CDH23 plays a crucial role in the normal organization of the static cilia bundle, and mutations in the CDH23 gene cause disturbances in the stereocilia, leading to disorders in the conversion of sound waves into electrical signals in the hair cells, causing noise deafness and Age-related hearing loss (AHL) (Noben-Trauth et al., 2003). The gene discussed is CDH23; the disease is acute hemorrhagic leukoencephalitis.