hearing/vestibular/ear
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• by 48 weeks of age, a decrease in the number of cochlear inner hair cells is seen in the basal turn of the cochlea
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• by 48 weeks of age, a decrease in the number of cochlear outer hair cells is seen in the basal turn of the cochlea
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• a small, but significant, increase in auditory threshold is detected at 32 kHz high frequencies at 4-12 weeks of age, which is further increased at 16-24 weeks of age
• by 48 weeks of age, significant hearing loss is detected at 8, 16 and 32 kHz
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• mice show mild progressive hearing loss
• however, mice show normal inner ear development and no signs of vestibular dysfunction
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homeostasis/metabolism
nervous system
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• by 48 weeks of age, a decrease in the number of cochlear inner hair cells is seen in the basal turn of the cochlea
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• by 48 weeks of age, a decrease in the number of cochlear outer hair cells is seen in the basal turn of the cochlea
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• mice exhibit a decrease in spiral ganglion density and large number of empty spaces that are scattered throughout Rosenthals canal, indicating loss of spiral ganglion neurons in the apical, middle, and basal turns of the cochlea at 48 weeks of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| X-linked deafness 1 | DOID:0111739 |
OMIM:304500 |
J:388624 | |


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