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Wolfram syndrome, also called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is a rare autosomal-recessive genetic disorder that causes childhood-onset diabetes mellitus, optic atrophy, and deafness as well as various other possible disorders including neurodegeneration.
The diabetes that accompanies the hearing loss can be similar to Type 1 diabetes or Type 2 diabetes; however, Type 1-like diabetes is the more common form of the two. MIDD has also been associated with a number of other issues including kidney dysfunction, gastrointestinal problems , and cardiomyopathy .
Central diabetes insipidus, recently renamed arginine vasopressin deficiency (AVP-D), [1] is a form of diabetes insipidus that is due to a lack of vasopressin (ADH) production in the brain. Vasopressin acts to increase the volume of blood (intravascularly), and decrease the volume of urine produced.
Despite the name, diabetes insipidus is unrelated to diabetes mellitus and the conditions have a distinct mechanism, though both can result in the production of large amounts of urine. [1] Treatment involves drinking sufficient fluids to prevent dehydration. [1] Other treatments depend on the type. [1]
This means that psychogenic polydipsia may lead to test results (e.g. in a water restriction test) consistent with diabetes insipidus or SIADH, leading to misdiagnosis. [14] Dry mouth is often a side effect of medications used in the treatment of some mental disorders, rather than being caused by the underlying condition. [15]
Both T2DM and hearing loss independently increase dementia risk. Among patients with diabetes, age, nephropathy, and neuropathy [5] were associated with hearing loss. [citation needed] Identifying high-risk individuals for complications like hearing loss can enable early diagnosis and intervention.
Desmopressin, sold under the trade name DDAVP among others, is a medication used to treat diabetes insipidus, bedwetting, hemophilia A, von Willebrand disease, and high blood urea levels. [1] In hemophilia A and von Willebrand disease, it should only be used for mild to moderate cases. [ 1 ]
However, symptomatic treatment is often provided. The patients with LNMS often experience ataxia, spasticity and contractures, restricting their movements and daily activities. Therefore, multi-disciplinary approach is required including physical therapies, psychiatric and ophthalmologic consultations, nutrition and well-balanced diet.