Analysis Tools
behavior/neurological
head shaking
(
J:84285
)
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• heterozygotes exhibit belly curling and spinning during tail suspension
• however, no obvious peripheral vestibular defects are detected in adulthood
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hearing/vestibular/ear
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• heterozygotes exhibit a positive Preyer's reflex in response to a 20 kHz, 90 dB SPL tone burst, indicating that they are not profoundly deaf
• however, heterozygotes show defects in planar cell polarity of the sensory epithelium
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• at E18.5 and 3-5 months, heterozygotes display excessive misoriented OHC stereociliary bundles, esp. in the apical cochlear turn relative to wild-type mice (apex 3.60%, base 1.68% rotated vs apex 0.66%, base 0.18% rotated, respectively)
• in the apex, OHC bundle misorientation is noted as early as E16.5
• in contrast, IHC stereociliary bundles remain unaffected
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• at 3-5 months, heterozygotes show minimal OHC degeneration in the basal cochlear turn
• however, no significant loss of OHCs is noted in the apex
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• at E18.5 and 3-5 months, heterozygotes display distortion of the upper surfaces of supporting cells that are immediately adjacent to misoriented OHCs
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nervous system
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• at E18.5 and 3-5 months, heterozygotes display excessive misoriented OHC stereociliary bundles, esp. in the apical cochlear turn relative to wild-type mice (apex 3.60%, base 1.68% rotated vs apex 0.66%, base 0.18% rotated, respectively)
• in the apex, OHC bundle misorientation is noted as early as E16.5
• in contrast, IHC stereociliary bundles remain unaffected
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• at 3-5 months, heterozygotes show minimal OHC degeneration in the basal cochlear turn
• however, no significant loss of OHCs is noted in the apex
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