Recently, mutations in mt-aaRS (ARS2) and cytoplasmic aaRS (ARS) have been associated with human diseases such as Charcot-Marie-Tooth disease, Perrault syndrome (LARS2, HARS2) [39,40], and pontocerebellar hypoplasia, all of which include sensorineural hearing impairment (Table 2). The gene discussed is HARS2; the disease is pontocerebellar hypoplasia.