Pendred syndrome is a genetic disorder that causes early hearing loss in children. Children born with Pendred syndrome may begin to lose their hearing at birth or by the time they are three years old. In most cases, their hearing will worsen over time.
Pendred syndrome is a genetic disorder that causes early hearing loss in children. Children born with Pendred syndrome may begin to lose their hearing at birth or by the time they are three years old. In most cases, their hearing will worsen over time. The loss of hearing often happens suddenly, and the condition may also affect the thyroid gland and sometimes create problems with balance. Eventually, some children with Pendred syndrome will become totally deaf.
Pendred syndrome accounts for about five to ten percent of hereditary hearing loss. Almost all children with Pendred syndrome have bilateral hearing loss, which means hearing loss in both ears, although one ear may have more hearing loss than the other.
Pendred syndrome isn’t curable, but a multidisciplinary medical team can offer informed choices about treatment options. They also can help prepare the child for increased hearing loss and other possible long-term consequences of the syndrome. Children with Pendred syndrome should start early treatment to gain communication skills, such as learning sign language or cued speech or learning to use a hearing aid. Most people with Pendred syndrome will have hearing loss significant enough to be considered eligible for a cochlear implant. While a cochlear implant doesn’t restore or create normal hearing, it bypasses injured areas of the ear to provide a sense of hearing in the brain. Children as well as adults are eligible to receive an implant.
If you suspect a child in your life may have a hearing loss, seek an immediate evaluation. An otolaryngologist will ask about the onset of the hearing loss, and evaluate inner ear structures and perhaps the thyroid in diagnosing Pendred syndrome. They also will evaluate the timing, amount, and pattern of hearing loss. Early hearing loss is one of the most common characteristics of Pendred syndrome; however, this symptom alone doesn’t mean a child has the condition. A physician may also use inner ear imaging techniques such as magnetic resonance imaging (MRI) or computed tomography (CT scan) to look for two characteristics of Pendred syndrome.
A good primer on Pendred Syndrome can be found on the National Institute on Deafness and Other Communications Disorders website here.
