Pendred syndrome is a genetic disorder that causes early hearing loss in children. It also can affect the thyroid gland and sometimes may affect a person’s balance. The syndrome is named after Vaughan Pendred, the physician who first described individuals with the disorder.

Children who are born with Pendred syndrome may begin to lose their hearing at birth or by the time they are three years old. The hearing loss is progressive, which means that a child will have less hearing over time. Some individuals may become totally deaf.

The loss of hearing often happens suddenly and in stages. Sometimes, after a sudden decrease in hearing, a person’s hearing will nearly return to its previous level. Almost all people with Pendred syndrome have bilateral hearing loss, or hearing loss in both ears. The hearing loss often is greater in one ear than in the other.