hearing/vestibular/ear
• disorganized formation of stereocilia rows
• some hair cells with several small clumps of stereocilia
|
• orientation of rows of cochlear hair cell stereocilia erratic
|
• no cochlear microphonics responses
|
• no summating potential responses
|
nervous system
• disorganized formation of stereocilia rows
• some hair cells with several small clumps of stereocilia
|
• orientation of rows of cochlear hair cell stereocilia erratic
|
• no cochlear microphonics responses
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Usher syndrome type 1 | DOID:0110826 |
OMIM:276900 |
J:46373 |