In 8 recessive families, bi-allelic mutations were found in known deafness genes, part of which were identified by parental genotyping including p.H3204R and p.N2356K in USH2A (OMIM 608400), p.Q2137X and p.W778X in CDH23 (OMIM 605516), c.3795 + 5 G>A and c.7051_7054 + 1dup in CDH23 (OMIM 605516), p.R1898Q and c.10258_10260 del in MYO15A (OMIM 602666), c.12177_12181delGGTTG and a duplication in ADGRV1 (OMIM 602851), partial or whole gene deletion in STRC (OMIM 606440), c.16 + 1C>T and c.535 + 5G>A in TMC1 (OMIM 606706) (Table 1). The gene discussed is ADGRV1; the disease is deafness.