Alport syndrome is a serious disease caused by mutations in genes that provide instructions to make a structural protein called type 4 collagen. These mutations lead to faulty or no type 4 collagen produced, which causes the symptoms of Alport syndrome — kidney disease, eye abnormalities, and hearing loss.

Hearing loss generally begins when patients are in early adolescence and can lead to profound deafness by middle age. Most patients with Alport syndrome are good candidates for hearing aids.

What is a hearing aid?

Hearing aids are medical devices that contain a microphone, which carries sound to a computer chip. This computer chip measures the sound, amplifies it based on settings and user preference, and transmits it to speakers, which send it to the ear. All hearing aids require a battery, but there are many styles of hearing aid that vary widely in style, price, and complexity.

For example, there are hearing aids that can be looped over the ear, in the ear, or in the ear canal, depending on what is most comfortable and secure for the patient. Some styles may be better for young children or those who play active sports.

In some U.S. states, financing may be available for patients who cannot afford their hearing aid. The Hearing Loss Association of America has more information about financing in each state.

Programmable hearing aids

Hearing aids can be programmed to reduce ambient noise such as background noise during conversations. Some hearing aids have wireless connectivity so that patients can control their hearing aid through Bluetooth-compatible devices, like a cellphone, or play sound from a music player or television.

For patients with two hearing aids, programming allows synchronization between the hearing aids. So adjusting the volume or programming on one of the hearing aids also adjusts the settings on the other.

Some hearing aids may have remote controls or direct audio input, for example from a music player or television. Some also have a telecoil, which is an internal component that is almost like a little antenna. The telecoil allows the hearing aid to pick up signals directly from compatible telephones and sound systems, such as churches, concerts, and some movie theaters, minimizing background noise and feedback between devices.

Hearing aids for Alport syndrome

Alport syndrome patients generally develop binaural deafness, that is, both ears become deaf. Therefore, most audiologists will recommend patients wear hearing aids in both ears.

Once a hearing test shows a patient has hearing loss that can be managed by a hearing aid, the patient’s audiologist will discuss the options available.

Other information

It is important to keep hearing aids away from moisture and heat. Patients should not use hairspray or other hair care products while wearing their hearing aids.

Hearing aids should be turned off when not in use to preserve the batteries. Dead batteries should be replaced immediately.

Patients should clean their hearing aids as recommended by the manufacturer, as earwax can damage the hearing aids.

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Alport Syndrome News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.