To date, biallelic variants in six causative genes have been associated with Perrault syndrome:HSD17B4 (MIM 233400) [7], HARS2 (MIM 614926) [3], LARS2 (MIM 615300) [8], CLPP (MIM 614129) [9], C10orf2 (MIM 616138) [10], and ERAL1 (MIM 607435) [6]. This evidence concerns the gene CLPP and Perrault syndrome.