Noninsulin-dependent diabetes mellitus with deafness

A mitochondrial disorder characterized by onset of SENSORINEURAL HEARING LOSS and diabetes in adulthood. Some patients may have additional features observed in mitochondrial disorders, including RETINITIS PIGMENTOSA; PTOSIS; CARDIOMYOPATHY; MUSCULAR DISEASES; KIDNEY DISEASES, and neuropsychiatric symptoms. Mutations in the MITOCHONDRIAL GENES MTTL1, MTTE, and MTTK have been identified. OMIM: 520000

Also Known As:

Networked: 109 relevant articles (0 outcomes, 8 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Type 2 Diabetes Mellitus (MODY)
2.	Hearing Loss (Hearing Impairment)
3.	Retinal Diseases
4.	MELAS Syndrome (Syndrome, MELAS)
5.	Stroke (Strokes)

Experts

1.	Paquis-Flucklinger, V: 7 articles (10/2009 - 11/2000)
2.	Vialettes, B: 6 articles (08/2009 - 11/2000)
3.	Laloi-Michelin, M: 4 articles (03/2010 - 01/2004)
4.	Virally, M: 4 articles (03/2010 - 05/2001)
5.	Bertin, E: 4 articles (08/2009 - 05/2001)
6.	Bouhanick, B: 4 articles (08/2009 - 05/2001)
7.	Charpentier, G: 4 articles (08/2009 - 05/2001)
8.	Chedin, P: 4 articles (08/2009 - 05/2001)
9.	Gin, H: 4 articles (08/2009 - 05/2001)
10.	Grimaldi, A: 4 articles (08/2009 - 05/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Noninsulin-dependent diabetes mellitus with deafness:

