Analysis Tools
behavior/neurological
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• starting at 10 days after birth, can not maintain upright posture
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• choreoathetosis movement associated with the dystonic posture
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abnormal gait
(
J:1273
)
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• starting at 10 days after birth
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nervous system
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• impaired development of the dendritic trees of the Purkinje cells
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• patchy loss of outer hair cells at 3 months of age
• almost complete loss of the outer hair cells by 6 months of age
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• decreased synaptic connections of parallel fibers on the dendritic spines of the Purkinje cells as early as 2 weeks after birth
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• decrease in the number of spiral ganglion cells observed starting at 3 months of age
• most spiral ganglion cells are lost throughout the turns by 6 months of age
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hearing/vestibular/ear
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• patchy loss of outer hair cells at 3 months of age
• almost complete loss of the outer hair cells by 6 months of age
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• the cochlea degenerate severely within three months after birth
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• the sensory epithelium of the saccule became atrophic at 3 months of age
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• the saccule degenerate severely within three months after birth
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• no wave which can be regarded as wave I to VI in ABR audiometry at any age
• at maximum stimulation of 110 dBSPL, a low amplitude of wave after a short latency was observed
• no waves below 100 dBSPL
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cellular
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• impaired development of the dendritic trees of the Purkinje cells
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