hearing/vestibular/ear
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• later than 4 weeks of age, selective degeneration of outer hair cells of the basal turn is observed; this progresses over time
• 1 year old animals exhibit a near complete loss of outer hair cells
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• at 42 weeks animals display a 50-60 dB shift in threshold compared to age-matched wild-type
• at P14 animals have a higher hearing threshold than controls but at P21 hearing ability at those higher frequencies approaches that of controls
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• after P21, animals develop variable hearing loss which develops more slowly than in homozygous null or dominant negative animals
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nervous system
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• later than 4 weeks of age, selective degeneration of outer hair cells of the basal turn is observed; this progresses over time
• 1 year old animals exhibit a near complete loss of outer hair cells
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