{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T18:32:38Z","timestamp":1759775558375,"version":"3.40.4"},"reference-count":29,"publisher":"American Academy of Audiology","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["j am acad audiol"],"published-print":{"date-parts":[[2024,1,1]]},"abstract":"<jats:p><jats:bold>Abstract<\/jats:bold>  <jats:bold>Background<\/jats:bold>\u2003Williams syndrome (WS) is a genetic, multisystemic, neurodevelopmental disorder, in which studies have demonstrated the presence of auditory deficits such as conductive and sensorineural hearing loss, cochlear weakness, and subclinical signs\n of hearing impairment. However, few studies have assessed this population's central auditory system. <jats:bold>Purpose<\/jats:bold>\u2003To analyze long-latency auditory evoked potential (LLAEP) in adults with WS and compare them with those obtained from neurotypical individuals with no hearing complaints.\n <jats:bold>Research Design<\/jats:bold>\u2003A cross-sectional observational study with subjects who were submitted to LLAEP assessment with the oddball paradigm and tone-burst stimuli. <jats:bold>Study Sample<\/jats:bold>\u2003A total of 30 individuals of both sexes, aged 18 to 37 years\u201415 had WS and comprised the\n study group (SG) and 15 did not have either the syndrome or hearing complaints and comprised the control group (CG); they were matched for sex and age. <jats:bold>Results<\/jats:bold>\u2003The LLAEP analysis showed larger P1\u2010N1 and P2\u2010N2 amplitudes but smaller N2\u2010P3 amplitude in SG,\n which also had delayed latencies in all components compared with CG. <jats:bold>Conclusion<\/jats:bold>\u2003Adults with WS had increased neuronal response in identifying stimulus characteristics, attentional difficulties in auditory tasks, and deficits in auditory information processing speed.<\/jats:p>","DOI":"10.1055\/s-0044-1790280","type":"journal-article","created":{"date-parts":[[2024,11,29]],"date-time":"2024-11-29T00:25:58Z","timestamp":1732839958000},"page":"24-29","source":"Crossref","is-referenced-by-count":1,"title":["Long-Latency Auditory Evoked Potentials in Adults with Williams Syndrome"],"prefix":"10.3766","volume":"35","author":[{"given":"Jacqueline A.","family":"Nascimento","sequence":"first","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapy of the Medical School at the University of Sao Paulo, S\u00e3o Paulo, S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liliane A.F.","family":"Silva","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapy of the Medical School at the University of Sao Paulo, S\u00e3o Paulo, S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra G.","family":"Samelli","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapy of the Medical School at the University of Sao Paulo, S\u00e3o Paulo, S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla G.","family":"Matas","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapy of the Medical School at the University of Sao Paulo, S\u00e3o Paulo, S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2087","reference":[{"issue":"02","key":"ref1","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/S0022-3476(88)80272-5","article-title":"Natural history of Williams syndrome: physical characteristics","volume":"113","author":"C A Morris","year":"1988","journal-title":"J Pediatr"},{"issue":"04","key":"ref2","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1177\/088307380201700406","article-title":"Prevalence estimation of Williams syndrome","volume":"17","author":"P Str\u00f8mme","year":"2002","journal-title":"J Child Neurol"},{"issue":"05","key":"ref3","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1111\/j.1469-7610.2004.00376.x","article-title":"Aversion, awareness, and attraction: investigating claims of hyperacusis in the Williams syndrome phenotype","volume":"46","author":"D J Levitin","year":"2005","journal-title":"J Child Psychol Psychiatry"},{"issue":"02","key":"ref4","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/j.jpeds.2010.07.056","article-title":"A comparative study of hearing loss in two microdeletion syndromes: velocardiofacial (22q11.2 deletion) and Williams (7q11.23 deletion) syndromes","volume":"158","author":"O Zarchi","year":"2011","journal-title":"J Pediatr"},{"issue":"02","key":"ref5","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1002\/ajmg.c.30262","article-title":"Auditory function and hearing loss in children and adults with Williams syndrome: cochlear impairment in individuals with otherwise normal hearing","volume":"154C","author":"J A Marler","year":"2010","journal-title":"Am J Med Genet C Semin Med Genet"},{"issue":"04","key":"ref6","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1002\/ajmg.a.30970","article-title":"Sensorineural hearing loss in children and adults with Williams syndrome","volume":"138","author":"J A Marler","year":"2005","journal-title":"Am J Med Genet A"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/j.heares.2010.10.004","article-title":"Cochlear active mechanisms in young normal-hearing subjects affected by Williams syndrome: time-frequency analysis of otoacoustic emissions","volume":"272","author":"A Paglialonga","year":"2011","journal-title":"Hear Res"},{"issue":"04","key":"ref8","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1002\/ajmg.a.35241","article-title":"Audiological findings in Williams syndrome: a study of 69 patients","volume":"158A","author":"S Barozzi","year":"2012","journal-title":"Am J Med Genet A"},{"issue":"03","key":"ref9","first-page":"e20160107","article-title":"Maturation