hearing/vestibular/ear
• 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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• the IK,n current in the OHCs is abolished; the ensuing depolarization of OHCs impairs sound amplification
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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 the hearing ability at those frequencies approaches that of controls
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• DPOAEs could be recorded from 3-week-old mutant mice, albeit with lower amplitude
• At 8-10 weeks of age, DPOAEs were not distinguishable from the noise floor in mutant mice, but were readily observed in controls
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• after P21, animals develop significant hearing loss
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nervous system
• 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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• the IK,n current in the OHCs is abolished; the ensuing depolarization of OHCs impairs sound amplification
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