Most variants of Perrault syndrome are triggered by the failure of mitochondrial RNA processing and translation, as exemplified by other causal mutations in the tRNA-amino acid synthases HARS2 and LARS2, in the mitoribosomal factor RMND1, in the RNA degradation factor PRORP, in the nucleoid replication/repair factor TWNK, or in CLPP [18,19]. Here, LARS2 is linked to Perrault syndrome.