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Gene Therapy Could Help Prevent Adult-Onset Hearing Loss

When a child is born, many people enjoy pointing out which physical features were inherited from each parent. Maybe the baby has her mom’s eyes or her dad’s smile, or she might have the same hair color as her grandfather or the same nose shape as her grandmother. However, did you know that you could inherit much more than eye or hair color? Researchers have long known that hearing loss could be inherited as well. Now, new research promises to reveal the exact genetic variant responsible for adult-onset hearing loss.
Although researchers have known for years that adult-onset hearing loss could be inherited—with heritability being responsible for an estimated 30 to 70 percent of cases —scientists had previously not known the cause. Research has already identified 118 genes linked to early-onset hearing loss (child or congenital hearing loss), but until now, none had been connected to adult-onset hearing loss. A new study appears to have identified one particular genetic variant that is potentially linked to adult-onset hearing loss.
The study was conducted by researchers at the Radboud University Medical Center in the city of Nijmegen in the Netherlands. The researchers used family and cohort studies featuring families with hearing loss. By using exome sequencing and characterization of the hearing phenotype, researchers discovered a single genetic variant that was present in 39 of 40 family members with confirmed hearing loss. The genetic variant is identified as RIPOR2. In the study, this genetic variant was also found in two individuals without hearing loss, as well as in 18 of 22,952 randomly selected people for whom no hearing loss information is available.
The authors of the study estimate that the RIPOR2 variant is present in “more than 13,000 individuals who are therefore at risk of developing HL or have developed HL already due to this variant.” The study indicates that in northwest Europe alone, this genetic variant may be present in approximately 30,000 additional individuals, indicating their risk for adult-onset hearing loss.
While this genetic variant is quite common—meaning many people are at risk for this particular type of adult-onset hearing loss—the authors of the study are optimistic that gene therapy may hold promise for prevention. “Because of the large number of subjects estimated to be at risk for HL due to the […] RIPOR2 variant, it is an attractive target for the development of a genetic therapy. The great progress that is being made in hearing disorders is promising.” Now that a particular genetic variant tied to adult-onset hearing loss has been found, gene therapy can specifically target RIPOR2 in an effort to prevent or minimize hearing loss.
Of course, additional research remains to be done. While gene therapy can provide hope to those looking to prevent adult-onset hearing loss, the therapies must still be developed. Furthermore, researchers will continue to study particular genes that may be linked to hearing loss.
To learn more about adult-onset hearing loss and how gene therapy could help prevent hearing loss, we invite you to contact our hearing practice today. We are happy to answer your questions and provide you with the care you need.

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What to Do If Your Hearing Aids Are Dropped or Get Wet

Wearing hearing aids can be life changing. If you or a loved one suffers from hearing loss and uses hearing aids, you know how critical they are in helping you hear. This not only means that you can watch the TV without turning up the volume—hearing aids can improve your relationships with friends and family, as well as boost your social life.
But what do you do when your hearing aid is damaged by dropping it or getting it wet? It can be easy to panic, but it’s important to remain calm and take a few simple steps that can help to prevent further damage. Here are some helpful tips and tools:

  • If you drop your hearing aid:
    • Whenever you wear a hat, headband, or another type of headgear, be aware that it may catch on your hearing aid and pull it out of your ear. Be careful when you remove your headgear (or anything else near your head, ears, or neck) to ensure that your hearing aid is not pulled out. Check immediately afterward that your hearing aid is still in place.
    • If you realize that your hearing aid has fallen out and has been dropped, stop right where you are. Ask anyone with you to stop as well and to stand still until you have searched for your hearing aid (so no one will step on it if it is on the ground nearby). Start by patting down your clothing, starting at your neck and making your way downward; your hearing aid might have fallen and gotten caught on your clothing.
    • If your hearing aid is not on your person or in the immediate area, think of when you last knew you had it and whether anything had brushed your ears since then. Carefully retrace your steps to see if you can find your hearing aid. If you find it and it has been damaged by the drop, contact your hearing aid professional.
  • If your hearing aid gets wet:
    • First, your hearing aids should never be worn when you are in water, whether that is the shower, bath, ocean, or swimming pool. Make it a habit to feel for your hearing aids with both hands before entering water. Put a note by the shower or clipped onto your swimming suit to remind yourself to remove your hearing aids. Keep a case handy to put your hearing aids in to keep them safe while you are in water.
    • If you do get your hearing aids wet, immediately remove them from both ears and dry them with a towel. Open the battery drawer and remove the batteries. Place your hearing aids in their case or a hearing aid dryer with the battery door open. Never use a hairdryer, microwave, or oven to dry your hearing aids. Allow the devices to rest for about one hour or overnight.
    • If your hearing aid gets wet by being dropped in a toilet, carefully consider whether it is safe to retrieve them. If you are in a public restroom or the toilet is un-flushed, you will likely want to consider them a loss. If you are at home and the toilet is clean, you may want to retrieve them and follow the steps above to dry them.
    • If your hearing aids have gotten wet and no longer work after you dry them, allow them to rest, and insert new batteries, contact your hearing aid professional.

Following these simple steps can help to keep your hearing aids safe and in working order. If your hearing aids have been damaged, do not hesitate to contact your hearing aid professional for additional assistance. We are eager to help!