hearing/vestibular/ear
small ears
(
J:83738
)
|
• the cochlea is shortened but otherwise normal including the organ of Corti
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• the short canal emerging from the horizontal crista extend vertically toward the cochlea rather than horizontal, toward the posterior canal
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• a blind-ending stub is formed instead of the horizontal canal
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• complete absence of all aspects of the posterior canal system; no posterior crista, no posterior canal, and no myelinated nerve fiber to the area of the posterior canal
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• the anterior and horizontal cristae are much smaller and barely segregated from the utricle
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• the anterior canal is largely continuous with the utricle without forming a recognizable canal
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• enlarged bulbs on the kinocilia
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• remaining hair cells of vestibular sensory epithelia show some aberrations in the formation of apical stereocilia
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• in most mutants, the utricle forms a central recess rather than a flat sheet of cells
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• the saccule shows slight dismorphogenesis, is somewhat smaller, and is in a somewhat different position, further away from the cochlea
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nervous system
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• enlarged bulbs on the kinocilia
|
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• remaining hair cells of vestibular sensory epithelia show some aberrations in the formation of apical stereocilia
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• the posterior canal sensory neurons from initially and project rather normally by E11.5 but they disappear within 2 days
|
craniofacial
small ears
(
J:83738
)
cellular
|
• enlarged bulbs on the kinocilia
|
growth/size/body
small ears
(
J:83738
)


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