Pendred Syndrome

Pendred syndrome is one of the most common causes of genetic hearing loss, affecting 2 to 3 out of every 1,000 live births in the US. It is named for the person who first described it in the 19th century, Dr. Vaughan Pendred. This syndrome can cause significant hearing loss at birth that will often increase throughout the child's life, sometimes leading to full deafness.

If your child has been diagnosed with a hearing condition of any kind, support is available. HearingPlanet can provide the information you need about a range of available hearing aids, including pricing and easy purchasing options. To learn more about the services we offer, call our hearing aid experts at 1-800-432-7669.

Pendred Syndrome and Your Child's Hearing

Pendred syndrome affects the development of a child's inner ear. It is often diagnosed with the use of an MRI or CAT scan to look for abnormalities in:

• The cochlea: Many people with Pendred syndrome develop an abnormality of the cochlea called Mondini dysplasia. This means that the cochlea - a snail shaped organ that contains audio receptor cells - has fewer twists than it should. As a result, the affected person may not have enough functional receptor cells to hear normally.
• The vestibular aqueduct: This is a small canal located outside of the cochlea. Pendred syndrome is characterized by an enlarged vestibular aqueduct, which causes excess fluid to collect in a small pouch attached to the canal, known as the endolymphatic sac. Although this is a recognized symptom of Pendred syndrome, its exact impact on people's health is not currently understood.

Children with this syndrome can suffer from a variety of symptoms in addition to hearing loss. One important issue is that their inner ears appear to be more susceptible to injury than average. This means that people with this condition must take care to protect their skulls and inner ears, as relatively minor head injuries can worsen their hearing impairments.

Contact a Hearing Aid Specialist

Children with Pendred syndrome often use hearing aids and other assistive devices to compensate for the hearing loss this condition causes. To learn more about hearing technology and available hearing aids, contact the hearing loss experts of HearingPlanet today at 1-800-432-7669.