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We previously reported a progressive hearing loss (HL) mouse model, the\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl\/erl<\/jats:sup>\n                    <\/jats:italic>\n                    mouse, which carries a 208T &gt; C mutation causing an amino acid substitution at S70P in C57BL\/6J mice. To investigate the differences in\n                    <jats:italic>Cdh23<\/jats:italic>\n                    mutation\u2010related HL in different genetic backgrounds, we used the CRISPR\/Cas9 system to generate homozygous mice in the CBA\/CaJ background that have the same base pair missense mutation (208T &gt; C) (\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl2\/erl2<\/jats:sup>\n                    <\/jats:italic>\n                    ) as\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl\/erl<\/jats:sup>\n                    <\/jats:italic>\n                    mice in the C57BL\/6J background or a single base pair deletion (235G) (\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>V2J2\/V2J2<\/jats:sup>\n                    <\/jats:italic>\n                    ) in the\n                    <jats:italic>Cdh23<\/jats:italic>\n                    gene at exon 5. The two mutant mice exhibit hearing impairment across a broad range of frequencies. The progression of HL in\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl2\/erl2<\/jats:sup>\n                    <\/jats:italic>\n                    mice is slower than that in\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl\/erl<\/jats:sup>\n                    <\/jats:italic>\n                    mice. We also found structural abnormalities in the stereocilia of cochlear hair cells in\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl2\/erl2<\/jats:sup>\n                    <\/jats:italic>\n                    and\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>V2J2\/V2J2<\/jats:sup>\n                    <\/jats:italic>\n                    mice.\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>V2J2\/V2J2<\/jats:sup>\n                    <\/jats:italic>\n                    mice show signs of vestibular dysfunction in open field behavior and swimming tests. In addition, we observed hair bundle defects in vestibular hair cells in\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>V2J2\/V2J2<\/jats:sup>\n                    <\/jats:italic>\n                    mice. Our results suggest an interaction between the\n                    <jats:italic>erl<\/jats:italic>\n                    locus and the C57BL\/6J background that exacerbates HL in\n                    <jats:italic>\n                      Cdh23\n                      <jats:sup>erl\/erl<\/jats:sup>\n                    <\/jats:italic>\n                    mice. Moreover, our study confirms that the\n                    <jats:italic>Cdh23<\/jats:italic>\n                    gene is essential for normal hearing and balance. These two novel mutant mouse strains provide excellent models for studying\n                    <jats:italic>CDH23<\/jats:italic>\n                    mutation\u2010related deafness in humans.\n                  <\/jats:p>","DOI":"10.1002\/jnr.24905","type":"journal-article","created":{"date-parts":[[2021,6,16]],"date-time":"2021-06-16T14:54:20Z","timestamp":1623855260000},"page":"2743-2758","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Phenotypic differences in the inner ears of CBA\/CaJ and C57BL\/6J mice carrying missense and single base pair deletion mutations in the\n                    <i>Cdh23<\/i>\n                    gene"],"prefix":"10.1002","volume":"99","author":[{"given":"Tong","family":"Zhao","sequence":"first","affiliation":[{"name":"Hearing and Speech Rehabilitation Institute College of Special Education Binzhou Medical University Yantai 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