Six of the variants not reported before, with rare genotypes and large effects on ARHI, are coding variants located in Mendelian deafness genes: LOXHD1, MPZL2, SLC411, SLC26A5, TBC1D24, and TMPRSS3. Rare variants in these genes have been reported to cause severe to profound hearing impairment described as either prelingual or childhood-onset (DFNB77, DFNB111, DFNB61, DFNA65, DFNB86, and DFNB8; OMIM #613079, #618145, #217400, #613865, #616044, #614617, and #601072). The gene discussed is DIRAS3; the disease is deafness.