Autoimmune Inner Ear Disease: Symptoms, treatment and prevention - PowerPoint PPT Presentation

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Autoimmune Inner Ear Disease: Symptoms, treatment and prevention


Autoimmune inner ear disease, as the term suggests is an immune disorder that affects the structure of the inner ear. – PowerPoint PPT presentation

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Title: Autoimmune Inner Ear Disease: Symptoms, treatment and prevention

Autoimmune Inner Ear Disease
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

  • 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
  • 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.

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. 

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

  • 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 following When 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.

  • 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

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

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