Phenotypically, two probands carrying del(125 kb) and GJB2 c.109G>A (p.Val37Ile) exhibited mild-to-moderate hearing loss (F10 II-1 and F34 II-1), while the other proband (F43 II-1) carrying del(GJB6-D13S1854) and GJB2 c.299_300del (p.His100ArgfsTer14) presented with profound hearing loss (Figure 2(b)). This evidence concerns the gene GJB2 and sensory perception of sound.