hearing/vestibular/ear
• decreased number of BK channels
|
• mitochondria are concentrated in the center of the cytoplasm rather than along the plasma membrane, under the cuticular plate, and around the nucleus as in controls
|
• massive outer hair cell loss from the apical region at P40
• however, inner hair cells, spiral ganglion and stria vascularis appear similar to controls
• outer hair cell loss begins by P30 and continues with age with near absence by P80
|
• outer hair cell loss begins by P30 and continues with age with near absence by P80
|
• at P40, show low-frequency but not high-frequency hearing loss
|
cellular
• mitochondria are concentrated in the center of the cytoplasm rather than along the plasma membrane, under the cuticular plate, and around the nucleus as in controls
|
nervous system
• decreased number of BK channels
|
• mitochondria are concentrated in the center of the cytoplasm rather than along the plasma membrane, under the cuticular plate, and around the nucleus as in controls
|
• massive outer hair cell loss from the apical region at P40
• however, inner hair cells, spiral ganglion and stria vascularis appear similar to controls
• outer hair cell loss begins by P30 and continues with age with near absence by P80
|
• outer hair cell loss begins by P30 and continues with age with near absence by P80
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
nonsyndromic deafness | DOID:0050563 | J:345577 |