In Family PKDF1419, sibship B is segregating a previously reported founder variant of CABP2 [50], while sibship A is segregating a novel substitution of a highly conserved residue located in the kinase domain of MAP3K1 that is associated with nonsyndromic deafness DFNB128, a newly reported locus for human HL. This evidence concerns the gene MAP3K1 and nonsyndromic deafness.