%0 Journal Article
%T Diabetes de herencia materna y sordera Maternally inherited diabetes and deafness
%A A. Sampedro
%A J.J. Barb車n
%A J.A. 芍lvarez
%A M.A. Andr谷s
%J Archivos de la Sociedad Espaˋola de Oftalmolog赤a
%D 2009
%I Scientific Electronic Library Online
%X Caso cl赤nico: Se describe el seguimiento de un paciente diab谷tico tipo 2 con una degeneraci車n macular en patr車n y sordera neurosensorial bilateral. En la electrofisiolog赤a mostraba un electrorretinograma (ERG) patr車n anormal y un electrooculograma (EOG) disminuido. Discusi車n: La presencia de diabetes de herencia materna y sordera neurosensorial, a los que suele sumarse una distrofia macular en patr車n, constituyen el s赤ndrome MIDD (Maternally Inherited Diabetes and Deafness), una rara enfermedad mitocondrial responsable de un 0,5% a 2,8% de los diab谷ticos. Case report: We described the follow up of a patient with diabetes mellitus type 2 who had a macular pattern dystrophy and bilateral neurosensory hearing loss. Electrophysiological studies revealed abnormal pattern electroretinography and impaired electro-oculogram responses. Discussion: Maternally Inherited Diabetes, neurosensory Deafness and generally macular pattern distrophy (MIDD syndrome), is a rare mitochondrial disease, responsible for approximately 0.5 to 2.8% of diabetes mellitus.
%K Diabetes
%K sordera
%K enfermedades mitocondriales
%K DNA mitocondrial
%K distrofia macular
%K Diabetes
%K deafness
%K mitochondrial diseases
%K mitochondrial DNA
%K macular dystrophy
%U http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0365-66912009000700007