Dysfunction on mitochondrial protein synthesis plays a fundamental role in SNHL development, when tryptophanyl-tRNA synthetase 2 (Wars2) and mitochondrial ribosomal protein S2 (MRPS2), which are critical to the process, were proved to lead to severe SNHL and SGN loss during mutation [50, 51]. The gene discussed is WARS2; the disease is sensorineural hearing loss disorder.