The neuroepithelial cells on the inside your inner ear contain hair cells, their function is to receive and transduce sound and signals. It has been recently discovered that mutations in these neuroepithelial cells cause deafness in humans. Concurrently it has been found that the Ames Waltzer (av) gene, (a recessive gene found in mice), causes deafness and balance disorders, due to the degradation of the inner ear neuroepithelial cells. It can also be seen that the Ames gene shows a deficit for a transmembrane protein called protocadherin. This is the first evidence that protocadherin is required to stop the degeneration of the neuroepithelial cells.
Neuroepithelial cells are a subtype of stem cell. When dividing, the neuroepithelial cell produces 2 identical offspring. After primary division, the daughter cells another identical daughter cell as well as a non-stem-cell progenitor or a neuron by dividing again. Mice, when showing symptoms of deafness caused by the av gene also exhibit hyperactive and circling behavior. As a mice are used as a mammalian model the same symptoms in humans should be shown, yet humans made deaf by the av gene do not exhibit this behavior.
http://www.nature.com/ng/journal/v27/n1/full/ng0101_99.html
