Autoimmune Inner Ear Disease: Symptoms, treatment and prevention (1)

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Autoimmune Inner Ear Disease
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Autoimmune Inner Ear Disease

• Autoimmune inner ear disease, as the term
  suggests is an immune disorder that affects the
  structure of the inner ear. To better understand
  the condition, here is a brief description about
  immune disorders and the ear structure. The
  immune system is a part of the human body that
  helps to fight off infections and diseases by
  attacking foreign bodies or microorganisms like
  bacteria, viruses and fungi that are often
  responsible for infections. But in some cases
  this system malfunctions. In such circumstances,
  the individuals immune system may turn on the
  body itself and begin targeting healthy cells.
  This is known as an autoimmune disorder.Autoimmu
  ne inner ear disease or AIED is basically an
  inflammatory condition that affects the inner ear
  and is caused by an attack of the immune system
  on cells of the inner ear. This is a relatively
  rare condition and is believed to be responsible
  for less than 1 percent of all cases of hearing
  impairment.

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Symptoms

• The signs and symptoms of autoimmune inner ear
  disease are not always easy to recognize, as they
  can often be confused with otitis media symptoms.
  Typical symptoms include the following 
• Progressive deterioration in hearing in both ears
  with hearing loss occurring over weeks or months.
  One ear may be affected before the other.
• Sensations of fullness and blocked ear are not
  uncommon.
• Dizziness and vertigo may also be observed.
• Tinnitus is another symptom that is rather common
  and patients may notice a ringing or constant
  hissing sound in the ear.

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Diagnosis of Autoimmune Inner Ear Disease

• Because of the similarity of symptoms to otitis
  media, autoimmune inner ear disease is not easy
  to diagnose and is usually only detected when it
  begins to affect the second ear. The diagnosis of
  autoimmune inner ear disease is made using
  several techniques. There is no single test
  that is recommended for the evaluation or
  detection of autoimmune inner ear disease and the
  diagnosis is instead arrived at through exclusion
  and observed responses to steroids. Patients will
  need to go through a plethora of tests, most of
  which will be of extremely low yield. Your ENT
  doctor will study your medical history, response
  to steroids and immunosuppressive medication and
  will carefully evaluate clinical findings and the
  immunologic function of your serum. 

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Diagnosis of Autoimmune Inner Ear Disease
Continue

• Lab tests  can help and will evaluate certain
  parameters as part of an immune screen. These
  include 
• Erythrocyte sedimentation rate
• Rheumatoid factor
• Anti-nuclear antibody titer
• Quantitative immunoglobulin determination
• Leukocyte migration inhibition test
• Some additional tests that are used include the
  following 
• A Complete Blood Count or CBC
• FTA/ABS blood screen
• MRI, with contrast, of brain and cerebellopontine
  angle
• Lymphocyte blast transformation
• Lipid panel
• Steroid trial

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Treatments

• There are several methods of treatment and
  patients may often need to undergo multiple
  treatment regimens to find a suitable therapy.
  Because of the rarity of this condition, experts
  find it hard to study and this is reflected in
  contradictory findings and inadequate studies. By
  and large, there are certain protocols followed
  in the treatment of autoimmune inner ear diseases
  that include the followingWhen bilateral
  hearing losing is progressing rapidly, steroid
  trials are started. This usually lasts for nearly
  a month. If responsive to steroids, health care
  providers may also prescribe a type of
  chemotherapy medication and plasmapheresis. An
  anti-tumour necrosis factor drug may also be
  administered and has been found to be quite
  promising. Unfortunately, it is not easily
  available because of difficulties in its
  manufacture. Although there is an alternative
  that is being used, newer drugs still need to be
  tested for their efficacy.

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Treatments
Continue

• Plasmapheresis, which is a process of plasma
  replacement therapy, may also help in the
  treatment of the condition. There are many
  other treatments that are being investigated such
  as the use of oral collagen, gamma globulin
  infusions and immune modulating drugs.
  Unfortunately, most patients get limited succour
  from currently available treatments. While
  initial treatment with steroids and other drugs
  does produce a favourable response, long term
  efficacy of such therapies remains questionable.
  Hearing aids can help some patients and in other
  cases cochlear implants can also be a viable
  option.

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Prevention of Autoimmune Inner Ear Disease 

• Unfortunately, at present there is no known way
  to prevent autoimmune inner ear disease. The
  causes of the condition are not always clearly
  understood and as knowledge about the causes and
  treatment still remain highly debated, there is
  not much you can do to prevent the condition.
  However, it is extremely rare and you could
  instead take steps to prevent other common ear
  conditions that could escalate and cause
  complications that may also result in hearing
  loss.

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