We demonstrated MITF to be a new causative gene for ARNSHL based on the following evidence: (1) other known genes were excluded; (2) MITF variants were a recurrent phenomenon in multiple affected families; (3) cosegregation of the homozygous variants with hearing loss, in the absence of homozygous mutations in hearing relatives and a control population; (4) deleterious effects of the mutations, as shown by in silico analysis and molecular modeling, disrupted expression of a downstream gene, and aberrant subcellular localization; and (5) the known roles of MITF in cochlear melanocytes. The gene discussed is MITF; the disease is hearing loss disorder.