{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T14:39:15Z","timestamp":1774535955988,"version":"3.50.1"},"reference-count":19,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2016,8,1]],"date-time":"2016-08-01T00:00:00Z","timestamp":1470009600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100003708","name":"Cornelia de Lange Syndrome Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003708","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.fr","clinicalkey.com.au","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["International Journal of Pediatric Otorhinolaryngology"],"published-print":{"date-parts":[[2016,8]]},"DOI":"10.1016\/j.ijporl.2016.06.032","type":"journal-article","created":{"date-parts":[[2016,6,18]],"date-time":"2016-06-18T14:13:05Z","timestamp":1466259185000},"page":"203-207","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":13,"special_numbering":"C","title":["Improvement in hearing loss over time in Cornelia de Lange syndrome"],"prefix":"10.1016","volume":"87","author":[{"given":"Kevin C.","family":"Janek","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David F.","family":"Smith","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonie D.","family":"Kline","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James R.","family":"Benke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mei-Ling","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amy","family":"Kimball","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stacey L.","family":"Ishman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.ijporl.2016.06.032_bib1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/cge.12499","article-title":"Cornelia de Lange syndrome","volume":"88","author":"Boyle","year":"2015","journal-title":"Clin. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib2","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1002\/ajmg.1320220110","article-title":"The Brachmann-de Lange syndrome","volume":"22","author":"Opitz","year":"1985","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib3","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1002\/ajmg.a.32016","article-title":"Descriptive epidemiology of Cornelia de Lange syndrome in Europe","volume":"146A","author":"Barisic","year":"2008","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib4","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1002\/ajmg.1320470703","article-title":"De Lange syndrome: a clinical review of 310 individuals","volume":"47","author":"Jackson","year":"1993","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib5","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1002\/ajmg.c.30137","article-title":"Natural history of aging in Cornelia de Lange syndrome","volume":"145C","author":"Kline","year":"2007","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib6","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1086\/424698","article-title":"NIPBL mutational analysis in 120 individuals with Cornelia de Lange syndrome and evaluation of genotype-phenotype correlations","volume":"75","author":"Gillis","year":"2004","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib7","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1038\/ng1779","article-title":"X-linked Cornelia de Lange syndrome owing to SMC1L1 mutations","volume":"38","author":"Musio","year":"2006","journal-title":"Nat. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib8","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1136\/jmedgenet-2014-102573","article-title":"Genetic heterogeneity in Cornelia de Lange syndrome (CdLS) and CdLS-like phenotypes with observed and predicted levels of mosaicism","volume":"51","author":"Ansari","year":"2014","journal-title":"J.\u00a0Med. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib9","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1086\/511888","article-title":"Mutations in cohesin complex members SMC3 and SMC1A cause a mild variant of Cornelia de Lange syndrome with predominant mental retardation","volume":"80","author":"Deardorff","year":"2007","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib10","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/nature11316","article-title":"HDAC8 mutations in Cornelia de Lange syndrome affect the cohesin acetylation cycle","volume":"489","author":"Deardorff","year":"2012","journal-title":"Nature"},{"key":"10.1016\/j.ijporl.2016.06.032_bib11","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1172\/JCI77435","article-title":"Global transcriptional disturbances underlie Cornelia de Lange syndrome and related phenotypes","volume":"125","author":"Yuan","year":"2015","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.ijporl.2016.06.032_bib12","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1111\/j.1399-0004.2007.00832.x","article-title":"Clinical score of 62 Italian patients with Cornelia de Lange syndrome and correlations with the presence and type of NIPBL mutation","volume":"72","author":"Selicorni","year":"2007","journal-title":"Clin. Genet."},{"key":"10.1016\/j.ijporl.2016.06.032_bib13","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1001\/archotol.1990.01870090060008","article-title":"Cornelia de Lange syndrome. Otolaryngologic manifestations","volume":"116","author":"Sataloff","year":"1990","journal-title":"Arch. Otolaryngol. Head. Neck Surg."},{"key":"10.1016\/j.ijporl.2016.06.032_bib14","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.ijporl.2014.03.038","article-title":"Audiological findings, genotype and clinical severity score in Cornelia de Lange syndrome","volume":"78","author":"Marchisio","year":"2014","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"10.1016\/j.ijporl.2016.06.032_bib15","doi-asserted-by":"crossref","first-page":"569","DOI":"10.3174\/ajnr.A0888","article-title":"Temporal bone CT findings in Cornelia de Lange syndrome","volume":"29","author":"Kim","year":"2008","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"10.1016\/j.ijporl.2016.06.032_bib16","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1097\/00129492-200101000-00011","article-title":"Temporal bone pathology in Cornelia de Lange syndrome","volume":"22","author":"Yamanobe","year":"2001","journal-title":"Otol. Neurotol."},{"key":"10.1016\/j.ijporl.2016.06.032_bib17","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1093\/brain\/119.3.741","article-title":"Auditory neuropathy","volume":"119","author":"Starr","year":"1996","journal-title":"Brain"},{"key":"10.1016\/j.ijporl.2016.06.032_bib18","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/0165-5876(94)01078-C","article-title":"Auditory brainstem responses in children with Cornelia de Lange syndrome","volume":"31","author":"Kaga","year":"1995","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"10.1016\/j.ijporl.2016.06.032_bib19","unstructured":"http:\/\/www.cdlsusa.org\/."}],"container-title":["International Journal of Pediatric Otorhinolaryngology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0165587616301732?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0165587616301732?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,5,15]],"date-time":"2021-05-15T10:31:59Z","timestamp":1621074719000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0165587616301732"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,8]]},"references-count":19,"alternative-id":["S0165587616301732"],"URL":"https:\/\/doi.org\/10.1016\/j.ijporl.2016.06.032","relation":{},"ISSN":["0165-5876"],"issn-type":[{"value":"0165-5876","type":"print"}],"subject":[],"published":{"date-parts":[[2016,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Improvement in hearing loss over time in Cornelia de Lange syndrome","name":"articletitle","label":"Article Title"},{"value":"International Journal of Pediatric Otorhinolaryngology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ijporl.2016.06.032","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Elsevier Ireland Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}