In 4 dominant families, four heterozygous variants associated with dominant deafness were identified, including c.534_535 in GGAGGCAGAGGAA in PAX3 (OMIM 606597), c.1174-2A > T in PAX3 (OMIM 606597), p.T303T in MITF (OMIM 156845), and p.Y113H in PROKR2 (OMIM 607123), as well as partial co-segregating with the phenotype (Figure 1). The gene discussed is PROKR2; the disease is deafness.