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Methods and technologies for viral delivery of genes into the inner ear have evolved over the past decade to render gene therapy a viable and attractive approach for treatment. Variants in\n                    <jats:italic>SYNE4<\/jats:italic>\n                    , encoding the protein nesprin\u20104, a member of the linker of nucleoskeleton and cytoskeleton (LINC), lead to DFNB76 human deafness.\n                    <jats:italic>Syne4<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice have severe\u2010to\u2010profound progressive hearing loss and exhibit mislocalization of hair cell nuclei and hair cell degeneration. We used AAV9\u2010PHP.B, a recently developed synthetic adeno\u2010associated virus, to deliver the coding sequence of\n                    <jats:italic>Syne4<\/jats:italic>\n                    into the inner ears of neonatal\n                    <jats:italic>Syne4<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice. Here we report rescue of hair cell morphology and survival, nearly complete recovery of auditory function, and restoration of auditory\u2010associated behaviors, without observed adverse effects. Uncertainties remain regarding the durability of the treatment and the time window for intervention in humans, but our results suggest that gene therapy has the potential to prevent hearing loss in humans with\n                    <jats:italic>SYNE4<\/jats:italic>\n                    mutations.\n                  <\/jats:p>","DOI":"10.15252\/emmm.202013259","type":"journal-article","created":{"date-parts":[[2020,12,22]],"date-time":"2020-12-22T17:09:11Z","timestamp":1608656951000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":65,"title":["Neonatal AAV gene therapy rescues hearing in a mouse model of SYNE4 deafness"],"prefix":"10.1038","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0787-4216","authenticated-orcid":false,"given":"Shahar","family":"Taiber","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roie","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1953-1982","authenticated-orcid":false,"given":"Ofer","family":"Yizhar\u2010Barnea","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6776-6957","authenticated-orcid":false,"given":"David","family":"Sprinzak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7182-8011","authenticated-orcid":false,"given":"Jeffrey R","family":"Holt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4913-251X","authenticated-orcid":false,"given":"Karen B","family":"Avraham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,12,22]]},"reference":[{"key":"BFEMMM202013259_CR1","doi-asserted-by":"crossref","first-page":"4496","DOI":"10.1073\/pnas.1817537116","volume":"116","author":"O Akil","year":"2019","unstructured":"Akil O, Dyka F, Calvet C, Emptoz A, Lahlou G, Nouaille S, Boutet de Monvel J, Hardelin JP, Hauswirth WW, Avan P et al (2019) Dual AAV\u2010mediated gene therapy restores hearing in a DFNB9 mouse model. 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The authors declare no other conflict of interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}],"article-number":"EMMM202013259"}}