Some patients with distal renal tubular acidosis that have mutations in ATP6V1B1 or ATP6V0A4, which encode the V-ATPase V1B1 and Voa4 subunits, respectively, develop sensorineural hearing loss, which is typically caused by damage to the sensory hair cells and/or nerve fibers of the inner ear (Karet et al., 1999; Stover et al., 2002; Vargas-Poussou et al., 2006; Subasioglu Uzak et al., 2013). The gene discussed is ATP6V1B1; the disease is hearing loss disorder.