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Question:

From Gainesville, Florida, USA:

I have a 7 year old daughter who was diagnosed with IDDM at age 4. Last year we found out that she had hearing loss in her right ear and requires a hearing aid. She had another hearing test in April of 1998 and now they say she needs a hearing aid in the left ear. They say the damage is due to nerve damage (cochlea) and they cannot tell us why it is getting worse. They still consider her loss mild but I was wondering if low blood sugars could cause damage to the nerve. They also don't know what is the cause of this hearing loss.

I was told when she was diagnosed that since she is under 8 we should avoid low blood sugars as much as possible, but she goes from 400mg/dl to 50 in a matter of hours. Could low blood sugars cause the hearing loss? and what is DIDMOAD I have read about?

Answer:

By far the commonest causes of Sensory Neural Hearing Loss (SNHL) at thisage are infections and trauma. It has also been reported in autoimmune conditions of which of course. Type 1A Diabetes is one. However, the most important next step is not so much a precise diagnosis as ensuring that she is seated in the front of the class and there is a full evaluation of her hearing needs. Hypoglycemia is not thought to be a cause of deafness.

There are a number of very rare forms of diabetes that may be associated with deafness. Some of these are inherited disorders of the mitochondria (small oganelles that lie outside the nucleus in all cells). This mtDNA is inherited only from the mother and plays a critical role in energy metabolism. Cells that are especially vulnerable to any defect of this sort may be damaged. DIDMOAD (Diabetes Insipidus,Diabetes Mellitus, Optic Atrophy and Deafness) or Wolfram's Syndrome is one of these: MELAS and MIDD(Maternally Inherited Diabetes and Deafness) are others. Another form of the DIDMOAD syndrome accompanies an abnormality of the short arm of chromosome 4 (4p). Needs for insulin are variable and there is an extraordinary range in the clinical manifestations of the syndrome which has to do with the way that mitochondral abnormalities are inherited. Also the specific diagnosis of these conditions is difficult and expensive and is probably not worth attempting at the present time, especially as it makes no real difference to the treatment at the present time.

DO'B

Original posting 19 Jun 1998
Posted to Complications

  
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Last Updated: Tuesday April 06, 2010 15:08:58
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