In contrast, in ciliopathy models displaying polarity defects but normal cochlear length, including Alms1, Gmap210, Ift25, and Tmem67 mutants (Abdelhamed et al., 2015; Leightner et al., 2013; May-Simera et al., 2015), SHH signaling is unaffected (Table 2; Chen et al., 2017; Keady et al., 2012; Chen et al., 2017; Smits et al., 2010). Here, ALMS1 is linked to ciliopathy.