WES data analysis revealed the presence of: (1) a nonsense variant segregating in all the affected family members in EYA4 (NM_004100.5) (c.714C > A, p.(Tyr238*)), a gene known for being causative of autosomal dominant NSHL (MIM#: 601316, OPRHA: 90635), and (2) two pathogenic mutations in USH2A gene (NM_206933.2) at the compound heterozygous state (i.e., c.11864G > A, p.(Trp3955*) and c.2276G > T, p.(Cys759Phe)), which is known for being causative of Usher syndrome type 2A (MIM#: 276901, ORPHA:886) in the proband, a girl of three years old (Table 1). The gene discussed is USH2A; the disease is Usher syndrome type 2A.