Maternally inherited diabetes and deafness (MIDD)
- caused by a mutation in mitochondrial DNA (3243 tRNA)
- mitochondrial DNA is contributed to the embryo by the oocyte and not by spermatozoa, and therefore this disease is inherited from maternal family members only
- mitochondrial DNA is contributed to the embryo by the oocyte and not by spermatozoa, and therefore this disease is inherited from maternal family members only
- diabetes is a non insulin dependent type that usually presents before the age of 40 years
- due to a defect in beta cell function with normal insulin sensitivity
- associated deafness is sensorineural and develops in most of the diabetic subjects
- hearing loss is variable
- of these diabetic patients carrying the mitochondrial DNA mutation at position 3243, 75% experience sensorineural hearing loss
- associated hearing loss with diabetes is typically more common and more quickly declining in men than in women
- hearing loss is variable
- MIDD may have other multi-organ features: for example, elevated serum lactate, neuromuscular and cardiac problems, pigmented retinopathy, and nephropathy with proteinuria
Reference:
- Murphy R et al. Clinical features, diagnosis and management of maternally inherited diabetes and deafness (MIDD) associated with the 3243A>G mitochondrial point mutation. Diabet. Med. 2008, 25(4),383-99.
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