Advancements in Hereditary Hearing Loss

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Advancements in Hereditary Hearing Loss

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Have you ever wondered why so many of your family members experience hearing loss around the same age? This pattern may not just be a simple coincidence but may actually be the result of a common problem called hereditary hearing loss.

Since many forms of hearing problems are caused by genetics, researchers have been working extremely hard to develop a solution. Hereditary hearing loss can begin at birth, referred to as congenital hearing loss. It may also occur as a delayed onset of hearing loss, often developing at a later point in life.

Hereditary hearing loss accounts for about 35-55% of all age-related hearing loss cases. Gene mutations often contribute to this type of hearing loss. Certain genes allow some individuals to be predisposed to hearing problems, either by aging or induced by noise, drugs or infections.

Different genetic syndromes may also influence hearing problems. Conditions such as Usher Syndrome and Pendred Syndrome factor in hearing loss as one of the symptoms of the disorders.

Results from a recent research study may provide a glimmer of hope for the future of hereditary hearing loss treatment. Advancements that were discovered may help stop the progression of this type of hearing loss and preserve the hearing of people who suffer from Usher Syndrome Type III (USH3).

The ability to hear effectively is highly reliant upon the inner ear sensory hair cells. If there are any gene mutations within these cells, then the cells will not function properly. This results in an individual experiencing hearing loss.

Gene mutation is responsible for the development of USH3.  The Clarin-1 gene provides the information for making a protein in the hair cells that help send sound signals to the brain. Researchers believe that utilizing gene therapy to replace this malfunctioning gene would be the key to helping patients who experience this form of hearing loss.

A study was conducted on mice subjects at Case Western Reserve University School of Medicine. Researchers were able to induce progressive hearing loss in these animals by introducing a disabling mutation in the Clarin-1 gene. This hearing loss was parallel to what humans with USH3 may experience.

Next, the researchers developed a method to postpone the hair cell degeneration in the mice for enough time to inject normal copies of the Clarin-1 gene into the ear. The hearing levels were measured after gene therapy to evaluate the success.

The results indicated that gene therapy not only blocked progressive hearing loss, but it also improved the hearing in the mice that were treated. Although human subjects have yet to be tested and evaluated, the finding in this study provides significant advancements in the future treatments of hereditary hearing loss.

Yes, the future in treating hereditary hearing loss may be in the hands of researchers who are further testing methods for gene therapy. However, if you are currently experiencing hearing problems, the best solution is to schedule an appointment with us for a complete hearing evaluation.

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