Fig. 3

Effect of anti-IL-1 therapy on hearing assessments in patients with MWS. a Comparison of two different anti-IL-1 therapy regimens. A total of 11 assessments (ears) of 9 patients improved with anti-IL1 therapy; 4/9 were children, five were adults. None of the patients had worsening of the contralateral ear. Improvement occurred with both treatment regimens. All but two patients with hearing improvement were females. Overall, worsening was exclusively observed in adult patients, a male and a female. Both patients had received anakinra therapy. b Responsiveness to change: Comparison between 4PTA and HF-PTA follow-up assessments after exposure to anti-IL-1 therapy. 4PTA and HF-PTA was calculated in all MWS patients following anti-IL-1 therapy. Hearing improved in five children and six adults, most commonly captured by HF-PTA (10/11), either by HF-PTA only (5/10, light grey bars) or also by the standard 4PTA (5/10, medium grey bars). Hence, a total of 91 % of improved assessments were detected by HF-PTA, while just in one patient improvement was detected by standard 4PTA only. Worsening was not documented in children, but deteriorated in two adults. In both, this was detected by standard 4PTA, in one also in the HF-PTA