{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T06:35:36Z","timestamp":1791959736024,"version":"4.3.4"},"reference-count":54,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,6,2]],"date-time":"2023-06-02T00:00:00Z","timestamp":1685664000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100010849","name":"Health and Family Planning Commission of Wuhan Municipality","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010849","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cell. Neurosci."],"abstract":"<jats:p>Hearing loss is the most common congenital sensory impairment. Mutations or deficiencies of the <jats:italic>GJB2<\/jats:italic> gene are the most common genetic cause of congenital non-syndromic deafness. Pathological changes such as decreased potential in the cochlea, active cochlear amplification disorders, cochlear developmental disorders and macrophage activation have been observed in various <jats:italic>GJB2<\/jats:italic> transgenic mouse models. In the past, researchers generally believed that the pathological mechanisms underlying <jats:italic>GJB2<\/jats:italic>-related hearing loss comprised a K<jats:sup>+<\/jats:sup> circulation defect and abnormal ATP-Ca<jats:sup>2+<\/jats:sup> signals. However, recent studies have shown that K<jats:sup>+<\/jats:sup> circulation is rarely associated with the pathological process of <jats:italic>GJB2<\/jats:italic>-related hearing loss, while cochlear developmental disorders and oxidative stress play an important, even critical, role in the occurrence of <jats:italic>GJB2<\/jats:italic>-related hearing loss. Nevertheless, these research has not been systematically summarized. In this review, we summarize the pathological mechanisms of <jats:italic>GJB2<\/jats:italic>-related hearing loss, including aspects of K<jats:sup>+<\/jats:sup> circulation, developmental disorders of the organ of Corti, nutrition delivery, oxidative stress and ATP-Ca<jats:sup>2+<\/jats:sup> signals. Clarifying the pathological mechanism of <jats:italic>GJB2<\/jats:italic>-related hearing loss can help develop new prevention and treatment strategies.<\/jats:p>","DOI":"10.3389\/fncel.2023.1208406","type":"journal-article","created":{"date-parts":[[2023,6,2]],"date-time":"2023-06-02T14:18:47Z","timestamp":1685715527000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":18,"title":["Research progress in delineating the pathological mechanisms of GJB2-related hearing loss"],"prefix":"10.3389","volume":"17","author":[{"given":"Yujun","family":"Wang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiong","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Xiong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiang","family":"Gu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shan","family":"Zeng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,6,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"18770","DOI":"10.1073\/pnas.0800793105","article-title":"ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear.","volume":"105","author":"Anselmi","year":"2008","journal-title":"Proc. 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