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. This evidence concerns the gene NARS2 and hearing loss disorder.