To clarify the pathogenic role of p.V37I in severe-to-profound hearing loss, further study evaluating the influence of genetic modifiers on the phenotype of homozygous p.V37I variant or exploring the yet-to-be identified, pathogenic DFNB1 allele in trans with single heterozygous p.V37I, such as copy number variation (CNV)17,37–40, is warranted. This evidence concerns the gene GJB2 and hearing loss disorder.