Síndrome de diabetes de herencia materna y sordera

El síndrome de diabetes de herencia materna y sordera es una causa infrecuente de hipoacusia neurosensorial de origen genético, causado por mutación en el ADN mitocondrial. Se caracteriza por diabetes mellitus de herencia maternal e hipoacusia neurosensorial en relación con distrofia macular, manifestaciones neuromusculares o psiquiátricas, miocardiopatía e insuficiencia renal. Presentamos el caso de un paciente que acude a la consulta por hipoacusia y presenta el síndrome de diabetes de herencia materna y sordera.

Maternally inherited diabetes and deafness (MIDD) syndrome is a rare disease associated with progressive sensorineural deafness due to a mitochondrial DNA mutation. Characterized by diabetes mellitus and sensorineural hearing impairment; MIDD is also associated with macular dystrophy, neuromuscular and psychiatric manifestations, cardiomyopathy as well as renal insufficiency. We report the case of a 55-year-old male patient complaining of hearing loss with maternally inherited diabetes and deafness syndrome.