Variants in HARS2 and LARS2 are associated with Perrault syndrome manifesting with a wide range of neurological symptoms, variable progression of hearing loss and POI (Carminho-Rodrigues et al. 2020; Kosaki et al. 2018; Pierce et al. 2013; Souissi et al. 2021). Here, HARS2 is linked to Perrault syndrome.