hearing/vestibular/ear
• the stereocilia degenerate shortly after birth
• the stereociliary degeneration appeared consistently more advanced in inner hair cells than that of outer hair cells
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• although stereocilia bundles were present at birth, the length and width of stereocilia at P0 were reduced
• the stereocilia on outer hair cells were significantly shorter
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• at P0
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• ABR assessment on homozygotes at 5 to 6 weeks of age shows no signs of hearing
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• VESPs are absent at the maximum stimulus intensity used
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behavior/neurological
• abnormal drop reflex; mice do not demonstrate expected dorsoflexion and spread out the front paws when quickly lowered from ~20 cm above a table surface, while controls do exhibit this behavior
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• mice exhibit poor swimming ability; mice can not maneuver in the water and can not remain at the surface
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nervous system
• the stereocilia degenerate shortly after birth
• the stereociliary degeneration appeared consistently more advanced in inner hair cells than that of outer hair cells
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• although stereocilia bundles were present at birth, the length and width of stereocilia at P0 were reduced
• the stereocilia on outer hair cells were significantly shorter
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• at P0
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