The other two variants, only detected in Iceland, are fully penetrant loss-of-function variants in known Mendelian deafness genes that have not been described before; a stop-gained variant in heterozygous state in EYA4 (p.Tyr285Ter, MAF = 0.01%, OR = 35.6, P = 1.1 × 10−7; a likelihood-ratio test was performed in all logistic regression associations) and a frameshift variant in homozygous state in OTOA (p.Ala988ArgfsTer3, MAF = 0.65%, OR = 159.60, P = 2.7 × 10−20), where all carriers have moderate to profound hearing loss. Here, OTOA is linked to hearing loss disorder.