hearing/vestibular/ear
• spaces normally containing fluid do not develop normally
• the organ of Corti is replaced by simple, cuboidal epithelium
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• hair cells degenerate
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• spaces normally containing fluid are absent or late to develop
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• there is no evidence of auditory function at any time point after birth
• auditory stimuli produced no behavioral or electrical responses from mutant mice
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nervous system
• hair cells degenerate
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• spiral ganglion cells degenerate
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normal phenotype
• mutant mice have normal saccular sensory epithelium unlike most other mutations producing deafness
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behavior/neurological