Maternally transmitted diabetes and deafness syndrome

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Description from OMIM

Maternally inherited diabetes-deafness syndrome (MIDD) is 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 pigmentary retinopathy, ptosis, cardiomyopathy, myopathy, renal problems, and neuropsychiatric symptoms (Ballinger et al., 1992; Reardon et al., 1992; Guillausseau et al., 2001). The association of diabetes and deafness is observed with Wolfram syndrome (see 222300), Rogers syndrome (249270), and Herrmann syndrome (172500), but all 3 of these disorders have other clinical manifestations.

Prevalence of clinical parameters (%)

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Number of patients in the reference Percent affected patients (Between 0 and 1, eg. 0.1 = 10%)
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List of symptoms

Symptom/sign Organ system Percent affected Pubmed id Added on(yyyy-mm-dd) Edit/add reference
Hearing loss nervous 98 % 11329229 2011-10-18
Retinitis pigmentosa nervous 73 % 18581092 2011-10-10
Diabetes mellitus type 1 endocrine 62 % 18581092 2011-10-11
Muscle weakness skeletal 43 % 11329229 2011-10-11
Diabetes mellitus type 2 endocrine 41 % 11329229 2011-10-11
Diabetes mellitus type 2 endocrine 38 % 18581092 2011-10-11
Nephropathy urinary 38 % 18581092 2011-10-10
Hypertension circulatory 28 % 11329229 2011-10-11
Nephropathy urinary 28 % 11329229 2011-10-11
Psychiatric symptom nervous 18 % 11329229 2011-10-11
Cardiomyopathy circulatory 15 % 11329229 2011-10-11
Diabetic retinopathy nervous 8 % 18581092 2011-10-10

List of references:

Maternally inherited diabetes and deafness: a multicenter study.
P J Guillausseau, P Massin, D Dubois-LaForgue, J Timsit, M Virally, H Gin, E Bertin, J F Blickle, B Bouhanick, J Cahen, S Caillat-Zucman, G Charpentier, P Chedin, C Derrien, P H Ducluzeau, A Grimaldi, B Guerci, E Kaloustian, A Murat, F Olivier, M Paques, V Paquis-Flucklinger, B Porokhov, J Samuel-Lajeunesse, B Vialettes,

Maternally inherited diabetes and deafness (MIDD), which is seen in 0.5% to 2.8% of patients with type 2 diabetes mellitus, is related to a point mutation at position 3243 of mitochondrial (mt) DNA. Its clinical description is incomplete.

Annals of internal medicine - May 2001

Retinal and renal complications in patients with a mutation of mitochondrial DNA at position 3,243 (maternally inherited diabetes and deafness). A case-control study.
P Massin, D Dubois-Laforgue, T Meas, M Laloi-Michelin, H Gin, B Bauduceau, C Bellanné-Chantelot, E Bertin, J-F Blickle, B Bouhanick, J Cahen-Varsaux, S Casanova, G Charpentier, P Chedin, O Dupuy, A Grimaldi, B Guerci, E Kaloustian, A Lecleire-Collet, F Lorenzini, A Murat, H Narbonne, F Olivier, V Paquis-Flucklinger, M Virally, M Vincenot, B Vialettes, J Timsit, P J Guillausseau, ,

We assessed the prevalence and determinants of retinal and renal complications in patients with maternally inherited diabetes and deafness (MIDD).

Diabetologia - Sep 2008