Analysis Tools
hearing/vestibular/ear
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• extreme disorganization is seen, including split, splayed and severely distorted bundles in outer hair cells at P7
• bundle disorganization is seen on some inner hair cells at P7
• despite bundle defects, mechanotransduction is acquired normally
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• orientation of bundle axes of kinocilia show greater deviation from 90 degrees
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• many stereocilia bundles exhibit subtle deviations in their orientation at P0, with polarity defects more obvious in outer hair cell bundles
• mice show progressive defects in stereocilia at P7, with most bundles on outer hair cells showing orientation defects and most bundles lacking the normal V shape
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• hearing auditory brainstem response (ABR) thresholds at 4, 12, and 24 kHz are elevated by approximately 30-60 dB above wild-type and heterozygous mice at 4 weeks of age, indicating hearing loss
• ABR measurements at 12 weeks of age shown an average shift of 40 to 70 dB in response to 4, 12, and 24 kHz tones
• however, endocochlear potentials are normal at 3 months of age
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• mice exhibit reduced distortion product otoaoustic emissions (DPOAEs) in response to 12 kHz stimuli at 4 weeks of age and significant DPOAEs only in response to stimuli at 70 and 80 dB SPL
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• hearing loss by 3 weeks of age
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• mice exhibit altered responses to linear acceleration, with higher vestibular evoked potential thresholds and longer peak latencies in response to linear accelerations
• however, mice do not exhibit circling or head shaking
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nervous system
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• extreme disorganization is seen, including split, splayed and severely distorted bundles in outer hair cells at P7
• bundle disorganization is seen on some inner hair cells at P7
• despite bundle defects, mechanotransduction is acquired normally
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• orientation of bundle axes of kinocilia show greater deviation from 90 degrees
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• many stereocilia bundles exhibit subtle deviations in their orientation at P0, with polarity defects more obvious in outer hair cell bundles
• mice show progressive defects in stereocilia at P7, with most bundles on outer hair cells showing orientation defects and most bundles lacking the normal V shape
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cellular
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• orientation of bundle axes of kinocilia show greater deviation from 90 degrees
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