Of 50 cases with severe-to-profound SNHL not caused by GJB2/GJB6, OTOF or MT-RNR1 mutations, a genetic justification for their HL phenotype was found in 21 (42%) after identifying 31 pathogenic/likely pathogenic variants in 16 genes: ACTG1, BSND, CDH23, CHD7, COL4A5, GATA3, MITF, MYO15A, P2RX2, POU3F4, PRPS1, RDX, SLC26A4, SOX10, STRC and USH2A (Tables 2 and 3). This evidence concerns the gene GJB2 and sensorineural hearing loss disorder.