The most significant rare coding association in our analysis was the aggregate of 68 pLOF variants (43 frameshift, 23 stop-gain and 2 stop-loss, (Supplementary Data 9)) in KLHDC7B (Kelch-like domain containing 7B), with an approximately two-fold increase in risk for hearing loss (OR = 2.14, P = 5 × 10−30). This evidence concerns the gene KLHDC7B and hearing loss disorder.