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 simila
|Contact: Andrew Gould|
The Peninsula College of Medicine and Dentistry