3-methylglutaconic aciduria Mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes Disorder of phospholipids, sphingolipids and fatty acids biosynthesis with central nervous system predominant involvement MEGDEL syndrome ICD10:E71.1 3-methylglutaconic aciduria with deafness - encephalopathy - Leigh-like syndrome OMIM:614739 Rare genetic neurological disorder Syndromic genetic deafness