Deafness, Congenital, and Adult-Onset Progressive Leukoencephalopathy, also known as deaple, is related to deafness, autosomal recessive 89 and hypomyelinating leukodystrophy. An important gene associated with Deafness, Congenital, and Adult-Onset Progressive Leukoencephalopathy is KARS1 (Lysyl-TRNA Synthetase 1). Affiliated tissues include brain and spinal cord, and related phenotypes are abnormal pyramidal sign and ataxia