Mutations in the genes encoding ciliopathy-associated proteins, ift88 (intraflagellar transport 88) and Kif3a (a component of the kinesin II motor) all of which are involved in maintenance of cilia and which localise in hair cells to the kinocilium or its basal body, result in varying degrees of mis-orientation and/or disturbance of bundle shape in some OHC [17–22]. The gene discussed is IFT88; the disease is ciliopathy.