A 39-year-old woman sustained an irreversible hearing loss in the left ear, followed 6 months later by a left facial paresis. Oral aphthous ulcers frequently recurred with fever of up to 39.5 degrees C. Her general condition was noticeably poor.
An inflammatory disease was suggested by an increased erythrocyte sedimentation rate (70/100 mm), leucocytosis (18,500/microliter), decreased haemoglobin (10.2 g/l) and leftward shift of the neutrophil granulocytes. Diagnostic tests for an infectious, autoimmune or haemato-oncological disease were negative.
Antibiotic and antimycotic treatment failed to bring lasting improvement. Thrombophlebitis and venous thrombosis developed in both upper limbs and acute episcleritis (scleritis) in the left eye. Behçet's disease with oral, ocular, neurological, cutaneous and vascular involvement was diagnosed and treated with chlorambucil (variable dosage around 7 mg/d orally), with rapid regression of symptoms. 3 months later joint pains set in, and another 18 months later she developed sicca symptoms (xerostomia with dry eyes): both responded to symptomatic treatment.
The combination of loss of hearing, sicca syndrome and Behçet's syndrome has not been previously reported.