Clinical features of acute profound deafness related to immunological impairments were investigated in 14 cases which satisfied the diagnostic criteria proposed by the Committee of “Acute Profound Deafness” of Japanese Ministry of Health and Welfare.
The results obtained were as follows;
1) Both ears satisfied the diagnostic criteria in only 5 cases out of 14 in spite of bilateral hearing impairments in all cases.
2) There were no remarkable features in onset of hearing loss or in associated symptoms at the onset of hearing loss.
3) Associated autoimmune disease was confirmed in only 4 cases out of 14. However, abnormal test results which suggested the existence of immunological impairments were found in all cases.
4) The positive TPHA tests were found in 3 cases out of 4 male patients.
5) The mean age at the onset of hearing loss was 39.0 years old.
6) The mean time interval of onset of hearing loss in both ears was 1 year and 10 months except 3 cases.
7) When the best record of pure tone audiogram was compared with the worst one after the start of steroid therapy, about 29dB was found as the fluctuation in mean hearing level at 5 frequencies between 250Hz and 4 kHz.
8) When the final audiogram was compared with the initial one, only 6 dB was found as the deterioration in mean hearing level at 5 frequencies between 250 Hz and 4 kHz.
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