Analysis Tools
behavior/neurological
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• homozygotes show no Preyer reflex
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• homozygotes display vertical head tossing
(J:55448)
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• homozygotes exhibit a general hyperactive behavior typical of the shaker-waltzer class of mutants
(J:55448)
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hearing/vestibular/ear
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• at E18.5, OHC morphology and patterning is markedly abnormal
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• at E18.5, no intact OHC stereociliary bundles can be identified with phalloidin staining
• at P0, long and aberrant stereocilia-like structures are identified on the surface of some degenerating OHCs
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• at P0, most of the basal sensory epithelium has degenerated
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• at E18.5, IHCs are readily identified in the apex but are clearly absent from the most basal 30-35% of the cochlear duct
• less severe IHC degeneration is noted at E16.5
• at P0, no IHCs can be identified in the basal parts
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• at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea
• less severe OHC degeneration is noted at E16.5
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• at E18.5, a slow degeneration of pillar cells occurs in a wave that begins from the base of the cochlea toward the apex
• at this stage, an undisrupted row of pillar cells is clearly present in the apex
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• homozygotes show no auditory brainstem responses up to 99 dB SPL
(J:55448)
• homozygotes exhibit increased auditory brainstem response (ABR) thresholds (>100 db SPL) relative to age-matched wild-type and heterozygous mice (35 db SPL)
(J:92833)
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• homozygotes are deaf; however, the anatomy of the inner ear is grossly normal
(J:55448)
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growth/size/body
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• affected homozygotes are slightly smaller than wild-type littermates
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nervous system
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• at E18.5, OHC morphology and patterning is markedly abnormal
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• at E18.5, no intact OHC stereociliary bundles can be identified with phalloidin staining
• at P0, long and aberrant stereocilia-like structures are identified on the surface of some degenerating OHCs
|
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• at P0, most of the basal sensory epithelium has degenerated
|
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• at E18.5, IHCs are readily identified in the apex but are clearly absent from the most basal 30-35% of the cochlear duct
• less severe IHC degeneration is noted at E16.5
• at P0, no IHCs can be identified in the basal parts
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• at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea
• less severe OHC degeneration is noted at E16.5
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