Timothy C. Hain,
MD • Page last modified:
July 19, 2020
Autoimmune inner ear disease or "AIED" consists of a syndrome of progressive hearing loss and/or dizziness which is caused by antibodies or immune cells which are attacking the inner ear.
At the present writing (10/2003), most do not feel that the "anticochlear antibody" blood tests for AIED are sensitive or specific, so the title of this page is somewhat of a misnomer.
As of 2/1999, a test called the Western blot immunoassay for anti-Hsp70 antibodies was reported to be correlated with active disease and steroid responsiveness in patients with bilateral rapidly progressive sensorineural deafness. This test is also called the "Heat Shock Protein" test or the anti-68KD (hsp 70) test by the Mayo Medical Lab.
The anti-68 KD test is closely related and will be considered here together with anti-hsp 70 (Shin et al, 1997). Gottschlich and associates reported 22% positivity in this syndrome. Unfortunately, in addition to being insensitive (only 22%) it also is not a specific test for AIED as 33% of patients with Meniere's disease are positive for anti-hsp70 and about 5% of the normal population also has positive antibodies (Shin et al, 1997; Gottschlich et al, 1995). Anti-HSP is not found in patients with otosclerosis or Cogan's syndrome (Moscicki et al, 1994).
More recently, several groups reported that the incidence of positive HSP-70 tests was identical in persons with clinical AIED and the general population (e.g. Yeom et al, 2000). Yeom et al (2003) later suggested that the HSP-70 test is directed against the wrong substrate (i.e. it is not a test for AIED at all). Until better data is available, at the present writing a trial of medication should be undertaken in appropriate cases defined by clinical criteria whether or not this test is available.