hearing/vestibular/ear
• the stereocilia bundle of outer cells along the length of the neuroepithelium is largely immature and underdeveloped at postnatal day 14
• lateral and apical aspects of the bundle appear rounded and deformed
• stereocilia ae disconnected and detached, found outside of the unit, and are bent
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• pronounced in the basal turn at 15 weeks of age
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• exhibit ABR threshold about 40 dB higher than the normal thresholds of control at 15 weeks of age
• rate of threshold improvement in mutant mice was approximately ten times slower than in normal mice
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• exhibit ABR threshold about 40 dB higher than the normal thresholds of control at 15 weeks of age, and this hearing loss progresses to deafness by 20 weeks of age
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• progressive hearing loss
• early onset hearing loss starting before 6 months of age
• deafness by 20 weeks of age
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behavior/neurological
• Background Sensitivity: BUB/BnJ mice show audiogenic seizure only when tested at young ages (<25 days od age), whereas original RB/1 mice homozygous for this allele retain seizure sensitivity well into adulthood
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nervous system
• Background Sensitivity: BUB/BnJ mice show audiogenic seizure only when tested at young ages (<25 days od age), whereas original RB/1 mice homozygous for this allele retain seizure sensitivity well into adulthood
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• the stereocilia bundle of outer cells along the length of the neuroepithelium is largely immature and underdeveloped at postnatal day 14
• lateral and apical aspects of the bundle appear rounded and deformed
• stereocilia ae disconnected and detached, found outside of the unit, and are bent
|
• pronounced in the basal turn at 15 weeks of age
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• pronounced in the basal turn at 15 weeks of age
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