{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,30]],"date-time":"2026-08-30T21:04:14Z","timestamp":1788123854073,"version":"build-2803163510"},"reference-count":13,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2016,11,15]],"date-time":"2016-11-15T00:00:00Z","timestamp":1479168000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["Z01\u2010DC\u2010000060\u201013"],"award-info":[{"award-number":["Z01\u2010DC\u2010000060\u201013"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["Z01\u2010DC\u2010000064\u201013"],"award-info":[{"award-number":["Z01\u2010DC\u2010000064\u201013"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["Z01\u2010DC\u2010000082\u201002"],"award-info":[{"award-number":["Z01\u2010DC\u2010000082\u201002"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives\/Hypothesis<\/jats:title>\n                    <jats:p>\n                      To characterize the severity and natural history of hearing loss, and the prevalence of having a cochlear implant in a maturing cohort of individuals with enlarged vestibular aqueduct (EVA) and zero or one mutant allele of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Study Design<\/jats:title>\n                    <jats:p>Prospective cohort study of subjects ascertained between 1998 and 2015 at the National Institutes of Health Clinical Center.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Study subjects were 127 individuals (median age, 8 years; range, 0\u201359 years) with EVA in at least one ear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Ears with EVA and zero or one mutant allele of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      had mean 0.5\/1\/2\/4\u2010kHz pure\u2010tone averages of 62.6 and 52.9 dB HL, respectively, in contrast to EVA ears with two mutant alleles of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      (88.1 dB HL;\n                      <jats:italic>P<\/jats:italic>\n                      &lt; .01). This association was independent of age, sex, or side of EVA (\n                      <jats:italic>P<\/jats:italic>\n                      &lt; .001). Natural history of hearing loss was not associated with number of mutant alleles (\n                      <jats:italic>P<\/jats:italic>\n                      = .94). The prevalence of having a cochlear implant was nine (12%) of 76, two (13%) of 15, and 12 (38%) of 32 subjects with zero, one, and two mutant alleles, respectively (\n                      <jats:italic>P<\/jats:italic>\n                      = .00833). This association was not independent (\n                      <jats:italic>P<\/jats:italic>\n                      = .534) but reflected underlying correlations with age at time of first audiogram (\n                      <jats:italic>P<\/jats:italic>\n                      = .003) or severity of hearing loss (\n                      <jats:italic>P<\/jats:italic>\n                      = .000).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Ears with EVA and zero or one mutant allele of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      have less severe hearing loss, no difference in prevalence of fluctuation, and a lower prevalence of cochlear implantation in comparison to ears with two mutant alleles of\n                      <jats:italic>SLC26A4<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Level of Evidence<\/jats:title>\n                    <jats:p>\n                      NA\n                      <jats:italic>Laryngoscope<\/jats:italic>\n                      , 127:E238\u2013E243, 2017\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lary.26418","type":"journal-article","created":{"date-parts":[[2016,11,16]],"date-time":"2016-11-16T12:36:37Z","timestamp":1479299797000},"source":"Crossref","is-referenced-by-count":31,"title":["Hearing loss associated with enlarged vestibular aqueduct and zero or one mutant allele of\n                    <i>SLC26A4<\/i>"],"prefix":"10.1002","volume":"127","author":[{"given":"Jane","family":"Rose","sequence":"first","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie A.","family":"Muskett","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly A.","family":"King","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher K.","family":"Zalewski","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Parna","family":"Chattaraj","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Butman","sequence":"additional","affiliation":[{"name":"Radiology and Imaging Sciences, Clinical Center National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret A.","family":"Kenna","sequence":"additional","affiliation":[{"name":"Department of Otology and Laryngology Harvard Medical School Boston Massachusetts"},{"name":"Department of Otolaryngology and Communication Enhancement Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wade W.","family":"Chien","sequence":"additional","affiliation":[{"name":"Office of the Clinical Director National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen C.","family":"Brewer","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew J.","family":"Griffith","sequence":"additional","affiliation":[{"name":"Otolaryngology Branch National Institute on Deafness and Other Communication Disorders, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,11,15]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.otohns.2006.12.011"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)74403-0"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2004.024208"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20884"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2013.4185"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2009.067892"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201611"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-007-0415-2"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.25079"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2004.00386.x"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.20722"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.25737"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1177\/0194599812458401"}],"container-title":["The Laryngoscope"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.26418","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flary.26418","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lary.26418","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T02:09:35Z","timestamp":1696558175000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/lary.26418"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,11,15]]},"references-count":13,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2017,7]]}},"alternative-id":["10.1002\/lary.26418"],"URL":"https:\/\/doi.org\/10.1002\/lary.26418","archive":["Portico"],"relation":{},"ISSN":["0023-852X","1531-4995"],"issn-type":[{"value":"0023-852X","type":"print"},{"value":"1531-4995","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,11,15]]}}}