This study was performed to investigate the association between human leuko
cyte antigen (HLA) and susceptibility to sudden sensorineural hearing loss
in the Korean population. HLA-A and HLA-B typing using a standard microlymp
hocytotoxicity technique and HLA-DRB1 genotyping were performed in 35 patie
nts with sudden sensorineural hearing loss and in 206 healthy controls. Pre
dnisone (usual dose 60 mg/day) was administered for 6 days and tapered for
an additional 4-6 days. Both initial hearing levels at the onset of deafnes
s and final hearing levels after treatment were examined and evaluated for
association with HLA alleles. The frequency of HLA-DRB1*14 was increased in
patients with sudden sensorineural hearing loss compared with controls (re
lative risk [RR] = 2.7, p = 0.016). The frequencies of HLA-A2, -A31, -B52:
-B61, -DRB1*04, -DRB1*11 and -DRB1*12 were slightly higher than in the cont
rols, but did not reach statistical significance. When an association betwe
en the treatment results and HLA alleles was also evaluated, the frequency
of HLA-DRB1*04 was found to be increased in the patients who did nor respon
d to steroid treatment compared with both patients who responded well to st
eroid (50% vs 16%, p = 0.034) and controls (RR = 3.0, p = 0.046). These res
ults suggest that there is an association between HLA-DRB1*14 and disease s
usceptibility and that the presence of HLA-DRB1*04 may be an useful marker
for predicting a poor prognosis in Korean patients with sudden sensorineura
l hearing loss.