hearing/vestibular/ear
• cupula and tectorial membranes are thinner (15.7+/-2.6um compared to 32.5+/-2.5um in wild-type mice)
• the acellular matrix that give rise to the tectorial membrane is thinner
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• utricle and saccule otolith membranes appear brittle, less compact and frequently break
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• utricle and saccule otolith membranes appear brittle, less compact and frequently break
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• an organic matrix is lacking
• more than 75% of mice exhibit otoliths that do not securely adhere to the membranous honeycomb layer
• loose otoliths are occasionally displaced into the semi-circular canals or become associated with the roof
• utricle and saccule otolith membranes appear brittle, less compact and frequently break
• however, other inner ear structures including all epithelial cells are normal
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• enlarged otoliths are visible early in development at E16.5
• otoliths contain dark crystal deposits
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