behavior/neurological
|
• low auditory startle response
|
|
• disoriented
|
hearing/vestibular/ear
|
• stereociliary bundles are disorganized in all four rows of hair cells of the cochlear sensory epithelium
• often lacking clear polarity
|
|
• stereocilia are often short
|
|
• auditory threshold of more than 90 dB, indicating that lines are severely hearing impaired in 8- to 12 week old animals
|
|
• not detectable
|
vision/eye
|
• melanosomes fail to localize to the apical processes of retinal pigment cells
|
nervous system
|
• stereociliary bundles are disorganized in all four rows of hair cells of the cochlear sensory epithelium
• often lacking clear polarity
|
|
• stereocilia are often short
|
pigmentation
|
• melanosomes fail to localize to the apical processes of retinal pigment cells
|
Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Usher Syndrome, Type I; USH1 | 276900 | J:157102 | |
