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Vol. 59. Issue 9.
Pages 472-473 (January 2008)
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Vol. 59. Issue 9.
Pages 472-473 (January 2008)
DOI: 10.1016/S2173-5735(08)70275-8
Maternally Inherited Diabetes and Deafness: A Case Report
Síndrome de diabetes de herencia materna y sordera
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Eduardo Masedaa,??
Corresponding author
maseda99@hotmail.com

Correspondence: Plaza Eduardo Úrculo, 3, 2.° J. 33001 Oviedo. Asturias. España.
, Antonio Sampedrob, Armando Ablanedoa, José Ricardo Alonsoa
a Servicio de Otorrinolaringología, Hospital San Agustín, SESPA, Avilés, Asturias, Spain
b Servicio de Oftalmología, Hospital San Agustín, SESPA, Avilés, Asturias, Spain
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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.

Key words:
Sensorineural deafness
Mitochondrial DNA
Maternally inherited diabetes and deafness syndrome

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 materna 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.

Palabras clave:
Hipoacusia neurosensorial
ADN mitocondrial
Síndrome de diabetes de herencia materna y sordera

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