hearing/vestibular/ear
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• basal cochlea has disorganized hair cells and an excess of inner hair cells, and the apex has extra rows of outer hair cells
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• the utricular macula is reduced from an ovate to a trapezoid shape
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• the saccular macula is often attached to the utricular macula and cochlear base
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• the utricle and saccule fail to properly separate
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• transheterozygotes have ABR thresholds 25-50 dB higher than controls at 1 month of age
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nervous system
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• basal cochlea has disorganized hair cells and an excess of inner hair cells, and the apex has extra rows of outer hair cells
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