Definition

What is maternally-inherited diabetes and deafness?

Maternally inherited diabetes and deafness (MIDD) is a mitochondrial disorder characterized by maternally transmitted diabetes and sensorineural deafness (especially of high tones).

How common is maternally-inherited diabetes and deafness?

Maternally-inherited diabetes and deafness is uncommon. About 1 percent of people with diabetes have MIDD. The condition is most common in the Japanese population and has been found in populations worldwide. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of maternally-inherited diabetes and deafness?

The common symptoms of maternally-inherited diabetes and deafness are:

  • Chorioretinal abnormality
  • Constipation
  • Macular dystrophy
  • Malabsorption
  • Sensorineural hearing impairment
  • Type II diabetes mellitus
  • Abnormality of lipid metabolism
  • Aplasia/Hypoplasia of the cerebellum
  • Arrhythmia
  • Congestive heart failure
  • External ophthalmoplegia
  • Glomerulopathy
  • Hypertension
  • Hypertrophic cardiomyopathy
  • Muscle weakness
  • Myalgia
  • Proteinuria
  • Ataxia
  • Cardiomyopathy
  • Cataract
  • Ptosis
  • Renal insufficiency
  • Retinopathy
  • Visual impairment
  • Dysarthria
  • Hyperglycemia
  • Mitochondrial inheritance
  • Pigmentary retinal degeneration
  • Retinal degeneration
  • Seizure
  • Unsteady walk
  • Vertigo

In MIDD, the diabetes and hearing loss usually develop in mid-adulthood, although the age that they occur varies from childhood to late adulthood. Typically, hearing loss occurs before diabetes.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes maternally-inherited diabetes and deafness?

The diabetes in MIDD is characterized by high blood sugar levels (hyperglycemia) resulting from a shortage of the hormone insulin, which regulates the amount of sugar in the blood. MIDD is caused by mutations in the MT-TL1, MT-TK, or MT-TE gene. These genes are found in mitochondrial DNA, which is part of cellular structures called mitochondria. Although most DNA is packaged in chromosomes within the cell nucleus, mitochondria also have a small amount of their own DNA (known as mitochondrial DNA or mtDNA). Because the genes involved with MIDD are found in mitochondrial DNA, this condition is inherited in a mitochondrial pattern, which is also known as maternal inheritance.

Because egg cells, but not sperm cells, contribute mitochondria to the developing embryo, only females pass mitochondrial conditions to their children. Mitochondrial disorders can appear in every generation of a family and can affect both males and females, but fathers do not pass mitochondrial traits to their children.

Most of the body’s cells contain thousands of mitochondria, each with one or more copies of mitochondrial DNA. These cells can have a mix of mitochondria containing mutated and unmutated DNA (heteroplasmy). The severity of MIDD is thought to be associated with the percentage of mitochondria with the mitochondrial DNA mutation

Risk factors

What increases my risk for maternally-inherited diabetes and deafness?

Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is maternally-inherited diabetes and deafness diagnosed?

Please consult with your doctor for further information.

How is maternally-inherited diabetes and deafness treated?

Please consult with your doctor for further information.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage maternally-inherited diabetes and deafness?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: January 25, 2018 | Last Modified: January 25, 2018

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