Analysis Tools
hearing/vestibular/ear
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• reduction in the number of inner hair cells in the base by 16 weeks of age
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• progressive degeneration of outer hair cells following a basal-to-apical gradient, with complete loss of outer hair cells in the basal turn of the organ of Corti in all 13 week old mutants, while the middle and apical regions show normal hair cells at this time, and variable outer hair cell loss in the middle turns by 16 weeks of age
• outer hair cells are more affected than inner hair cells
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• severe degeneration in the base
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• severe degeneration in the base
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• severe degeneration of supporting cells (Deiters cells and pillar cells) in the base, with the basilar membrane fully intact
• middle sections show some signs of supporting cell degeneration at 16 weeks of age, with collapse of Nuels space
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• the ABR thresholds at 16 and 32 kHz are modestly elevated at 6 weeks of age, with a further increase between 6 and 12 weeks and 36 weeks
• by 12 weeks of age, mice have undergone an approximate 57 dB sound pressure level threshold shift compared with controls at 16 and 32 kHz, corresponding to the middle and basal regions of the cochlea
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• DPOAEs at frequencies between 10 and 16 kHz are reduced in amplitude or are absent at 8 and 12 weeks of age, indicating outer hair cell impairment
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• mice show progressive hearing loss starting after 6 weeks of age
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nervous system
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• reduction in the number of inner hair cells in the base by 16 weeks of age
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• progressive degeneration of outer hair cells following a basal-to-apical gradient, with complete loss of outer hair cells in the basal turn of the organ of Corti in all 13 week old mutants, while the middle and apical regions show normal hair cells at this time, and variable outer hair cell loss in the middle turns by 16 weeks of age
• outer hair cells are more affected than inner hair cells
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Cockayne syndrome | DOID:2962 | J:219993 | ||
