hearing/vestibular/ear
• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
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• at 13 months, heterozygotes show a significantly greater OHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
• at 13 months, heterozygotes show a significantly greater, though variable, OHC loss in the apex relative to homozygotes
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• in response to 4-kHz tone bursts from 90 to 40 dB peak SPL, 13-mo-old heterozygotes exhibit variable ABR waveform morphology relative to wild-type mice
• at 4 kHz, heterozygotes with minimal hair cell loss display ABR peaks of similar morphology and amplitude to those of wild-type mice with maximal hair cell loss
• at 4 kHz, ABRs of heterozygotes with maximal hair cell loss are smaller and more poorly defined than those of homozygotes with the least hair cell loss
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• at 13 months, heterozygotes display significantly elevated ABR thresholds at 16 and 32 kHz (but not at 8 kHz) relative to wild-type mice
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• at 13 months, heterozygotes exhibit increased susceptibility to age-related hearing loss and cochlear hair cell loss, though to a lesser degree than homozygotes
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nervous system
• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
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• at 13 months, heterozygotes show a significantly greater OHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
• at 13 months, heterozygotes show a significantly greater, though variable, OHC loss in the apex relative to homozygotes
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