The most common form of hereditary nonsyndromic hearing loss is autosomal recessive deafness 1A (DFNB1A, MIM#220290) caused by pathogenic variants in the GJB2, GJB6, and GJB3 genes encoding connexin 26 (Cx26), connexin 30 (Cx30), and connexin 31 (Cx31) proteins, respectively. Here, GJB3 is linked to autosomal recessive nonsyndromic hearing loss 1A.