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Mice with heterozygous <jats:italic>Chd7<\/jats:italic> deficiency have circling behavior and semicircular canal defects and are an excellent animal model for exploring the pathogenesis of CHARGE features. Inner ear vestibular defects have been characterized in heterozygous <jats:italic>Chd7<\/jats:italic>\u2010deficient embryos and early postnatal mice, but it is not known whether vestibular defects persist throughout adulthood in <jats:italic>Chd7<\/jats:italic>\u2010deficient mice or whether the vestibular sensory epithelia and their associated innervation and function are intact. Here we describe a detailed analysis of inner ear vestibular structures in mature mice that are heterozygous for a <jats:italic>Chd7<\/jats:italic>\u2010deficient, gene\u2010trapped allele (<jats:italic>Chd7<\/jats:italic><jats:sup><jats:italic>Gt\/+<\/jats:italic><\/jats:sup>). <jats:italic>Chd7<\/jats:italic><jats:sup><jats:italic>Gt\/+<\/jats:italic><\/jats:sup> mice display variable asymmetric lateral and posterior semicircular canal malformations, as well as defects in vestibular sensory epithelial innervation despite the presence of intact hair cells in the target organs. These observations have important functional implications for understanding the clinical manifestations of <jats:italic>CHD7<\/jats:italic> mutations in humans and for designing therapies to treat inner ear vestibular dysfunction. J. Comp. Neurol. 504:519\u2013532, 2007. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/cne.21460","type":"journal-article","created":{"date-parts":[[2007,8,13]],"date-time":"2007-08-13T16:47:46Z","timestamp":1187023666000},"page":"519-532","source":"Crossref","is-referenced-by-count":56,"title":["Defects in vestibular sensory epithelia and innervation in mice with loss of <i>Chd7<\/i> function: Implications for human CHARGE syndrome"],"prefix":"10.1002","volume":"504","author":[{"given":"Meredith E.","family":"Adams","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Hurd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa A.","family":"Beyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald 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