Treating AIED Autoimmune Inner Ear Disease
The treatment goal in people severely affected with AIED is
to improve their hearing/discrimination enough so that they
can benefit from wearing hearing aids. In those with mild to
moderate losses, the goal is to recover hearing to normal or
near normal levels.
Doctors think that AIED is
potentially reversible. Therefore they like to treat it with
steroid drugs and see if they can stop the progress of AIED
or make it retreat since steroids are the drug of choice for
other autoimmune diseases. Sometimes it seems to work.
Sometimes it doesn't do a thing. In fact, doctors don't have
a clue what would happen if no treatment was given since,
when patients come to them, they always "try" something. Dr.
Shelley Broughton wrote, "The natural history of untreated
immune-mediated inner ear disease is unknown at this time."
In other words, doctors don't know what would happen if they
kept their hands off and let nature take its course.
Because many doctors believe that AIED is caused by
inflammation and altered immunity, they try the same
immunosuppressive drugs they use to treat rheumatoid
arthritis and cancer. According to the American Academy of
Otolaryngology—Head and Neck Surgery, Inc., they use high
and Cyclophosphamide, but with "little
guidance about dosages or duration of treatment and with
less than ideal proof of efficacy."
Not all people respond to steroid therapy the same way. Treatment in some people results in better hearing, or better discrimination or both. With others, hearing fluctuation or hearing loss progression stabilizes where it is without improving any. With the remainder, they continue to lose their hearing in spite of immunosuppressive therapy.
In cases of rapidly progressing bilateral hearing loss, doctors try steroids such as Prednisone (Deltasone) or Dexamethasone (Decadron) for 4 weeks. If the person responds to this treatment, then they often put them on long-term chemotherapy drugs such as Cyclophosphamide (Cytoxan),Methotrexate (Rheumatrex<), orAzathioprine (Imuran).
In one study using Methotrexate, the patients had significant improvement in their discrimination scores, but did not have any improvement in their hearing.
Overall, steroid response rates are approximately 60%. The best relief is from intra ear injection. Success is defined as a hearing improvement of just 10-15 dB, or a "significant" improvement in discrimination scores. For a severe loss, this is not all that great an improvement, but at least the hearing loss isn’t progressing any more!
Plasmapheresis treatment may also be beneficial. This involves filtering the patient’s blood, which removes antibody, antigen, and immune complexes and other immune mediators. 75% of the patients in one study regained some of their hearing after this treatment.
The anti-TNF drug,Etanercept (Enbrel) shows promise too.
Prednisone, although an effective immunosuppressant drug, has a host of negative side effects, especially in the long term. You want to be aware of them. You need to know that you can get both cataracts and glaucoma (among other things) from takingPrednisone. If thePrednisone treatment isn’t successful in restoring your hearing, you could find yourself up a creek without a paddle, ending up both hard of hearing and without much sight.
You also want to be careful when taking any cytotoxic drugs because their side effects can also be serious. For example,Cyclophosphamide is often prescribed, yet it can cause increased risk of malignancy (cancer).
The best drug for early treatment is