1.	Mitochondrial DNA (mtDNA)IBA 05/01/2016 - "Genetic study identified the heteroplasmic mutation 3243A>G in mitochondrial DNA, which supports syndrome maternally inherited diabetes and deafness (MIDD) or Ballinger-Wallace disease. " 05/01/2023 - "Maternally inherited diabetes and deafness (MIDD) syndrome refers to a rarely diagnosed disorder caused by pathogenic variants in mtDNA. " 04/01/2023 - "Maternally inherited diabetes and deafness (MIDD) is a rare diabetic syndrome mainly caused by a point mutation in the mitochondrial DNA. " 01/01/2021 - "Purpose: Maternally inherited diabetes and deafness (MIDD) is caused by a heteroplasmic m.3243A>G mutation in the mitochondrial DNA. " 08/31/2017 - "Maternally inherited diabetes and deafness is a rare form of diabetes caused by a mitochondrial DNA mutation. "
2.	Leu Transfer RNAIBA 11/01/2008 - "Maternally inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. " 04/01/2006 - "Maternally-inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. " 06/01/2000 - "An A to G substitution at base pair 3243 in the mitochondrial tRNA(Leu(UUR)) gene (mt3243) is commonly associated with maternally inherited diabetes and deafness, and other diseases. " 06/01/1997 - "Maternally inherited diabetes and deafness (MIDD) is a new sub-type of diabetes and results from an A to G substitution at position 3243 of the mitochondrial tRNA(leu(UUR)) gene. " 01/01/1996 - "We showed previously that a mutation in the mitochondrial tRNALeu(UUR) gene at position 3243 associates with maternally inherited diabetes and deafness (MIDD). "
3.	Transfer RNA (tRNA)IBA 09/30/2016 - "Several mitochondrial tRNA mutations have been associated with maternally inherited diabetes and deafness. " 06/01/1999 - "To study the association of retinal disease and the syndrome of maternally inherited diabetes and deafness caused by an A to G mutation in the tRNA leucine gene at base pair 3243 (A3243G) of the mitochondrial genome. " 11/02/2000 - "A mutation in the tRNA leucine gene at position 3243 has been previously reported in mtDNA of maternally inherited diabetes and deafness (MIDD) patients. " 07/05/2018 - "T-to-C transition at nucleotide position 14709 in the mitochondrial tRNA glutamic acid (tRNAGlu) gene has previously been associated with maternally inherited diabetes and deafness. " 12/12/2002 - "A heteroplasmic T to C transition at nucleotide position 14709 in the mitochondrial tRNA glutamic acid (tRNA(Glu)) gene has previously been associated with maternally inherited diabetes and deafness (MIDD). "
4.	Leucine (L-Leucine)FDA Link 06/01/1999 - "To study the association of retinal disease and the syndrome of maternally inherited diabetes and deafness caused by an A to G mutation in the tRNA leucine gene at base pair 3243 (A3243G) of the mitochondrial genome. " 07/01/2013 - "Maternally inherited diabetes and deafness (MIDD), which is caused by an A to G substitution at position 3243 (m.3243A>G) in the transfer ribonucleic acid leucine gene, is characterized by diabetes and hearing loss. " 11/02/2000 - "A mutation in the tRNA leucine gene at position 3243 has been previously reported in mtDNA of maternally inherited diabetes and deafness (MIDD) patients. " 09/01/1999 - "To evaluate the prevalence of macular pattern dystrophy (MPD) in maternally inherited diabetes and deafness (MIDD), a new subtype of diabetes mellitus that cosegregates with a mutation of mitochondrial DNA (i.e., the substitution of guanine for adenine at position 3243 of leucine transfer RNA) and to report the clinical characteristics of MPD. "
5.	GuanineIBA 07/01/1997 - "Several studies show an association of a guanine for adenine substitution (A-->G) at position 3243 in mitochondrial DNA (mtDNA) with a recently recognized diabetic subtype designated maternally inherited diabetes and deafness (MIDD). " 02/01/2020 - "Objective: Maternally inherited diabetes and deafness (MIDD) is a rare diabetic syndrome mainly caused by a point mutation in the mitochondrial DNA (mtDNA), mt3243 adenine to guanine (A>G). " 11/01/2008 - "Maternally inherited diabetes and deafness syndrome is caused by the mitochondrial deoxyribonucleic acid mutation 3243 A>G (where A = adenine and G = guanine). " 09/01/1999 - "To evaluate the prevalence of macular pattern dystrophy (MPD) in maternally inherited diabetes and deafness (MIDD), a new subtype of diabetes mellitus that cosegregates with a mutation of mitochondrial DNA (i.e., the substitution of guanine for adenine at position 3243 of leucine transfer RNA) and to report the clinical characteristics of MPD. "
6.	AdenineFDA LinkGeneric 07/01/1997 - "Several studies show an association of a guanine for adenine substitution (A-->G) at position 3243 in mitochondrial DNA (mtDNA) with a recently recognized diabetic subtype designated maternally inherited diabetes and deafness (MIDD). " 02/01/2020 - "Objective: Maternally inherited diabetes and deafness (MIDD) is a rare diabetic syndrome mainly caused by a point mutation in the mitochondrial DNA (mtDNA), mt3243 adenine to guanine (A>G). " 11/01/2008 - "Maternally inherited diabetes and deafness syndrome is caused by the mitochondrial deoxyribonucleic acid mutation 3243 A>G (where A = adenine and G = guanine). " 09/01/1999 - "To evaluate the prevalence of macular pattern dystrophy (MPD) in maternally inherited diabetes and deafness (MIDD), a new subtype of diabetes mellitus that cosegregates with a mutation of mitochondrial DNA (i.e., the substitution of guanine for adenine at position 3243 of leucine transfer RNA) and to report the clinical characteristics of MPD. "
7.	Glucose (Dextrose)FDA LinkGeneric 02/01/2023 - "Pilot Trial on the Effect of 5-Aminolevulinic Acid on Glucose Tolerance in Patients with Maternally Inherited Diabetes and Deafness." 04/01/1995 - "D-Glucose metabolism was examined in the lymphocytes of six subjects with the mitochondrial tRNALeu(UUR) gene mutation responsible for the maternally inherited diabetes and deafness MIDD syndrome and compared with control subjects. " 02/01/2023 - "We investigated whether administering 5-ALA/SFC improves glucose tolerance with an increase in insulin secretion in patients with maternally inherited diabetes and deafness (MIDD), which is characterized by an insulin secretory disorder due to impaired mitochondrial ATP production. " 07/01/2001 - "Diminished insulin secretory response to glucose but normal insulin and glucagon secretory responses to arginine in a family with maternally inherited diabetes and deafness caused by mitochondrial tRNA(LEU(UUR)) gene mutation."
8.	Metformin (Glucophage)FDA LinkGeneric 10/01/2015 - "To further validate these methods, we performed a more detailed study using metformin, the most commonly prescribed antidiabetic drug that is still sometimes used in Maternally Inherited Diabetes and Deafness (MIDD). "
9.	Hypoglycemic Agents (Hypoglycemics)IBA 10/01/2015 - "To further validate these methods, we performed a more detailed study using metformin, the most commonly prescribed antidiabetic drug that is still sometimes used in Maternally Inherited Diabetes and Deafness (MIDD). "
10.	AcidsIBA 02/01/2023 - "Pilot Trial on the Effect of 5-Aminolevulinic Acid on Glucose Tolerance in Patients with Maternally Inherited Diabetes and Deafness."

Therapies and Procedures

1.	Transjugular Intrahepatic Portasystemic Shunt 12/01/2006 - "Maternally inherited diabetes and deafness in a North American kindred: tips for making the diagnosis and review of unique management issues."
2.	Photochemotherapy (Photodynamic Therapy) 04/01/2023 - "Abbreviations: AMD = age-related macular degeneration, anti-VEGF = anti-vascular epithelial growth factor, AOFVD = adult-onset foveomacular vitelliform dystrophy, BCVA = best corrected visual acuity, CNV = choroidal neovascularization, FAF = fundus autofluorescence, FFA = fundus fluorescein angiography, GA = geographic atrophy, LE = left eye, MIDD = maternally inherited diabetes and deafness, OCT = optical coherence tomography, OCTA = optical coherence tomography angiography, OU = oculus uterque, both eyes, PD = pattern dystrophy, PDSFF = pattern dystrophy simulating fundus flavimaculatus, PDT = photodynamic therapy, PRPH2 = peripherine-2, RE = right eye, RPE = retinal pigment epithelium, VA = visual acuity."
3.	Cochlear Implants (Cochlear Implant) 05/01/2002 - "We describe a rare case of a gradually progressive hearing loss in a patient with maternally inherited diabetes and deafness (MIDD) syndrome successfully rehabilitated with a cochlear implant. "