace