New national registry for inherited hearing loss

The HEDGE study (Hearing, Ear, Deafness and Genetics) is the first of its kind in the UK. It’s a patient registry created by experts at Great Ormond Street Hospital (GOSH), University College London Hospital (UCLH), and University College London (UCL), and managed by the National Institute of Health and Care Research UCLH Biomedical Research Centre.

Adults or children with a known genetic cause of hearing loss or a strong family history can sign up to take part.

The registry is currently recruiting families under the care of GOSH or UCLH. Anyone who thinks they may be eligible can email uclh.hedge@nhs.net to find out more.

When someone joins the registry, the team collects their hearing and health history, test results, and samples like blood or saliva for genetic testing.

Researchers will then look for links between symptoms (like hearing loss, tinnitus, or dizziness) and genetic information. If relevant clinical trials open, patients in the registry may be invited to take part – giving them access to cutting-edge research on new treatments and technologies, including hearing devices, drugs, or gene therapies.

Dr Emma Clement, consultant in clinical genetics and genomic medicine at GOSH, said: “It is great to be able to offer children, young people and families affected by hearing loss the opportunity to participate in HEDGE. We hope that the registry will allow us to better support research into genetic deafness and ultimately inform and improve patient care.”

Mr Nish Mehta, ear, nose and throat surgeon at UCLH, said: “Many types of inherited hearing loss still don’t have a clear genetic diagnosis. Even when we know which gene is involved, it’s often not clear how it causes hearing loss. Through this registry, we want to change that – and find better ways to treat people.”

Couple Sandra and Lukasz, and their daughter Phoebe, from Hertfordshire, have signed up to be part of HEDGE. Phoebe has her cochlear implant at GOSH.

Sandra and Lukasz said: “We’re incredibly grateful to be part of the HEDGE study. Taking part felt like a natural step for us – it’s our way of giving back after receiving such life-changing support throughout our daughter’s cochlear implant journey. We hope that by contributing to this important research, we can help break down barriers, raise awareness, and make the path clearer for other families.”