Hearing organ disorders in patients with systemic sclerosis

In systemic sclerosis (SSc), there may develop hearing and balance disorders as a result of the immune-mediated vasculitis and fibrosis in the inner ear. The objective of the study was evaluation of the hearing organ function in patients with SSc with relationship to duration of the disease and Raynaud phenomenon and also to type and severity of the disease. Twenty unselected, consecutive patients with SSc diagnosed in compliance with the international diagnostic criteria of the American Rheumatism Association (1982), were enrolled into the study. The control group consisted of 26 otologically healthy persons matched to the SSc group for age and sex. Case history was recorded for all patients from questionnaire data. Otolaryngological examination and battery of audiological tests (pure tone audiometry, speech audiometry, impedance audiometry and auditory brainstem response—ABR) were performed. In the anamnesis 60% of patients reported vertigo, 55% headaches, 50% tinnitus, 40% hyperacusis, 40% hearing loss and 30% ear fullness. It was found that patients with SSc had significantly poorer mean hearing thresholds than the control group for 0.5, 1, 6 and 8 kHz. In ABR there were no differences between SSc and control groups although an increase of latency averages in the group of limited patients with SSc compared with the diffuse patients with SSc was observed. In eight patients (40%) sensorineural hearing loss, mostly bilateral and symmetrical was found. Furthermore, no relation was seen between hearing level and duration, type and severity of the disease. Ear involvement is frequent in systemic sclerosis and should be taken into consideration during diagnostic and therapeutic procedures.