ABSTRACT
The auditory system is one of the most remarkable organs in vertebrate organisms. Not only is the auditory system required for our perception of sound, but also for our perception of spatial orientation. Although much is known about how this system develops and functions, little is known about the role of cilia during these processes, which is unfortunate considering that auditory dysfunction is one of the phenotypes of several human ciliopathy syndromes. In this chapter we summarize what is currently known about cilia function in the auditory system with a particular focus on the mammalian cochlea and the kinocilium found on the hair cells of the Organ of Corti. We describe the different regions of the cochlea in which primary cilia have been identified and discuss signaling pathways that are thought to be regulated by cilia function in the inner ear. We examine the auditory phenotype of various ciliopathy animal models and conclude by discussing human diseases relevant to ciliary defects in the auditory system.
