Of these, in addition to the variants in SLC22A4 and P4HA2, we evaluated by Sanger sequencing in all available family members the segregation with hearing loss of the variants in SNX2, IK, PCDHB3, and PCDHB16. The SNX2 NM_003100:c.934C>A (p.Q312K) does not segregate with NSHL in the right branch of the pedigree, being the affected IV7 subject wild type. The gene discussed is PCDHB16; the disease is hearing loss disorder.