Maternally Inherited Diabetes and Deafness (MIDD) with Undetectable C- Peptide Level and Cerebellar Atrophy

Maternally inherited diabetes and deafness (MIDD), also called mitochondrial diabetes mellitus, is a rare form of diabetes that comprises 0.5-2.8% of the diabetic population. Most cases of MIDD are associated with a point mutation in the mitochondrial DNA (mtDNA) at position 3243 of the leucine tRNA gene (A3243G) . Patients with MIDD are characterized by 1) young onset of diabetes, 2) absence of obesity, 3) neurosensory hearing loss, 4) maternal family history of diabetes and 5) progressive insulin secretory defect. In most cases of MIDD, diabetes is non-insulin dependent at onset, but progresses to require insulin therapy thereafter. However, to our knowledge, few cases with MIDD show complete loss of C-peptide level during the course of the disease.