behavior/neurological
• at 3 and 6 months, mutants are unable to swim with their heads above water
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hearing/vestibular/ear
• at P17, cochlear supporting cells are proliferating but in wild-type no proliferation is occurring at this age
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• in the basal turn, the tunnel of corti has collapsed in 6-week old animals
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• at P1 cochlear hair cells are still undergoing proliferation but in wild-type no proliferation is occurring at this age
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• more inner hair cells than wild-type
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• many outer hair cells lack bundles; atypical bundles including ring-shaped bundles and 2 bundles within one hair cell are occasionally observed
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• more outer hair cells than wild-type
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• total hair cell loss by 3 months of age
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• from P6 to P17, there is apoptosis in the outer hair cell region of the organ of corti
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• at P17, utrical hair cells are proliferating but in wild-type no proliferation is occurring at this age
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• numbers of vestibular hair cells decrease from early postnatal to adult stages
• at P6, there are three to four layers of hair cells in the utricle, but at 3 months, only 1-2 layers remain
• by 6 months, there are thin and disorganized hair bundles in most hair cells in the utricles
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• at 3 and 6 months, mutants display moderate circling behavior and can not swim with their heads above water
• at 6 months, partial vestibular function remains in mutants with hair cell and behavioral abnormalities
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nervous system
• at P1 cochlear hair cells are still undergoing proliferation but in wild-type no proliferation is occurring at this age
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• more inner hair cells than wild-type
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• many outer hair cells lack bundles; atypical bundles including ring-shaped bundles and 2 bundles within one hair cell are occasionally observed
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• more outer hair cells than wild-type
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• total hair cell loss by 3 months of age
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• from P6 to P17, there is apoptosis in the outer hair cell region of the organ of corti
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• at P17, utrical hair cells are proliferating but in wild-type no proliferation is occurring at this age
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• numbers of vestibular hair cells decrease from early postnatal to adult stages
• at P6, there are three to four layers of hair cells in the utricle, but at 3 months, only 1-2 layers remain
• by 6 months, there are thin and disorganized hair bundles in most hair cells in the utricles
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cellular
• at P17, cochlear supporting cells are proliferating but in wild-type no proliferation is occurring at this age
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