Researchers at the Peninsula Medical School have received a grant of over £39,000 from the charity Deafness Research UK, to investigate the role of brain tumours causing deafness in children and adults and the development of therapies using in vitro models.

These tumours and a variety of other tumours are caused by mutations affecting a protein called merlin, which in turn cause cancers in a range of cell types including Schwann cells in the nervous system. Schwann cells produce the sheaths that surround and insulate neurons.

Although the tumours are benign, they are frequent. They can be inherited and come in numbers. The sheer number of them can overwhelm a patient, often leading to deafness and eventually to death. Patients can suffer from 20 to 30 tumours at any one time, and the condition typically affects older children and young adults.

No therapy, other than invasive surgery aiming at a single tumour and which may not eradicate the full extent of the tumours, exists. The hereditary condition affects one in every 2,500 people worldwide. It can affect any family, regardless of past history, through gene mutation and currently there is no cure.

In some cases, pressure from the tumours affect the process of hearing leading to hearing loss or total deafness. The research will use a human acoustic neuroma in vitro model to identify how and why the tumours grow and eventually lead to hearing damage, and to test drug-based therapies designed to alleviate the problem.

The research is led by Professor Oliver Hanemann, Chair of Clinical Neurobiology at the Peninsula Medical School. He said: “We have already achieved some success in re-profiling an existing drug called sorafenib by using the human in-cell model. The re-profiled drug has a positive affect on multiple brain tumours. As a consequence, we can go straight to clinical trials and introduce therapies to patients sooner rather than later using sorafenib or similar drugs..”

Vivienne Michael, Chief Executive of Deafness Research UK, said: “This is clearly an important research project that could significantly improve the treatment available for those with acoustic neuroma and the charity is delighted to be funding it. Acoustic neuroma is worrying enough in its own right; when multiple tumours occur they need treating and traditional cancer therapies are not an option. Currently, treatment involves surgical removal of the tumours but this leads to damage of the auditory nerve and can cause total deafness with potentially devastating effects on the lives of those involved.

“Great progress has been made in identifying target molecules for treatment, and this research will go a long way towards establishing the best potential drug for non-invasive therapy. Ultimately this would avoid the need for surgery and mean that people who currently face deafness as a result of acoustic neuroma would retain their hearing.”

Deafness Research UK is the only organisation totally dedicated to medical research of this type - researching treatments and cures for deafness, tinnitus, and other deafness-related conditions.

Source:
Andrew Gould

The Peninsula College of Medicine and Dentistry

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