Connexin 26 sensorineural hearing loss is a type of nonsyndromic deafness caused by an error or mutation in a gene which codes for Connexin 26. Connexin 26 is a protein found in cells throughout the body, including the cochlea of the inner ear. The cochlea is the snail-shaped structure which aids in hearing by changing the vibrations we hear into signals that can be sent to the brain through our nerves. Connexin 26 helps form channels or connections between cells so that the cell may communicate with each other. These channels are important to the cells of the cochlea. If an individual has an error in the gene for Connexin 26, the protein will not work or function as it should. The cochlea cannot function properly without Connexin 26 and congenital deafness often results. This means the baby with a mutation in the gene for Connexin 26 may be born with complete hearing loss. Mutations that affect Connexin 26 are responsible for at least 20% of all genetic hearing loss and 10% of all childhood hearing loss. There type of sensorineural hearing loss is not usually associated with other birth defects (nonsyndromic). There are a number of options available for individuals with deafness or sensorineural hearing loss, including learning sign language, working with a speech pathologist to learn spoken language, and/or cochlear implants. Parents should seek information about the range of options open to their child before deciding which options may work best for their child and family. Specialists may include an otologist, an audiologist, a speech-language pathologist, a clinical geneticist, and a genetic counselor. Support organizations and other families may also be excellent resources.