In this study, to better understand the role of primary cilia in the cochlea, we analyzed cochlear phenotypes in three different categories of ciliopathy mouse models, including no cilia (Ift88 mutants), morphologically defective cilia (Tbc1d32bromi mutants), and abnormally elongated cilia (Cilk1 mutants) (Jones et al., 2008; Ko et al., 2010; Moon et al., 2014). The gene discussed is IFT88; the disease is ciliopathy.