INS and deafness: These features are typically associated with MDM and are supported by previous studies: (1) maternal inheritance of diabetes and deafness; (2) early middle aged onset of diabetes and deafness; (3) thin and short stature; (4) absence of anti-GAD autoantibodies; (5) low heteroplasmy in leukocytes; (6) high incidence of neurosensory deafness; (7) early requirement for insulin therapy due to the progressive insulin secretory defect (24–30).