The genes tested include well-known genes causal for hereditary progressive hearing loss such as GJB3 (CX31, DFNA2B, DFNB91), CRYM, GRHL2 (DFNA28), DFNA5, and ATP6B1 (distal renal tubular acidosis (dRTA) with hearing loss), all of whose pathophysiology remains unknown because their rodent models did not recapitulate the deafness phenotype. Here, SERPINB6 is linked to distal renal tubular acidosis.