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 |
DO ID | OMIM ID(s) | Ref(s) | |
| Usher syndrome type 1 | DOID:0110826 |
OMIM:276900 |
J:157102 | |


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