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67% of the congenital SNHL cases. In mice cochlea, Shp2, which is encoded by , is distributed in the spiral ganglion neurons, hair cells and supporting cells of the inner ear. In zebrafish, knockdown of and overexpression of mutant were associated with a significant decrease in hair cells and supporting cells. We concluded that congenital SNHL could be a major symptom in -associated NSML or NS. Other features may be mild, especially in children. Screening for in patients with congenital hearing loss and variant-based diagnoses are recomm
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