Analysis Tools
nervous system
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• at P3 homozygotes have regions of cochlear hair cell crowding and disorganization from the mid-turn toward the apex and the entire cochlea retains an occasional fourth row of hair cells
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hearing/vestibular/ear
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• imaging shows at 1 month of age the stria vascularis degenerates, endolymph volume is reduced in the scala media and Reissner's membrane is found collapsed
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• at P3 homozygotes have regions of cochlear hair cell crowding and disorganization from the mid-turn toward the apex and the entire cochlea retains an occasional fourth row of hair cells
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• poorly developed stria vascularis at P3 and there is degeneration of the stria vascularis by 1 month of age
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• whole mounts of adult inner ears show gross malformations of the semicircular canals
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• complete deafness by 4 weeks of age, the earliest timepoint assessed
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behavior/neurological
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• at 3 months of age some homozygotes entirely fail to orient in water and others swim at the surface but only in a circular pattern
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