Researchers are working on a way to re-grow tiny hair cells in the ears of children with a rare syndrome that causes the loss of both hearing and sight. 

Dr Elaine Wong, Adjunct Senior Research Fellow at The University of Western Australia and Ear Science Institute Lead Scientist, is heading a team to help children with Usher Syndrome. 

“If they are not born deaf or with partial hearing then children with Usher Syndrome start losing their hearing and aspects of their vision from about ten years of age,” Dr Wong said. 

Dr Wong said the syndrome had a significant impact on their language and social development.

“The hearing loss experienced by these children is caused by the failure of the tiny hair cells in the cochlea and once they are lost, they don’t regrow,” she said.

“For the past five years Ear Science researchers have been working on a solution to regrow hair cells in the ear.”

Dr Wong’s work in treating Usher Syndrome-related deafness has recently been helped with a grant from the Channel 7 Telethon Trust.

“Our starting point in this ground-breaking work has been to take skin cells from people with Usher Syndrome and then, in the laboratory, change them into pluripotent stem cells which can develop in to many different types of cells or tissues in the body,” she said

“Other techniques then transform them into cells with the characteristics of those we find in the cochlea, called inner ear organoids.”

Dr Wong and her team’s research has led to an international patent and they are ready to proceed to research using organoids on a larger scale. 

“The aim is to finally be able to test a sample of drugs that have shown promise in treating Usher related deafness by arresting the loss of hair cells,” Dr Wong said.

“We want to also develop methods by which these drugs can be safely and effectively delivered to the cochlea.”