mortality/aging
• mice die shortly after birth
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hearing/vestibular/ear
• short cochlea with no obvious turns with some degree of bifurcation at the apex region at E13.5
• prosensory domain development, as determined by marker expression, is disrupted
• however, initial cochleovestibular ganglion generation is normal
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• disorganized, miniature, short cochlear duct with no obvious turns
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• patchy formation of the organ of Corti-like structures, containing sometimes limited supporting cells either lacking or variable numbers of hair cells
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• disorganized
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• in some organ of Corti-like structures
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• in some organ of Corti-like structures
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• in some mice
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• no obvious turns
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• absent horizontal crista
• however, the anterior cristae seems normal
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nervous system
• disorganized
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• in some organ of Corti-like structures
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• in some organ of Corti-like structures
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• auditory fibers and projection defects
• however, neural fibers to the utricle and anterior cristae are normal
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• loss of spiral ganglion nerves during early development
• however, initial cochleovestibular ganglion generation is normal
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• missing inferior vestibular ganglion
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cellular
• in the cochlear duct beyond the prosensory region at E12.5 and E13.5
• in the spiral ganglion nerves during early development
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