Maternally Inherited Diabetes and Deafness (MIDD) Syndrome: A Clinical and Molecular Genetic Study of a Taiwanese Family

Recent advances in molecular analyses haveenabled the recognition of a specific diabetic syndrome characterized by the development of maternally inherited diabetes mellitus (DM) and sen-sorineural deafness. Furthermore, a point mutation at nucleotide pair (np) 3243 of the mitochondrial DNA (mtDNA) was identified from most diagnosed patients.(1) This syndrome was subsequently identi-fied in diabetic patients from various racial origins and is referred to as a different phenotype of A3243G mutation-related mitochondrial encephalo-myopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome.(2,3) It was later denoted as maternally inherited diabetes and deafness (MIDD) syndrome in order to signify its difference from the ordinary diabetic syndrome.(4) A recent multicenter prospective study of 54 patients with type 2 DM and the mtDNA 3243 mutation concluded the MID