In Korea and Japan, the c.919-2A>G appears to be the second-most common, by frequency, pathogenic SLC26A4 variant (after c.2168A>G (p.His723Arg)) in patients with hearing loss, and its frequency falls within 20–40% in Korea and 5–10% in Japan, respectively [5,11,19,56,57]. Here, SLC26A4 is linked to hearing loss disorder.