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. This evidence concerns the gene GRHL2 and distal renal tubular acidosis.