Autoimmune Inner Ear Disease and Hearing Loss

Autoimmune Inner Ear Disease (AIED)

AIED is the progressive hearing loss syndrome accompanied by dizziness that is caused by the immune cells (or antibodies) which gets activated and attacks the cells in the inner ear, mistaking it for a toxin, virus or bacteria.

The Ear, Hearing and AIED

In majority of the cases, there is a gradual reduction of hearing over months which are accompanied by tinnitus (ringing, roaring, hissing). Variants are mutual attacks of hearing loss and tinnitus that is similar to Meniere's disease, and attacks of imbalance and dizziness accompanied by abnormal blood test for antibodies.

The working of immune system is complicated and there are numerous ways that it can damage the inner ear. Both allergic and traditional autoimmune disease such as ankylosing spondylitis, ulcerative colitis, Sjoegren’s syndrome (dry eye syndrome), Wegener’s granulomatosis, systemic lupus erythematosus (SLE), Cogan’s disease, rheumatoid arthritis, psoriatic arthritis and scleroderma can cause or be associated with AIED. Bechet is another multisystem disease that is linked with audiovestibular problems.

There are numerous reasons as to how these might arise, corresponding to other types of autoimmune disorders:

  • Bystander damage: Cytokines (regulatory proteins) are released as a result of damage to the inner ear which provokes (after delay) further immune reactions. This is the rationale which might explain the attack/remission cycle of disorders like Meniere's disease.

  • Cross-reactions: Accidental ear damage can be caused by antibodies or rogue T-cells because the ear shares the common antigens with potentially harmful toxins, virus or bacteria that the immune system tends to fight.

  • Intolerance: Like the eye, ears may only be moderately immune privileged locus. This means the immune system of the body may not be aware of all the inner ear antigens, and when they are released (for example, because of infection or surgery), the immune system may falsely mount an attack on the unfamiliar antigen.

  • Genetic factors: Genetically controlled characteristic of the immune system may escalate or otherwise be correlated with increased susceptibility to common hearing disorders, such as Meniere's disease.

Prevention

Majority of the patients suffering from AIED (Autoimmune Inner Ear Disease)respond well to the treatment of steroids, methotrexate and prednisone (a chemotherapy agent). Some patients may benefit from the use of hearing devices. In cases, where the patients don’t get much benefit from drug therapy or hearing aids, an inner ear cochlear implant may be considered.