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, vertigo, hissing sounds 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 attacks reoccur again and again.
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 of Meniere'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, and conditions of mysterious origin all account for some cases.
In animals, attempts have been made to treat variants of AIED with oral collagen (Kim et al, 2001). Relapsing polychondritis is a disorder in which there may be antibodies to collagen and acquired deafness.