hearing/vestibular/ear
| N |
• no signs of vestibular dysfunction
(J:174130)
|
|
• 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
|
|
• significantly increased threshold at 4 weeks of age
|
|
• profound hearing loss at 4 weeks of age
|
behavior/neurological
| N |
• no signs of vestibular dysfunction
(J:174130)
|
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 |
OMIM ID | Ref(s) | |
| Deafness, Autosomal Recessive 12; DFNB12 | 601386 | J:174130 | |
