hearing/vestibular/ear
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• there are slightly increased numbers of inner hair cells in the middle region of E18.5 embryos (15.2 vs. 13.5 in wild-type)
• in the apex region, cochleae have many extra hair cells
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• there is a significant increase in the number of outer hair cells compared to controls
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• adult mice exhibit short and disorganized stereocilia bundles that have a loss of polarity
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• some of the outer hair cell bundles are missing in the middle and apical regions
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• all mice have shortened cochlear
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• there is a significant reduction in the number of hair cells found in the sacculle of mice (875 vs. 1705 in wild-type)
• there is a similar reduction in the number of hair cells found in the utricle
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• all mice at E18.5 have truncated posterior semicircular canals
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• all mice at E18.5 have abnormal ampulla, either truncated or absent
• 13 of 20 mice have at least one ear with an absent anterior ampulla
• all mice have an absent ampulla in the posterior section
• all mice have a malformed ampulla in the lateral section
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• 13 of 20 mice at E18.5 have truncated anterior semicircular canals
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• all mice have severely affected utricle
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• all mice at E18.5 have malformed saccule
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• 16 mice of 20 mice at E18.5 have a truncated endolymphatic duct
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nervous system
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• there are slightly increased numbers of inner hair cells in the middle region of E18.5 embryos (15.2 vs. 13.5 in wild-type)
• in the apex region, cochleae have many extra hair cells
|
|
• there is a significant increase in the number of outer hair cells compared to controls
|
|
• adult mice exhibit short and disorganized stereocilia bundles that have a loss of polarity
|
|
• some of the outer hair cell bundles are missing in the middle and apical regions
|
|
• there is a significant reduction in the number of hair cells found in the sacculle of mice (875 vs. 1705 in wild-type)
• there is a similar reduction in the number of hair cells found in the utricle
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