Hearing Dysfunction in Sjogren's Syndrome

ABSTRACT

Background. Hearing dysfunction, caused by the involvement of the vestibulocochlear nerve or by direct damage on inner ear structures has been described in patients with Sjogren's Syndrome (SS). Previous studies evaluating the prevalence and incidence of hearing dysfunction in SS showed conflicting results, therefore, to date, the exact prevalence has not been extensively evaluated. Objectives. The aim of this study is to evaluate the prevalence of hearing involvement in patients with primary SS (pSS). Materials and methods. Patients with pSS (AECC criteria) with >=18 years of age attending a dedicated Sjogren's syndrome clinic were consecutively enrolled Auditory function was investigated by pure tone audiometry (PTA), It-Matrix test (Speech Reception Threshold in noise leading to 50% correct sentences-SRT) and the Hearing Handicap Inventory (HHI) during a baseline visit and at a follow up visit. A questionnaire of auto-evaluation of hearing loss impact on life was also administered to the patients. Results. Twenty-five patients with pSS (24 females) were enrolled in the study. The median age was 56.2 years (IQR 49-64) The mean disease duration was 3.7 years, 8 were treated with hydroxychloroquine (HCQ) and 1 with methotrexate. At baseline evaluation PTA revealed hearing loss in 17 patients (68%) with severity ranging from mild to severe. Fifteen patients (60%) presented mild hearing loss, 1(4%) moderate e 1 (4%) severe. The It-Matrix score ranged from -9.9 to 0.9 (median - 3.50). Median HHI score was 12.17 (min 0, max 68, SD 177.9). For Covid restrictions, a follow-up evaluation was available for 10 patients only. In these patients, a worsening of PTA and HHI was observed. Interestingly, the it-Matrix scores of patients with a stable disease showed an improvement. Conclusions. These preliminary findings suggest that hearing involvement is common in patients with SS and that it progresses over time. If confirmed on larger cohorts, these data will be useful for physicians in counseling patients about their disease and, in case of suspicious symptoms, an early evaluation by an otolaryngologist may prevent delay in diagnosis and allow an appropriate diagnostic evaluation and therapeutic intervention.