Science Watch: Progress in search for Usher's cure

Following hot in the footsteps of one or two or more recent scientific breakthroughs in the search for cures for hearing problems, this is from BBC News who are reporting that scientists are now using a genetic sticking plaster on faulty DNA to stop hearing and sight loss in people who have Usher syndrome.

A tiny "genetic patch" can be used to prevent a form of deafness which runs in families, according to animal tests.
Patients with Usher syndrome have defective sections of their genetic code which cause problems with hearing, sight and balance.

Read more here

Scientists in America make medical progress to restore hearing

Scientists this week have reported amazing recent discoveries made at the Massachusetts Eye and Ear infirmary and Harvard Medical School in the search for a cure for deafness.  The scientists have been testing a drug on mice to see if it would repair the damage to the ear which causes hearing loss.  The mice were completely deaf and had relatively few hairs in their cochlear, i.e. those which detect sound.

The test results suggest new hair cells can be induced. The drug apparently works by converting cells operating in one function into cells that detect sounds. Those tiny hairs in the inner ear then send a signal to the brain.  The findings indicate partial recovery of hearing in ears damaged by noise trauma can be achieved. However they are not near the stage of generating these hairs in humans.

The drug is currently named LY411575.   The research was was published in the journal Neuron,

The full story is here and Action on Hearing Loss have provided a transcript of the video on that page here.

Breaking: A new test to help identify more genetic causes of deafness

Charity Action on Hearing Loss has revealed tonight that researchers have been developing a new test which can identify up to 34 causes of genetic hearing loss in children and its ready to be used in a trial.

The research, which was conducted by Doctors at the University of Antwerp, has led to a test that may help families learn about how the deafness could develop or if it will stay the same. Until now, the NHS were able to only screen for only one or two causes of deafness because of the costs and time involved. The new test, being piloted by Great Ormond Street Hospital next year, can provide much more information in the same amount of time and  for the same cost.

The test can even be expanded in future to give families an early indication of syndromes, like Usher syndrome, which can cause deaf-blindness and normally is only detected in young adulthood. For the moment though, the test is restricted to the most common genetic causes of hearing loss.

The full story is here: 

The report from the American Journal of Medical Genetics is here:

Your cochlear is a battery that could power your hearing aid

Research out from America reported in the Daily Mail shows that hearing aids could be powered by the bodies own power source, rather than hearing aid batteries.


Researchers at the Massachusetts Institute of Technology (MIT) have been working on a way to harness the energy that is generated by the cochlear in the process of natural hearing. They have proved in animal tests that tiny electrodes, inserted into the cochlear, can generate enough current to keep a miniature circuit board running and think that future developments could lead to tiny hearing aids or implants being powered by the body itself  possibly requiring no external parts.


Tests have been carried out on gerbils in the lab and proved successful but it remains to be seen if this technique would work with all types of hearing loss or along side cochlear implants. Check out the article here.

Hat tip to Fil

Physics words get sign language equivalents

STEM-DC (Science, Technology, Engineering and Mathematics Disability Committee) have been working on a project to ensure that hearing loses don’t deter students from learning science in lessons. As a reult  116 new Physics and Engineering terms and definitions have been developed in British Sign Language. New signs helping to communicate the topics of movement, the Universe, light and sight, and energy and radiation have been developed to give secondary school science signing students a better opportunity to engage with Physics and Engineering.

Phrases like light year and x-ray now have their own sign when previously finger-spelling and lip-reading were the most common methods for teachers assistants to communicate the meaning of their lessons. The signs use common BSL techniques to help students understand the concepts behind the phrases. BSL metaphors are used to produce signs for ‘mass’ and ‘weight’; the researchers use a closed fist to communicate ‘mass’ while ‘weight’ is communicated by a fist being pulled down (the effect of gravity on mass being metaphorically implied).

Using the five features of British Sign Language’s phonology (handshape, orientation, location, movement and facial expression) the new signs have been developed. The project has also developed, in BSL, definitions for each term. These definitions explain the meaning of the term or provide an example of how it can be used.

The research team from The Scottish Sensory Centre, launched the new signs during the event at the Royal Academy of Engineering, 19 June.

'Physics in BSL' Event in London - Tuesday 19th June 2012

Thank you to David Kettle from the British Deaf Astronomy Association for passing on these details.

Students, teachers, BSL/English interpreters, communication support workers and others involved in making the science curriculum accessible to students who sign are invited to attend a free dissemination event for the project on 19th June 2012 at The Royal Academy of Engineering in Central London .

The team of Deaf scientists and sign linguists from the Scottish Sensory Centre (SSC) who developed the BSL science sign glossary www.sciencesigns.ac.uk targeted at KS3 and KS4 science (Biology, Chemistry and Physics) have been working on the production of new BSL signs for Physics and Engineering-related terms.

Audrey Cameron, Gary Quinn and Rachel O'Neill will be presenting the findings from the project which is funded by the STEM Disability Committee.

The afternoon session featuring an interactive science show based around experiments that show the signs in action shows very engaging.

To reserve a place email diversity@raeng.org.uk or call them on 0207 766 0600.

Sign_Language Science London Event

Science Musuem Deaf-led Events

For anyone not yet aware, on the first Saturday of every month, the Science Musuem have deaf-led events with voice-over interpreters, suitable for deaf and hearing family audiences.

Bubbles, bubbles, bubbles with BSL takes place Saturday 7th April. Further details here

Space in your face (with BSL) takes place on Friday 13th April. Further details here

Are You confused in your Science lessons?

Have you started back to school? Are you worried about upcoming science exams? Are you learning about forces, renewable energy or volcanoes? Are you still confused? Check out these really useful episodes of 'The Secret Lab' at http://www.bslbt.co.uk/programmes/the-secret-lab/