Whole exome sequencing (WES) was performed on proband, and variants in BOR syndrome genes (EYA1 and SIX1) and other genes were evaluated to associate with hearing loss (definitive, strong, and moderate) by ClinGen Hearing Loss Clinical Domain Working Group were filtered [21].Heterozygous variants OTOG (NM_001292063.2):c.2402 A > G and EYA1 (NM_000503.6):c.639 + 3 A > C (ClinVar accession ID: SCV004176794) were identified. This evidence concerns the gene SIX1 and hearing loss disorder.