AUTOIMMUNE INNER EAR DISEASE
DESCRIBED BY McCABE 1979
USUALLY MANIFESTS AS A SNHL +/- VESTIBULAR Sx OVER A PERIOD OF DAYS TO WEEKS—–TOO SLOW TO BE SSNHL OR VIRAL LABYRINTHITIS AND TOO FAST TO BE PRESBYCUSIS—-USUALLY WEEKS TO MONTHS
MEAN AGE 29
SDS CAN BE EITHER GOOD OR POOR
70% + AB TO INNER EAR AG (68 KD PROTEIN—OR 66 KD– (HEAT SHOCK PROTEIN–70)-MAY BE OUT LIERS)—WESTERN BLOT
NOW LTT (LYMPHOCYTE TRANSFORMATION TEST)
NO EVIDENCE OF OCULAR PATH (INTERSTITIAL KERATITIS/EPISCLERITIS)—THINK COGANS OR SYPHILIS
ALL OTHER STUDIES –NEGATIVE—C-ANCA, AUTOIMMUNE PROFILE
SNHL IMPROVES WITH IMMUNOSUPPRESSION—STEROIDS—-90% RESPOND TO TREATMENT—HAVE BEEN ABLE TO INDUCE SNHL IN LAB ANIMALS WITH SPECIFIC ANTIBODIES
CAVEATE: 25% WILL HAVE OR WILL DEVOLOPE CONCOMMITANT AUTOIMMUNE DESEASES (COUNSEL YOUR PTS ON THIS)—CASES OF LATER AORTITIS AND DEATH (ANOTHER WAY TO LOOK AT IT IS THAT ONLY 1/3 OF THESE PATIENTS WILL DEV. ASSOC AUTOIMMUNE DISEASE)
ELS=THE SEAT OF THE IMMUNE SYSTEM IN THE INNER EAR—ICAM-1, IGM, ECT…
INFLAMMATION CAN LAST 3 WEEKS PAST THE INSULT—-SO TREAT—-SEE FIBRO-OSSEOUS PROLIFERATION WITHIN THE SCALA TYMPANI—-RESULTANT HYDROPS
RX:  USUALLY TREAT WITH 1 MG/KG FOR AT LEAST A MONTH (20MG/KG PO TID X 1 MONTH)—60-MG PO Q.D. FOR 1 MONTH AND ALSO GIVE ZANTAC—-IF THEY RESPOND—TAPER PREDNISONE—-GIVE MTX 15 MG Q WEEK WITH 1 MG OF FOLATE—-IF NO RESPONSE OR FLORID DISEASE ADD CYTOXAN (CYCLOPHOSPHAMIDE) OR IMMURAN (AZATHIOPRINE) OR PLASMAPHORESIS.