For example, in ciliopathy models exhibiting both cochlear shortening and polarity defects, including Ift88, Kif3a, Ift27, Cilk1, and Tbc1d32bromi mutants (Figure 2; Jones et al., 2008; May-Simera et al., 2015; Sipe and Lu, 2011), SHH signaling is impaired (Table 2; Bangs and Anderson, 2017; Eguether et al., 2018; Huangfu et al., 2003). Here, SHH is linked to ciliopathy.