To date, ten syndromic or non-syndromic deafness genes have been identified using targeted genomic enrichment and whole exome sequencing (WES): TPRN, GPSM2, CEACAM16, SMPX, HSD17B4, HARS2, MASP1,OTOGL, DNMT1, and TSPEAR. In addition several studies have shown the efficacy of WES to identify the causative mutations in recessive deafness forms [1], [4], [6], [7]. This evidence concerns the gene TSPEAR and deafness.