of long latency auditory evoked potentials in hearing children: systematic review","volume":"29","author":"L AF Silva","year":"2017","journal-title":"CoDAS"},{"issue":"02","key":"ref10","doi-asserted-by":"crossref","first-page":"90","DOI":"10.2310\/7070.2001.20811","article-title":"Hyperacusis in Williams syndrome","volume":"30","author":"L B Johnson","year":"2001","journal-title":"J Otolaryngol"},{"issue":"04","key":"ref11","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1080\/09297040490909288","article-title":"Characterizing the musical phenotype in individuals with Williams syndrome","volume":"10","author":"D J Levitin","year":"2004","journal-title":"Child Neuropsychol"},{"issue":"03","key":"ref12","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1212\/01.wnl.0000196643.35395.5f","article-title":"Hyperacusis in Williams syndrome: characteristics and associated neuroaudiologic abnormalities","volume":"66","author":"D Gothelf","year":"2006","journal-title":"Neurology"},{"volume-title":"Late potentials of the auditory system (evoked potentials)","year":"1996","author":"D L McPherson","key":"ref13"},{"volume-title":"New handbook of auditory evoked responses","year":"2006","author":"J Hall","key":"ref14"},{"key":"ref15","first-page":"482","volume-title":"Principles and applications of cortical auditory evoked potentials","author":"B E Martin","year":"2007"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.bandc.2018.01.005","article-title":"Attentional rather than sensory differences characterize auditory processing in Williams syndrome","volume":"121","author":"M Jacobs","year":"2018","journal-title":"Brain Cogn"},{"issue":"04","key":"ref17","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1001\/archotol.1970.04310040005002","article-title":"Clinical experience with impedance audiometry","volume":"92","author":"J Jerger","year":"1970","journal-title":"Arch Otolaryngol"},{"volume-title":"The National health survey: hearing studies series. Bulletin 2","author":"W Beasley","key":"ref18"},{"key":"ref19","first-page":"371","article-title":"The ten-twenty electrode system of the International Federation","volume":"10","author":"H H Jasper","year":"1958","journal-title":"Eletroenceph Clin Neurophysiol"},{"key":"ref20","volume-title":"Estat\u00edstica B\u00e1sica","author":"W O Bussab","year":"2010","edition":"6th Ed."},{"key":"ref21","first-page":"816","volume-title":"Discovering statistics using IBM SPSS statistics","author":"A Field","year":"2018","edition":"5th ed."},{"issue":"03","key":"ref22","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1002\/ajmg.a.30400","article-title":"Multisystem study of 20 older adults with Williams syndrome","volume":"131","author":"E M Cherniske","year":"2004","journal-title":"Am J Med Genet A"},{"issue":"09","key":"ref23","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/j.ejmg.2013.07.001","article-title":"Audiological follow-up of 24 patients affected by Williams syndrome","volume":"56","author":"S Barozzi","year":"2013","journal-title":"Eur J Med Genet"},{"issue":"01","key":"ref24","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.ridd.2010.09.011","article-title":"Abnormal processing of emotional prosody in Williams syndrome: an event-related potentials study","volume":"32","author":"A P Pinheiro","year":"2011","journal-title":"Res Dev Disabil"},{"issue":"03","key":"ref25","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1515\/REVNEURO.2003.14.3.233","article-title":"Regulation of spine morphology and synaptic function by LIMK and the actin cytoskeleton","volume":"14","author":"Y Meng","year":"2003","journal-title":"Rev Neurosci"},{"issue":"01","key":"ref26","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/S0896-6273(02)00758-4","article-title":"Abnormal spine morphology and enhanced LTP in LIMK-1 knockout mice","volume":"35","author":"Y Meng","year":"2002","journal-title":"Neuron"},{"issue":"02","key":"ref27","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1002\/bies.10402","article-title":"LIMK1 and CLIP-115: linking cytoskeletal defects to Williams syndrome","volume":"26","author":"C C Hoogenraad","year":"2004","journal-title":"BioEssays"},{"issue":"08","key":"ref28","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1080\/87565641.2013.825617","article-title":"Genetic mapping of brain plasticity across development in Williams syndrome: ERP markers of face and language processing","volume":"38","author":"D L Mills","year":"2013","journal-title":"Dev Neuropsychol"},{"issue":"06","key":"ref29","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1111\/psyp.12407","article-title":"Hyperactive auditory processing in Williams syndrome: evidence from auditory evoked potentials","volume":"52","author":"O Zarchi","year":"2015","journal-title":"Psychophysiology"}],"container-title":["Journal of the American Academy of Audiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ingentaconnect.com\/content\/aaa\/jaaa\/2024\/00000035\/f0020001\/art00003","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,17]],"date-time":"2025-04-17T04:31:07Z","timestamp":1744864267000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ingentaconnect.com\/content\/10.1055\/s-0044-1790280"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,1]]},"references-count":29,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,1,1]]}},"alternative-id":["1050-0545(20240101)35:1L.24;1-"],"URL":"https:\/\/doi.org\/10.1055\/s-0044-1790280","relation":{},"ISSN":["1050-0545"],"issn-type":[{"type":"print","value":"1050-0545"}],"subject":[],"published":{"date-parts":[[2024,1,1]]}}}