hearing/vestibular/ear
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• abnormal pigmentation in the inner ear such as unpigmented stria
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• variable cochlea defects that increased in severity as mutants aged
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• form of the organ of Corti was completely changed with no recognizable structure remaining in mutants older than 300 days
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• outer hair cells in the organ of Corti were mostly missing or malformed
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• vascularity was reduced
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• thinner than normal
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• the tectorial membrane was distorted and no longer in contact with the organ of Corti
• the tectorial membrane grew thicker and was usually attached to Reissner's membrane by 300 days of age
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• some hair cells in the macula were lost and many others became enlarged and spherical
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• variable saccule defects that increased in severity as mutants aged
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• increase in the amount of otoliths
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• otolith granules were larger
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nervous system
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• outer hair cells in the organ of Corti were mostly missing or malformed
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• some hair cells in the macula were lost and many others became enlarged and spherical
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• about 50% had abnormal spiral ganglion, with cells appearing immature and occurring in clumps in certain regions
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• reduced cell density and severe degeneration in mutants over 140 days old so that spiral canal was virtually empty
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pigmentation
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• abnormal pigmentation in the inner ear such as unpigmented stria
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• mice are white with contrasting but normal black eye color
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cardiovascular system
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• vascularity was reduced
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craniofacial
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• abnormal pigmentation in the inner ear such as unpigmented stria
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integument
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• abnormal pigmentation in the inner ear such as unpigmented stria
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• mice are white with contrasting but normal black eye color
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reproductive system
growth/size/body
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• abnormal pigmentation in the inner ear such as unpigmented stria
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