Autoimmune inner ear disease and ulcerative colitis.

Dear Sir, We read with interest the paper by Jacob et al. (1990) on a case of sensorineural hearing loss which occurred in a patient with ulcerative colitis, episcleritis and giant cell arteritis. We would like to report upon two more patients, both of whom had ulcerative colitis without any previous systemic complications, who developed total deafness and were in fact seen in our cochlear implant assessment clinics. Case 1: A 30-year-old woman developed acute ulcer-ative colitis in 1982 and was managed with topical ste-roids and salazopyrine. In 1985 she developed vertigo and left sided hearing loss of sudden onset. Four weeks later she again developed vertigo and this time right sided hearing loss. On both occasions she was treated with histamine, dextran and steroids to no avail and when assessed in the cochlear implant clinic in 1988 she was totally deaf. She has since been implanted with a Nucleus 22 channel intracochlear device and has good open set speech with little recourse to lip reading. Case 2: A 33-year-old man had sudden onset of total right sided vestibulocochlear failure at the age of 15 at about the same time as being diagnosed as having ulcer-ative colitis. Three months later the hearing on the left side started to diminish and deteriorated over the space of a year until he developed total hearing loss. He is still under consideration for implantation. Neither patient had been treated with any commonly recognized ototoxic drug. The two main proposals for the aetiology of ulcerative colitis are i) infective and ii) immunological. Strong evidence was provided for the immunological theory when Takahasi and Das (1985) isolated an auto-antigen in normal colon to which much of the tissue-bound immunoglobulin in ulcerative colitis was specific. They later found the antigen in skin and biliary tract which would offer an explanation for the systemic effects of pyoderma gangrenosum and pericholangitis (Das et al., 1987). There have been several reports in the literature of SNHL associated with ulcerative colitis. Of note is that fact that the hearing loss often recovers, at least in part, with aggressive treatment with steroids and cyclophos-phamide or azathoprine (Summers and Harker, 1982, Weber etal., 1984, David and Rees, 1987). Plasmaphe-resis, although of value in some other cases of auto-immune hearing loss, proved disappointing in the treatment of a patient with sensorineural hearing loss and ulcerative colitis (Luetje, 1989). Autoimmune inner ear disease may occur …