SHH and deafness: Preaxial polydactyly has been associated with ectopic Shh expression in limbs (Dunn et al., 2011; Johnson et al., 2014; Lettice et al., 2003; Lettice et al., 2008; Liem et al., 2009; Zhulyn and Hui, 2015); Anal stenosis or imperforate anus have been related to misregulation of Shh pathway (Kang et al., 1997; Mo et al., 2001; Roberts et al., 1995), as well as deafness and dysplastic ears (Driver et al., 2008).