KARS1 and hearing loss disorder: Finally, our data indicate that although the contribution of KARS1, HARS2, LARS2 and NARS2 is quantitatively small (5 out of 193 non-DFNB1 cases (2.6%) taking the Spanish and Colombian cohorts and the four genes in aggregate), they must be included in targeted gene-sequencing panels, as hearing loss is the first clinical sign that the patients manifest and their early genetic diagnosis will improve their follow up and management.