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Mitochondrial Disorder: Maternally Inherited Diabetes and Deafness.

Fabiha Ahmed1, Stephen H Tsang2, Alicia R P Aycinena3

  • 1Columbia University, New York, NY, USA.

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|July 30, 2025
PubMed
Summary

Maternally inherited diabetes and deafness (MIDD) is an insulin-dependent diabetes condition. It typically begins in adulthood and is characterized by progressive hearing loss.

Keywords:
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Area of Science:

  • Endocrinology
  • Genetics
  • Otolaryngology

Background:

  • Maternally inherited diabetes and deafness (MIDD) is a rare mitochondrial disorder.
  • Characterized by insulin-dependent diabetes mellitus and sensorineural hearing loss.
  • Typically presents in the third or fourth decade of life.

Purpose of the Study:

  • To describe the clinical characteristics of patients with MIDD.
  • To highlight the association between diabetes and deafness in this condition.
  • To emphasize the importance of early diagnosis and management.

Main Methods:

  • Review of patient records.
  • Clinical examination and audiological assessment.
  • Genetic testing for mitochondrial DNA mutations.

Main Results:

  • Patients present with insulin-dependent diabetes and low body mass index (BMI).
  • Progressive neurosensory deafness is a consistent feature.
  • Onset of diabetes typically occurs in adulthood.

Conclusions:

  • MIDD is a distinct clinical entity with specific diagnostic criteria.
  • Early identification of MIDD is crucial for timely intervention.
  • Further research is needed to explore potential therapeutic strategies.