CIDPUSA.org Autoimmune diseases
Autoimmune Inner Ear Disease (AIED) or Menieres disease
A new name for a well known old ear disease is AIED, previously known as Menieres disease. That is Autoimmune inner ear disease or "AIED" consists of a syndrome of sudden hearing loss which may be accompanied by dizziness which is caused by antibodies or immune cells which are attacking the inner ear.
The classic picture consists of sudden attacks of pain in the ear accompanied by loss of hearing which happens quickly may be accompanied by tinnitus (ringing, hissing, roaring) these attacks remit and relapse. The patient gets better and then improves. Variants are bilateral attacks of hearing loss and tinnitus which resemble Meniere's disease, and attacks of dizziness accompanied by abnormal blood tests for self-antibodies. About 50% of patients with AIED have imbalance. The attacks can last a few days or a few weeks.
The immune system consists of a number of cells to defend the human cells from being attacked by invaders. However the invaders learn that by using the very own defense forces of the human body a attack can be mounted and there are several ways that it can damage the inner ear. Both allergy and traditional "autoimmune disease" such as Ankylosing spondylitis (bamboo spine), Behcet's (oro genital ulceration), Systemic Lupus Erythematosis (SLE or lupus), Sjogren's syndrome (dry eye syndrome), Cogan's disease, ulcerative colitis, Wegener's granulomatosis (cherry red gums), relapsing polychondritis, rheumatoid arthritis, and scleroderma (tight lips and fingers) can cause or be associated with AIED.
Allergy involving the inner ear is traditionally felt to be food related, but there is presently no agreement as to the importance of food allergy.
Ankylosing spondylitis (AS) is a progressive bone disease associated with fusion of the spine. Persons with severe cases of AS may be disabled because of their lack of flexibility. Although one might expect AS to be associated with conductive hearing loss, AS has been reported to be associated with a sensorineural hearing loss in about 28% of patients.
Susac syndrome (microangiopathy of ear, brain and eye) is a disorder in which deafness, reduced vision, and encephalopathy may all present simultaneously. The encephalopathy manifests with headache, confusion, memory loss, behavioral changes, dysarthria and occasional mutism. The hearing loss is usually bilateral and accompanied by tinnitus and vertigo. According to Susac, it is generally a low-frequency sensorineural loss, resembling that ofMeniere's disease. The reduced vision is caused by retinal artery occlusions. MRI images, which are invariably abnormal, reveal multifocal white matter lesions, including the corpus callosum (Susac et al, 2004). This is an unusual location for a white matter lesion as it is not found in the more common types of demyelinating disease, such as MS. Treatment is with immunosuppresants (Clement et al, 2003).
Not all individuals with bilateral sensorineural hearing loss have autoimmune disease. Genetic defects,infections,toxins, advanced age, noise exposure, andconditions of mysterious origin all account for some cases.
How Common is Autoimmune Inner Ear Disease ?
AIED is rare, probably accounting for less than 1% of all cases of hearing impairment or dizziness. The precise incidence is controversial. About 16% of persons with bilateralMeniere's disease, and 6% of persons with Meniere's disease of any variety may be due to immune dysfunction.
What Causes Autoimmune Inner Ear Disease ?
Stress is a big cause of vertigo, Menieres disease and AEID, stress will trigger immune dysfunction and lead to ear problems. Prayer and reduction in stress will help improve this condition.
The cause of AIED is generally assumed to be related to either antibodies or immune cells that cause damage to the inner ear. There are several theories as to how these might arise, analogously to other putatative autoimmune disorders:
Cross-reactions: In this theory, antibodies or rogue T-cells cause accidental inner ear damage because the ear shares common antigens with a potentially harmful substance, virus or bacteria that the body is fighting off. This is presently the favored theory of AIED. COCH5B2 has recently been reported to be a target antigen in AIED (Boulassel et al, 2001).
Intolerance: The ear, like the eye may be only an partially "immune privileged" locus, meaning that the body may not know about all of the inner ear antigens, and when they are released (perhaps following surgery or an infection), the body may wrongly mount an attack on the "foreign" antigen.
In the eye, there is a syndrome called "sympathetic ophthalmia", where following a penetrating injury to one eye, the other eye may go blind. The corresponding situation in the ear would be to go deaf in one ear, following trauma or surgery performed on the opposite ear. This theory is not presently in favor for the ear. Nevertheless, over about 20 years of practice the author of this page has seen several patients who develop ear disease in a delayed fashion in the opposite ear treated for anacoustic neuroma, including one patient following gamma knife "surgery". There have also been cases reported of development of AIED in the opposite ear after surgery forMeniere's disease (Ochoa and Weider, 2003), 11 years after a temporal bone fracture (ten Cate and Bachor, 2005), and in 2 of 148 patients after stapes surgery (Richards et al, 2002). Lacking much organized data other than the stapes study, and considering our personal observations, we suspect that this occurs in about 1% of patients in which inner ear antigen is released into the body.
These data are thus conflicted. If there is indeed an association with HLA, at least in certain populations, it would suggest that more of Menieres disease and other progressive syndromes may be caused by immune dysfunction than is presently generally thought. It is important to remember that HLA-typing is relevant when considered in the context of the patient's genetic background. In other words, studies of Korean subjects for example, such as reported by Yeo, may not apply to persons of non-Korean ethnicity.
How is the diagnosis of Autoimmune Inner Ear Disease made?
The diagnosis is based on history, findings on physical examination, blood tests, the results of hearing and vestibular tests, MRI scans, and response to immunosuppressive medications. The usual clinical picture is a subacute bilateral progressive sensory neural hearing loss. Asauditory neuropathycan present with a progressive bilateral sensorineural hearing loss, testing should be done in persons with enough hearing for the test to be practical. Otoacoustic emmission tests should be done in those in whom ABR testing cannot be done. MRI scans ot the brain are useful to diagnose Susac's syndrome (see above), as well as to exclude possible confounding disorders, such as acoustic neuroma.
While specific tests for autoimmunity to the inner ear would be desirable, at this writing (7/2004) there are none that are both commercially available and proven to be useful. (Garcia Berrocal et al, 2002).