Mutations in Gjb2 and Gjb6 followed by digenic mutations in these genes are among the most common causes of non-syndromic autosomal recessive sensorineural hearing loss (SNHL) in many populations around the world (Lerer et al., 2001; Del Castillo et al., 2002; Pallares-Ruiz et al., 2002; Stevenson et al., 2003; Wu et al., 2003; Bolz et al., 2004; Frei et al., 2004; Gualandi et al., 2004; Nickel and Forge, 2008; Batissoco et al., 2009; Chan et al., 2010; Asma et al., 2011; Da Silva-Costa et al., 2011; Mei et al., 2017). Here, GJB2 is linked to sensorineural hearing loss disorder.