In 26 patients with the GJB2 variants in a homozygous or compound heterozygous state, hearing loss is characterized as early (detected up to 3 years) (92.3%), symmetric (88.5%), sensorineural (100.0%) and variable in severity (moderate—11.6%, severe—26.9%, and profound—61.5%). Here, GJB2 is linked to hearing loss disorder.