Case Presentation:
We present our case, which is a 37-year-old male middle eastern with a past medical history of Haemophilia A. He presented to our outpatient ophthalmology clinic with a right eye sudden decrease in vision for four days. On evaluation of the eye, he had features of unilateral pan-uveitis with para-macular retinitis, mild vitritis, peripheral punctuate retinal hemorrhage, and macular exudative retinal detachment, findings highly suggestive of Behçet’s disease. He was started on a tapering dose of oral prednisolone and referred urgently to the Rheumatology clinic. Upon enquiring, he mentioned a history of recurrent painful oral and genital ulcers for several years for which he did not seek medical attention before. On review of systems, he had no history of joint pain or swelling, no history of skin rash, respiratory or gastrointestinal symptoms, and no history of deep vein thrombosis.
On examination, no oral ulcers or scars were present, but he had one papule on the scrotum. Review of other systems were unremarkable. Blood tests (table.1) at this point were unremarkable, including autoimmune screening apart from slightly elevated C-reactive protein (CRP, 17 mg/dl). HLA typing for HLA-B51 was negative. Pathergy test was negative. A clinical diagnosis of Behçet’s disease was made based on history of recurrent painful oral ulcers, genital ulcers and panuveitis. Hence, Immunosuppressive therapy was started with prednisolone, azathioprine and infliximab.
After a few weeks of treatment, his vision improved with a resolution of signs of uveitis. He was maintained on azathioprine and infliximab, with the latter stopped after 15 months.
The patient was referred after seven months to an audiology clinic for evaluation of tinnitus and hearing loss. Symptoms of hearing loss had started in left ear almost at the time of panuveitis but it stabilized after starting immunosuppression and later involved the right ear, few months after stopping Infliximab. Physical examination of the ears were normal, with intact tympanic membrane bilaterally. A pure tone audiogram showed bilateral sensorineural hearing loss more on high frequencies (Figure 1). Magnetic resonance imaging (MRI) of the head and internal auditory meatus was done and were normal (Figure 2). All vestibular tests including video-nystagmography, oculomotors and post headshake test, computerized dynamic posturography, caloric testing, vestibular evoked myogenic potential and video head impulse test were unremarkable. Based on the previous diagnosis of Behçet’s disease, the sensorineural hearing loss was attributed to cochlear involvement by autoimmune inner ear disease related to Behçet’s disease. The patient was kept under close follow-up by Rheumatology, Ophthalmology, and Audiology. He was started on oral steroid prednisolone 70 mg per day for one month in addition to 4 doses of intratympanic steroid injections, which partially relieved symptoms. However, he continued to have repeated hearing loss episodes whenever steroid doses were tapered. Infliximab was resumed as a steroid-sparing agent with a dose of 5mg/kg every six weeks, along with azathioprine 100 mg per day and it helped to successfully taper and stop steroid. The patient was followed for the next two years, and his symptoms were controlled with stable audiogram levels.