Analysis Tools
hearing/vestibular/ear
| N |
• no signs of vestibular dysfunction
|
|
• the tunnel of Corti is less well formed at 4 weeks of age
|
|
• absence of stereociliary tip tenting of IHC second tallest row stereocilia indicating disruption of tip links
|
|
• some indication of hair cell degeneration in the basal cochlear region at 4 weeks of age
• by 8 and 12 weeks of age signs of hair cell degeneration are clearly apparent in the basal and midcochlear turns
|
|
• collapse of the organ of Corti in the basal and midcochlear turns at 8 and 12 weeks of age
|
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• significantly increased threshold at 4 weeks of age
|
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• profound hearing loss at 4 weeks of age
|
behavior/neurological
| N |
• no signs of vestibular dysfunction
|
nervous system
|
• absence of stereociliary tip tenting of IHC second tallest row stereocilia indicating disruption of tip links
|
|
• some indication of hair cell degeneration in the basal cochlear region at 4 weeks of age
• by 8 and 12 weeks of age signs of hair cell degeneration are clearly apparent in the basal and midcochlear turns
|
|
• loss of spiral ganglion neurons in the basal and midcochlear turns at 8 and 12 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal recessive nonsyndromic deafness 12 | DOID:0110467 |
OMIM:601386 |
J:174130 | |
