Three of the 15 coding and burden associations were in genes that have not previously been implicated in Mendelian hearing loss in humans: KLHDC7B, FSCN2, and SYNJ2. In KLHDC7B, we confirmed a previously reported association of the common Val504Met and a rare frameshift variant with increased risk for hearing loss18,20, and identified associations of increased risk with a burden of additional pLOF variants. Here, FSCN2 is linked to hearing loss disorder.