behavior/neurological
• absence of Preyer reflex
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hearing/vestibular/ear
• at 15 days of age, IHCs appear normal
• at 30 days of age, a loss of IHCs is seen
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• at 15 days of age, OHCs appear normal
• at 30 days of age, a loss of OHCs is seen
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• the ABR threshold is higher in 15-day-old mutant mice than in controls of the same age
• the threshold already exceeds 85 dB in some 15-day-old mutant mice
• the ABR threshold of mutant mice progressively increased from 15 to 30 days after birth and is also seen at 4 months of age
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• absence of DPOAEs in mutant mice at 15, 30 days of age as well as 3 months of age; these are easily seen in heterozygote and wild-type controls
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nervous system
• at 15 days of age, IHCs appear normal
• at 30 days of age, a loss of IHCs is seen
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• at 15 days of age, OHCs appear normal
• at 30 days of age, a loss of OHCs is seen
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• progressive with age
• a pronounced degeneration of the cochlear ganglion, with a marked reduction in cell number, is seen in 30-day-old mutant mice; by 60 days, most neurons are lost from the ganglion and the remaining cells appear to consist mostly of microglia
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