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 individual’s 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.
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:
Diagnosis of Autoimmune Inner Ear Disease
- 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.
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.
can help and will evaluate certain parameters as part of an immune screen. These include:
Some additional tests that are used include the following:
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.