Previous studies have reported mild to moderate hearing loss in patients carrying biallelic mutations in OTOF gene for the temperature-sensitive phenotype (Varga et al. 2006; Romanos et al. 2009; Marlin et al. 2010; Wang et al. 2010; Matsunaga et al. 2012; Zhang et al. 2016), in one case of progressive hearing loss (Chiu et al. 2010), and in some siblings of two families with intrafamilial variable severity of hearing loss, which may suggest a progressive phenotype (Yildirim-Baylan et al. 2014; Fedick et al. 2016). Here, OTOF is linked to hearing loss disorder.