MAP3K1 and deafness: Echoing the mouse auditory phenotype associated with Map3k1-kinase-deficient alleles, here, we report a human family segregating a novel recessive variant c.4460G>A p.(Arg1487His) in MAP3K1 associated with nonsyndromic severe-to-profound deafness, suggesting that this variant results in a focused phenotype that damages the auditory system.