By screening other gap junction genes, another subject (SH175-389) carrying a single heterozygous p.V193E in GJB2 allele harbored a single heterozygous p.A194T mutant allele of GJB3 (NM_001005752) (SH175-389) with known pathogenicity (Figure 4D).31 This 2-year-old female showed severe autosomal recessive SNHL with a mean hearing threshold of 87.5 dB HL. The gene discussed is GJB3; the disease is sensorineural hearing loss disorder.