First, we performed targeted exome sequencing of the two regions obtained from the linkage analysis among four subjects (#284, #289, #290, and #291), leading to identification of two nonsynonymous variants [TMEM43-p.(Arg372Ter) and FBLN2-p.(Asp851His)] in Region #2 that cosegregated with the ANSD phenotype (SI Appendix, Table S2, Top). This evidence concerns the gene FBLN2 and auditory neuropathy.