Analysis Tools
hearing/vestibular/ear
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• mutant mice bearing extra copies of the Gjb2 gene, where Gjb2 protein translation is restored to wild-type levels in the cochlea, exhibit complete rescue of the deafness phenotype observed in Gjb6tm1Kwi homozygotes, as shown by prevention of hair-cell death in the organ of Corti, normal ABR thresholds across a frequency range of 4-32 kHz at 4 weeks and 4 months, and complete recovery of large positive endocochlear potentials in rescued mice
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