Autoimmune Inner Ear Disease

Introduction of 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.
Autoimmune 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.

Symptoms of Autoimmune Inner Ear Disease

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.

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.

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

Treatments of Autoimmune Inner Ear Disease

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 option.

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.