Action on Hearing Loss Logo
    Total results:
    Search
      Total results:

      A new breakthrough towards preventing hearing loss in Usher syndrome

      Researchers in the US have made a breakthrough towards developing a treatment for Usher syndrome using gene therapy. Carina Santos, from our Biomedical Research team, tells us more.

      By: Carina Santos | 11 December 2017

      Usher syndrome type III (USH3) is an inherited condition that affects both vision and hearing. People with this syndrome are born hearing but during their lifetime, their hearing progressively deteriorates so that by middle age, they are profoundly deaf. USH3 is caused by a mutation in a gene called clarin-1. This gene carries the information to produce a protein that maintains the structural integrity of the hair cells in the ear, which are responsible for turning sound information into electrical signals that the brain can understand, and which are crucial for hearing.

      People with USH3 are born hearing and only start to lose their hearing later in life (usually during their teenage years). This means there is a period of time, called a therapeutic window, where it is possible to treat the condition, and prevent the hearing loss before it starts. Researchers are developing treatments for USH3 that could be used in this therapeutic window. To test these treatments, they need animal models of the human condition, animals that develop progressive hearing loss like people with USH3. A team from Case Western Reserve University School of Medicine in Ohio, USA, led by Professor Kumar Alagramam, generated a model of USH3 by mutating the clarin-1 gene in mice. Like people with USH3, these mice developed a delayed progressive hearing loss. This is the first animal model of USH3 – this work was published in October in the journal “Scientific Reports”. You can read the original article here

      Professor Alagramam’s team genetically modified the mice so that clarin-1 protein was produced as normal in the hair cells during embryonic development, but then levels of the protein decreased after birth. This meant that the mice were born with normal hair cells and no hearing loss, but then developed a delayed hearing loss, that could be detected at 1 month of age (mice this age are equivalent to human teenagers). Using this new USH3 animal model, the researchers tested a gene therapy approach, in which a healthy copy of the clarin-1 gene was delivered into the hair cells, to try to restore the levels of clarin-1 protein in the cells. The results were very exciting, as they showed that treating mice with the gene therapy before they started to lose their hearing prevented the hearing loss. The team also showed that the treatment had to be administered before the hair cells started to degenerate, otherwise it was not possible to prevent the hearing loss in the mice.

      This work has important implications for the development of treatments for USH3-associated hearing loss. Firstly, this new animal model of USH3 will allow other researchers to test how effective the treatments they are developing for USH3 are in preventing hearing loss. Secondly, this work has demonstrated that gene therapy may be a way to prevent hearing loss in people with USH3. Finally, if this gene therapy approach is approved for treating people in the future, it could be combined with genetic screening of newborn children. Genetic screening would allow clinicians to identify those children with clarin-1 mutations. This means they could then be given gene therapy to correct the mutation and prevent them from losing their hearing later in their life.

       

      Recent Posts

      Victoria runs Marathon for mother who lost hearing in operation

      Victoria Briand is taking on the London Marathon this Sunday to raise funds for Action on Hearing Loss. The mum-of-three is taking on the big challenge after her mother was diagnosed with a brain tumour and lost her hearing following lifesaving surgery. Since, their entire family’s lives have changed forever. Read Victoria’s touching story in her own words.

      By: Victoria Briand
      19 April 2018

      Jackie Edwards, 60, runs Marathon

      Being a busy grandmother-of-eight hasn’t stopped Jackie Edwards, 60, from taking on 26.2 miles that is the London Marathon this weekend. Hailing from Evesham in Worcestershire, Jackie has been deaf since the age of four and now wears two hearing aids. By running the marathon with her daughter, Bev, Jackie hopes to raise vital funds for Action on Hearing Loss, a charity that is close to her heart.

      By: Jackie Edwards
      18 April 2018

      The latest in hearing aid evolution

      Hearing aids have been around since the 18th century. From the earliest ear horns, they have evolved into what we see today – high tech digital hearing aids. These devices can carry out thousands of digital processes to try and ensure the sound quality is of the highest standard for their users. However, they are still far from perfect, so what comes next? Jesal Vishnuram, our Technology Research Manager, explains.

      By: Jesal Vishnuram
      16 April 2018

      Getting medicines into the inner ear and improving hearing tests – new grants for hearing research

      We’ve awarded new grants through our Flexi Grant scheme, which provides small grants to researchers around the world to support a variety of activities that benefit hearing research as well as people with hearing loss or tinnitus. Tracey Pollard, from our Biomedical Research team, tells us more.

      By: Tracey Pollard
      12 April 2018

      Recent Posts

      Victoria runs Marathon for mother who lost hearing in operation

      Victoria Briand is taking on the London Marathon this Sunday to raise funds for Action on Hearing Loss. The mum-of-three is taking on the big challenge after her mother was diagnosed with a brain tumour and lost her hearing following lifesaving surgery. Since, their entire family’s lives have changed forever. Read Victoria’s touching story in her own words.

      By: Victoria Briand
      19 April 2018

      Jackie Edwards, 60, runs Marathon

      Being a busy grandmother-of-eight hasn’t stopped Jackie Edwards, 60, from taking on 26.2 miles that is the London Marathon this weekend. Hailing from Evesham in Worcestershire, Jackie has been deaf since the age of four and now wears two hearing aids. By running the marathon with her daughter, Bev, Jackie hopes to raise vital funds for Action on Hearing Loss, a charity that is close to her heart.

      By: Jackie Edwards
      18 April 2018

      The latest in hearing aid evolution

      Hearing aids have been around since the 18th century. From the earliest ear horns, they have evolved into what we see today – high tech digital hearing aids. These devices can carry out thousands of digital processes to try and ensure the sound quality is of the highest standard for their users. However, they are still far from perfect, so what comes next? Jesal Vishnuram, our Technology Research Manager, explains.

      By: Jesal Vishnuram
      16 April 2018

      Getting medicines into the inner ear and improving hearing tests – new grants for hearing research

      We’ve awarded new grants through our Flexi Grant scheme, which provides small grants to researchers around the world to support a variety of activities that benefit hearing research as well as people with hearing loss or tinnitus. Tracey Pollard, from our Biomedical Research team, tells us more.

      By: Tracey Pollard
      12 April 2018

      More like this

      Are you looking for reliable information on all aspects of deafness, hearing loss and tinnitus? Read our range of factsheets and leaflets on a range of topics.

      Challenge yourself in 2018