{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T17:03:41Z","timestamp":1791133421535,"version":"4.1.0"},"reference-count":31,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2011,3,15]],"date-time":"2011-03-15T00:00:00Z","timestamp":1300147200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J intellect Disabil Res"],"published-print":{"date-parts":[[2011,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2003<\/jats:bold> Williams Syndrome (WS) is a neurodevelopmental disorder of genetic origin, characterised by relative proficiency in language in the face of serious impairment in several other domains. Individuals with WS display an unusual sensitivity to noise, known as hyperacusis.<\/jats:p><jats:p><jats:bold>Methods\u2003<\/jats:bold> In this study, we examined the extent to which hyperacusis interferes with the perception of speech in children and adults with WS. Participants were required to discriminate words which differed in one consonant of a cluster when these contrasts were embedded in a background of noise.<\/jats:p><jats:p><jats:bold>Results\u2003<\/jats:bold> Although the introduction of noise interfered with performance on a consonant cluster discrimination task equally in the WS and control groups, the severity of hyperacusis significantly predicted individual variability in speech perception within the WS group.<\/jats:p><jats:p><jats:bold>Conclusions\u2003<\/jats:bold> These results suggest that alterations in sensitivity to input mediate atypical pathways for language development in WS, where hyperacusis exerts an important influence together with other non\u2010auditory factors.<\/jats:p>","DOI":"10.1111\/j.1365-2788.2011.01411.x","type":"journal-article","created":{"date-parts":[[2011,3,16]],"date-time":"2011-03-16T02:35:29Z","timestamp":1300242929000},"page":"563-571","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Severity of hyperacusis predicts individual differences in speech perception in Williams Syndrome"],"prefix":"10.1111","volume":"55","author":[{"given":"M.","family":"Elsabbagh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Rosen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Karmiloff\u2010Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,3,15]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1044\/jshr.3701.205"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-0965(03)00026-2"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-199705260-00025"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0166-2236(99)01397-1"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ridd.2005.09.002"},{"key":"e_1_2_6_7_1","unstructured":"CohenM. ElsabbaghM. RosenS. WittonC. RipponG.&Karmiloff\u2010SmithA.(2005)Peripheral auditory function does not explain hyperacusis in Williams syndrome: preliminary data from a hyperacusis questionnaire and audiometric assessment. Poster session presented at the European Federation of Audiological Societies Guttenberg."},{"key":"e_1_2_6_8_1","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1002\/1096-8628(200022)97:2<164::AID-AJMG8>3.0.CO;2-F","article-title":"Williams syndrome: from genotype through to the cognitive phenotype","volume":"97","author":"Donnai D.","year":"2000","journal-title":"American Journal of Medical Genetics: Seminars in Medical Genetics"},{"key":"e_1_2_6_9_1","volume-title":"British Picture Vocabulary Scales","author":"Dunn L. M.","year":"1997"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000196643.35395.5f"},{"key":"e_1_2_6_11_1","first-page":"615","article-title":"Is the language of people with Williams syndrome mere mimicry? Verbal short\u2010term memory in a foreign language","volume":"15","author":"Grant J.","year":"1996","journal-title":"Cahiers de Psychologie Cognitive"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-200108280-00021"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:JADD.0000018078.82542.ab"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0028-3932(98)00128-6"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1364-6613(98)01230-3"},{"key":"e_1_2_6_16_1","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1207\/S15326942DN231&2_10","article-title":"Dethroning the myth: cognitive dissociations & innate modularity in Williams syndrome","volume":"23","author":"Karmiloff\u2010Smith A.","year":"2003","journal-title":"Developmental Neuropsychology"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7610.2004.00322.x"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1044\/jshd.5502.339"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/1469-7610.00769"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-7610.2004.00376.x"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30970"},{"key":"e_1_2_6_22_1","unstructured":"MarriageJ.(1995)Hyperacusis in Williams syndrome. Doctoral thesis University of Manchester UK."},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1023\/B:JADD.0000018069.69562.b8"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1162\/089892900561977"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1207\/S15327078IN0402_06"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1044\/jshr.3403.643"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1467-7687.2004.00327.x"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1080\/01690960042000021"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1080\/01688639108401040"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1996.tb15062.x"},{"key":"e_1_2_6_31_1","first-page":"121","article-title":"Hyperacusis in Williams syndrome: a sample survey study","volume":"8","author":"Van Borsel J.","year":"1997","journal-title":"Genetic Counseling"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.aud.0000151575.58269.19"}],"container-title":["Journal of Intellectual Disability Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2788.2011.01411.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-2788.2011.01411.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,15]],"date-time":"2023-10-15T03:09:21Z","timestamp":1697339361000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-2788.2011.01411.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,3,15]]},"references-count":31,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2011,6]]}},"alternative-id":["10.1111\/j.1365-2788.2011.01411.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-2788.2011.01411.x","archive":["Portico"],"relation":{},"ISSN":["0964-2633","1365-2788"],"issn-type":[{"value":"0964-2633","type":"print"},{"value":"1365-2788","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,3,15]]},"assertion":[{"value":"2011-03-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}