Stickler Syndrome and Hearing Loss

Stickler syndrome is a genetic condition named for the man who first described it in 1965, Dr. G.B. Stickler. It affects collagen, a form of protein that composes the body?s bones and connective tissues (tendons, cartilage, etc). The effects of Stickler syndrome vary widely between each person who has it; some people experience many of its symptoms, while others experience almost none.

Researchers estimate that at least 40 - 60% of people with Stickler syndrome experience hearing loss as a result. The problem can be present from birth or develop over time. If your life has been interrupted by a hearing impairment, HearingPlanet can help you find the solutions you need. Call our hearing health professionals today at 1-800-432-7669.

Bone Structure and Hearing Loss

Because Stickler syndrome is so rare, affecting about one in every 9,000 newborns, doctors still have a lot of questions about it. It is not always clear why an individual person with this syndrome has hearing loss, but some possible reasons include:

• Abnormalities of the Eustachian tube. This is a tube that links the middle ear and the throat. It plays an important role in stabilizing pressure in the ears and draining mucus from them. Many people with Stickler syndrome are born with cleft palates, which can damage this tube.
• Sensorineural hearing loss. The link between this syndrome and damage to the auditory nerve is likely related to facial structure abnormalities. Sensorineural hearing loss often affects the higher frequencies of sound, and often progresses over time.
• Abnormalities in the middle ear. This is the part of the ear containing three small bones known as ossicles, as well as the ear drum. When the ossicles are poorly formed or not working correctly, hearing will be impaired. This is also known as conductive hearing loss.

Anyone with Sticker syndrome will need regular hearing checks throughout his or her life. While some will never develop hearing problems, others may need hearing aids or other assistive devices to counteract hearing impairment.

Contact a Hearing Aid Specialist

At HearingPlanet, our goal is to connect hearing impaired people with the technology they need. Contact our hearing aid experts today at 1-800-432-7669.