Background Systemic sclerosis (SSc) is one of the most complex systemic autoimmune diseases1. Ear involvement is not uncommon and should be taken into consideration during diagnostic and therapeutic procedures of SSc2
Objectives The aim of the present study was to assess hearing in asymptomatic SSc patients and correlate the findings with clinical characteristics and disease severity.
Methods The study included 35 female SSc patients and 35 matched controls. Skin thickness was assessed using modified Rodnan skin score (mRSS) and disease activity by Medsger severity score (MSS). Audiological tests including pure tone audiometry, speech audiometry, impedance audiometry and auditory brainstem response (ABR) were performed
Results The mean age of patients was 40.3±6.2 years, and disease duration 7.3±4.6 years. Mild bilateral symmetrical sensorineural hearing loss (SNHL) was present in 27/35 (77.1%) mostly at high frequencies. Hearing thresholds, pure tone average and speech reception threshold were significantly higher (p=0.001) in SSc patients than in control. In those with hearing loss, word discrimination score (WDS) was lower and ABR-I delayed compared to those without (p=0.001,p<0.0001 respectively) with consequently shorter inter-peak interval (IPI) I-III (p=0.01) and IPI I-V (p=0.004). In patients with hearing loss there was an increased frequency of acro-osteolysis (51.9%), telangiectasia (59.3%), arthralgia (70.4%) and peripheral neuritis (44.4%) compared to those with normal hearing. The MSS and mRSS significantly correlated with the high frequency hearing threshold and negatively with WDS. Audiometric parameters were comparable between patients with diffuse (n=14) and limited (n=21) cutaneous SSc.
Conclusions Audiometric tests may detect subclinical SNHL mostly cochlear in SSc patients. The significant association between SNHL with telangiectasia and neuritis raises the likelihood that the SNHL may be due to an underlying vasculopathy or neuritis.
Pattanaik D, Brown M, Postlethwaite BC, Postlethwaite AE. Pathogenesis of Systemic Sclerosis. Front Immunol. 2015;6:272.
Maciaszczyk K, Waszczykowska E, Pajor A, Bartkowiak-Dziankowska B, Durko T. Hearing organ disorders in patients with systemic sclerosis. Rheumatol Int. 2011;31(11):1423–8.
Disclosure of Interest None declared