Analysis Tools
hearing/vestibular/ear
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• at 6 months of age, mice exhibit normal hearing (as measured by ABR thresholds) and no overt vestibular deficits (as measured by vestibular evoked potential thresholds)
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• at P4, stereocilia in the organ of Corti appear normal, except for transiently elongated microvillar-like protrusions on the outer hair cell (OHC) surface, opposite to the hair bundle; however, extraneous protrusions disappear from OHCs by P10
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• in the extrastriolar region, vestibular hair bundles show a striking reduction in staircase slope, due to both a shortening of the tallest row of stereocilia and elongation of shorter rows; in the utricle, extrastriolar stereocilia are of nearly uniform length
• length difference between the tallest and shortest stereocilia in extrastriolar bundles is reduced by >80%
• MYO3B is no longer detectable at stereocilia tips of extrastriolar hair cells; in contrast, MYO15A persists at stereocilia tips in both affected and unaffected bundles
• however, hair bundles in the striolar region of the utricle and the saccule show normal morphology
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• in the extrastriolar region, the diameter of utricle and saccule stereocilia is significantly less than that in wild-type controls
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nervous system
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• at P4, stereocilia in the organ of Corti appear normal, except for transiently elongated microvillar-like protrusions on the outer hair cell (OHC) surface, opposite to the hair bundle; however, extraneous protrusions disappear from OHCs by P10
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• in the extrastriolar region, vestibular hair bundles show a striking reduction in staircase slope, due to both a shortening of the tallest row of stereocilia and elongation of shorter rows; in the utricle, extrastriolar stereocilia are of nearly uniform length
• length difference between the tallest and shortest stereocilia in extrastriolar bundles is reduced by >80%
• MYO3B is no longer detectable at stereocilia tips of extrastriolar hair cells; in contrast, MYO15A persists at stereocilia tips in both affected and unaffected bundles
• however, hair bundles in the striolar region of the utricle and the saccule show normal morphology
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• in the extrastriolar region, the diameter of utricle and saccule stereocilia is significantly less than that in wild-type controls
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