hearing/vestibular/ear
• gradient of inner hair cell loss from base to apex at 16 weeks of age
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• complete loss of outer hair cells in the basal and middle turns of the cochlea at 16 weeks of age
• outer hair cells are more severely affected than inner hair cells
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• mice show an increase in hearing thresholds at more than 8kHz at 13 weeks of age and thresholds remain elevated at 16 weeks
• auditory shifts are more more pronounced at 24 and 32 kHz than at 16 and 8 kHz
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• DPOAEs are reduced in amplitude at 12 and 16 kHz or absent at 24 kHz by 16 weeks of age
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• mice show progressive hearing loss
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nervous system
• gradient of inner hair cell loss from base to apex at 16 weeks of age
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• complete loss of outer hair cells in the basal and middle turns of the cochlea at 16 weeks of age
• outer hair cells are more severely 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 |