Deafness, Autosomal Recessive 89, also known as dfnb89, is related to deafness, congenital, and adult-onset progressive leukoencephalopathy and nonsyndromic hearing loss. An important gene associated with Deafness, Autosomal Recessive 89 is KARS1 (Lysyl-TRNA Synthetase 1), and among its related pathways/superpathways are Metabolism of proteins and Peptide chain elongation. Related phenotypes are sensorineural hearing impairment and vestibular hypofunction