Usher syndrome study shows promising results for treating hearing loss

People have hearing loss for a variety of different reasons. 

Some causes are more common than others, such as age-related hearing loss, which affects 50-60 per cent of people over 60 in Australia and New Zealand1. There's also noise-related hearing loss, thought to account for just over a third of all hearing loss cases in the country2

However, exciting progress has been made in treating a rare cause of hearing loss, Usher syndrome. 

What is Usher syndrome? 

Usher syndrome is a hereditary disease that affects vision as well as hearing. Usher syndrome is a hereditary disease that affects vision as well as hearing.

Researchers at Johns Hopkins Medicine looked to target two of the most debilitating symptoms of Usher Syndrome, a rare condition that affects approximately 3-10 people in 100,0003. Symptoms associated with the hereditary condition include balance problems and dizziness3

At present, there is no cure for Usher syndrome, but for those who experience the associated hearing loss, sign language and hearing aids remain two possible tools for communication. 

A breakthrough for treating hearing loss and dizziness

The study involved mice with symptoms that mimicked Usher syndrome, meaning that they had serious balance, vision and hearing issues from birth. By using a revolutionary inner ear gene treatment, the researchers managed to cure the mice's balance, as well as treating their hearing. 

"Inner ear gene therapy offers tremendous potential as a new way to help patients with hearing loss and dizziness," said Dr Wade Chien, lead researcher for the study.

While the research would have to be expanded with human subjects to establish its use in treating hereditary hearing loss, the results of the study are still incredibly heartening. 

"Our data indicate that gene therapy is likely to become a treatment option for hereditary disorders of balance and hearing," the authors write5

If you think you, or someone you love is struggling to hear in everyday situations, it might be time to see an audiologist. You can request a FREE* hearing check with Western Hearing Services by clicking here, or by giving our friendly team a call on 1800 940 981.

1The National Foundation for the Deaf, Age-related hearing loss. Accessed May, 2017.
2Department of Education and Training, Noise Induced Hearing Loss. Accessed May, 2017.
3National Organisation for Rare Disorders, Usher Syndrome. Accessed May, 2017.
4Johns Hopkins Medicine, Novel Gene Therapy Experiment Offers Hope For People with Certain Hearing Loss and Dizziness Disorder. Accessed May, 2017.
5Molecular Therapy, Gene Therapy Restores Balance and Auditory Functions in a Mouse Model of Usher Syndrome. Accessed May, 2017.