July 2019 The West Australian

A team at the Ear Science Institute have taken a Nobel- winning concept — where a stem cell can be generated from our own adult cells rather than embryos — and are working towards programming them to become hair cells. Hair cells are the hearing cells of the inner ear, in the cochlea, which cannot be regenerated and are lost as we age. The cells’ death causes age-related hearing loss, experienced by one in three over the age of 65 and half of those over 75. “We are developing new stem cell methods around how we might replace or regenerate the hair cells in a patient using their own skin cells as a stem cell donor,” Dr Rod Dilley says.

Dr Rod DilleyDr Rod Dilley heads up a team at the Ear Science Institute working at the forefront of a new area of science around pluripotent cells.

Working with developmental biologist Dr Elaine Wong and researchers at Lions Eye Institute, the project is focusing on patients with Usher syndrome — a condition characterised by partial or total hearing loss and vision loss that worsen over time. Regular skin cells are being collected from the patients and grown in the lab. With the pluripotent technique the scientists can reprogram those skin cells into stem cells — effectively taking them from adult cells back to embryonic- like cells. The team have successfully taken cells from Usher syndrome patients and reprogrammed them to the pluripotent state. Now in the lab they are making good progress towards making them into new hair cells. “We have to give them the instructions to go from their general embryonic stem cells state, where they can just proliferate and make more of themselves ... to go down those stages of development to become a hair cell,” Dr Dilley says. “Once we have hair cells in the dish we can start looking at if these patients’ hair cells are normal or not, looking at genotyping or seeing how their disease developed. We are also looking to make treatments to correct the function of these patient’s cells. Well down the track we can think about how to get the hair cells back in the patient’s cochlea and regenerate their hearing and vision function. That’s the long term goal.” The even bigger vision goes beyond Usher syndrome. “Some of these new models we have developed are really exciting prospects in terms of treating age-related hearing loss or noise-induced hearing loss in the future,” Dr Dilley says. “We are aiming for the disease of Usher syndrome where people are very powerfully impacted and then we can look at applying our experimental work towards these much bigger population problems like age-related hearing loss, as well as a range of other genetic disorders that lead to hearing loss.”

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