hearing/vestibular/ear
• at the basal turn, the organ of corti is collapsed into a compact cell layer that lacks peracellular spaces and contains only two rows of outer hair cells instead of three as in wild-type mice
• many outer hair cells are missing from the reticular lamina and replaced by large polygonal cells unlike in wild-type mice
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• at the basal turn, the organ of corti only has two rows of outer hair cells instead of three as in wild-type mice
• however, the number of outer hair cell rows in the apical turn is normal
• at P80, outer hair cells from the apex of the cochlea are missing from the first row
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• in all three rows of the basal turn and the first two rows in the apical turn at P14
• in all three rows of the basal and apical turns at P80
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• at P14 in the basal turn, outer hair cells from the first and second rows are missing due to degeneration
• at P14, the apical turn exhibits milder degeneration of outer hair cells from the first row
• degeneration of the hearing organ slows after P14 with some outer hair cells remaining at P80
• however, cultured explants of the organ of Corti do not exhibit degeneration
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• routine examination for auditory function at 4-5 weeks of age revealed the mutant mice to have no auditory brainstem response at any frequency tested
(J:87349)
• at P28, auditory brainstem response threshold is increased at high- and low-frequencies compared to in wild-type mice
(J:152154)
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• at P16, hearing loss is severe and deafness has an early onset
(J:152154)
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• the concentration of potassium ions in the perilymph is higher than in wild-type mice
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nervous system
• at the basal turn, the organ of corti only has two rows of outer hair cells instead of three as in wild-type mice
• however, the number of outer hair cell rows in the apical turn is normal
• at P80, outer hair cells from the apex of the cochlea are missing from the first row
|
• in all three rows of the basal turn and the first two rows in the apical turn at P14
• in all three rows of the basal and apical turns at P80
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• at P14 in the basal turn, outer hair cells from the first and second rows are missing due to degeneration
• at P14, the apical turn exhibits milder degeneration of outer hair cells from the first row
• degeneration of the hearing organ slows after P14 with some outer hair cells remaining at P80
• however, cultured explants of the organ of Corti do not exhibit degeneration
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behavior/neurological
N |
• mice exhibit normal balance on a rotating rod
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• routine examination for auditory function at 4-5 weeks of age revealed the mutant mice to have no acoustic startle response at any frequency tested
(J:87349)
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