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In this study, we characterized the auditory and vestibular phenotypes of\n                    <jats:italic>Myo6<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>sv\/sv<\/jats:italic>\n                    <\/jats:sup>\n                    mice. These mice exhibit profound hearing loss, with cochlear hair cell stereocilia beginning to fuse soon after birth, ultimately leading to disorganization of hair bundles and degeneration of hair cells. Mice also exhibit behavioral phenotypes characterized by severe imbalance, hyperactivity with bouts of circling, and delayed spatial learning of a novel environment, but preserved normal behavioral circadian rhythms. These behaviors emerge in association with the loss of the characteristic staircase morphology of vestibular hair cell stereocilia soon after birth and the subsequent profound elongation of the stereocilia. Adeno-associated virus (AAV) gene replacement therapy, delivered on the day of birth or one day after, failed to restore auditory or vestibular function. Our findings underscore the essential role of\n                    <jats:italic>Myo6<\/jats:italic>\n                    in the auditory and vestibular systems and imply prenatal intervention may be required for effective therapy.\n                  <\/jats:p>","DOI":"10.1007\/s00335-026-10263-y","type":"journal-article","created":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T15:31:08Z","timestamp":1787931068000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Assessment of auditory and vestibular function and gene therapy in the Snell\u2019s waltzer mouse model of human deafness and balance dysfunction"],"prefix":"10.1007","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-4331-533X","authenticated-orcid":false,"given":"Eyal","family":"Marton","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-5051-7208","authenticated-orcid":false,"given":"Roni","family":"Hahn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2275-1609","authenticated-orcid":false,"given":"Lior","family":"Bikovski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2825-6521","authenticated-orcid":false,"given":"Gwena\u00eblle S. 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