{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T05:13:03Z","timestamp":1788757983344,"version":"build-2803163510"},"reference-count":67,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T00:00:00Z","timestamp":1788652800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T00:00:00Z","timestamp":1788652800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["advanced.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Advanced Science"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Identifying new genes responsible for non\u2010syndromic hearing loss remains a critical goal as many patients still lack a molecular diagnosis despite comprehensive genetic testing. The tectorial membrane (TM) is a specialized acellular matrix of the inner ear, essential for stimulating mechanosensitive hair cell stereocilia bundles and maintaining frequency tuning and auditory sensitivity. Although mutations in genes encoding several non\u2010collagenous proteins found in the TM (TECTA, CEACAM16, OTOG, OTOGL) have been identified as deafness genes, definitive evidence implicating \u03b2\u2010tectorin (TECTB) has been lacking. Here, we present multiple lines of genetic and experimental evidence linking heterozygous missense variants in\n                    <jats:italic>TECTB<\/jats:italic>\n                    (NM_058222.3:c.674G&gt;A, p.Cys225Tyr and NM_058222.3:c.853C&gt;T, p.Arg285Cys), with hearing loss. Each variant affects highly conserved residues within or directly flanking the zona pellucida domain. Using a\n                    <jats:italic>Tectb\u2010C225Y<\/jats:italic>\n                    knock\u2010in mouse model, we show that homozygous animals exhibit severe hearing loss and profound disruption of TM morphology, while heterozygous animals display decreased staining density within the TM and increased susceptibility to noise\u2010induced hearing loss, despite normal auditory thresholds. These findings identify\n                    <jats:italic>TECTB<\/jats:italic>\n                    as a novel human deafness gene, further elucidate its contribution to maintaining TM integrity and resilience against environmentally\u2010related auditory decline.\n                  <\/jats:p>","DOI":"10.1002\/advs.77543","type":"journal-article","created":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T05:03:24Z","timestamp":1788757404000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["TECTB Variants Reveal Tectorial Membrane Vulnerability in Dominant Non\u2010Syndromic Hearing Loss"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2781-0412","authenticated-orcid":false,"given":"Evan B.","family":"Hale","sequence":"first","affiliation":[{"name":"Mass Eye and Ear Eaton Peabody Laboratories  Boston Massachusetts USA"},{"name":"Department of Otolaryngology Head and Neck Surgery Harvard Medical School  Boston Massachusetts USA"},{"name":"Speech and Hearing Bioscience and Technology Program Harvard University  Cambridge Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6719-3447","authenticated-orcid":false,"given":"Barbara","family":"Vona","sequence":"additional","affiliation":[{"name":"Institute for Auditory Neuroscience and InnerEarLab University Medical Center G\u00f6ttingen  G\u00f6ttingen Germany"},{"name":"Auditory Neuroscience and Optogenetics Laboratory German Primate Center  G\u00f6ttingen Germany"},{"name":"Collaborative Research Center 1690 (CRC1690) University of G\u00f6ttingen  G\u00f6ttingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Goodyear","sequence":"additional","affiliation":[{"name":"Sussex Neuroscience School of Life Sciences University of Sussex  Falmer, Brighton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0347-9614","authenticated-orcid":false,"given":"Richard T.","family":"Osgood","sequence":"additional","affiliation":[{"name":"Mass Eye and Ear Eaton Peabody Laboratories  Boston Massachusetts USA"},{"name":"Department of Otolaryngology Head and Neck Surgery Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Department of Pathology Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sami S.","family":"Amr","sequence":"additional","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Department of Pathology Harvard Medical School  Boston Massachusetts USA"},{"name":"Laboratory for Molecular Medicine Mass General Brigham Personalized Medicine  Cambridge Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2210-5586","authenticated-orcid":false,"given":"Karen","family":"Mojica","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology Vivian Pellas Hospital  Managua Nicaragua"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela A. 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