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. As auditory 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).

Immunoflourescence of supporting cells of guinea pig organ of Corti (cochlea) has also been shown to correlate with disease and steroid responsiveness. According to Gray and others, immunoflourescence is more sensitive and specific (86%, 41%) than is Western Blot (59%, 29%) (Gray and others, ARO abstracts, 1999, #246). Western Blot is helpful in some hands (Garcia et al, 2003). Yeom et al (2003) recently reported that immunoflourescence is more sensitive and specific than anti-HSP 70. The specificity of both tests to us seems unacceptably low.

The lymphocyte transformation assay, like the anti-cochlear antibody test, is presently of uncertain value.

Antiendothelial antibodies may be associated with some cases of AIED (Cadoni et al, 2003). At this writing there is no commercially available test.

Several studies have reported an association between autoimmune thyroid disease and ear disease (Brenner et al, 2004; Medugno et al, 2000), which is the rationale for testing for anti-microsomal or thyroid peroxidase antibodies.

It has recently been reported that antibodies to sulfoglucuronosyl glycolipids are common in autoimmune inner ear disease. (Yamawaki M, 1998). It remains to be seen if this finding will be confirmed and whether a commercial assay will be developed.

At this writing (1/2003), it is not generally felt that anti cochlear antibody(also called anti-HSP70) blood tests are specific enough to be very useful. Antibodies to HSP-70 can also be found in Lyme disease, ulcerative colitis, cancers, and in about 5% of healthy individuals. Yeom et al (2003) recently suggested that all anti-HSP tests are directed against the wrong substrate. Whether this is true or not, because of the poor specificity of anti-HSP 70 testing, diagnosis is generally based on evidence from broader tests of autoimmunity, or a positive response to steroids.

A small study recently suggested that FDG PET scans may be useful in AIED. (Mazlumzadeh et al, 2003). More investigation of this modality is needed before it's role in diagnosis can be defined.

As there are no specific tests for AIED, a common approach is to look for other evidence for autoimmune involvement.

Blood tests for autoimmune disorders, ordered from most to least useful, include:

• Sed Rate and CRP   (the rest of the tests should not be done) 
• ANA
• Thyroid (anti-microsomal and thyroglobulin antibodies)
• Rheumatoid Factor
• Complement C1Q
• anti-gliadin and anti-endomysial antibodies (for Celiac disease)

 Blood tests for conditions that resemble autoimmune disorders, again from most to least useful, include:

• FTA (for SYPHILIS )
• HBA1C (for diabetes, which is often autoimmune mediated also)
• HIV (HIV is associated with a ear nerve damage as well as syphilis)
• Lyme titer

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