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Restoring hearing in patients through novel technologies and stem-cell based therapies

Hear Again: restoring function and fidelity of the ear

The Royal National Throat Nose and Ear Hospital

With over 130 years of history as a specialist hospital the Royal National Throat, Nose and Ear Hospital (RNTNEH) is the UK’s largest ear, nose and throat hospital and the UK and Europe’s centre for audiological medicine and research. It is recognised internationally as a centre of excellence, unique in the breadth of knowledge and specialities represented on one site on Gray’s Inn Road, central London

The UCL Ear Institute

The Ear Institute is the largest and most broad-based academic unit for research into hearing and deafness in the UK. In addition to the expertise of our internationally recognised researchers, our teaching programmes also draw on the knowledge of dedicated lecturers in Audiology.

The UCL Ear Institute website

Gene Therapy for Hearing Loss

Scanning electron micrographs of normal and abnormal bundles of hairs on mammalian cochlear hair cells. mammalian cochlea. The image on the left shows the normal size and shape in wild type hair cells, whereas the image on the right shows cells that are missing the plastin1 gene. This gene is a new candidate gene for different genetic forms of progressive hearing loss and a potential therapeutic target.

Can we regenerate Hair Cells?

Can we grow a replacement ear in a dish. Here we see epithelial cells that have been maintained in vitro and can be used as a model system for how the inner ear responds to damage. The cells are labelled with markers for the cytoskeleton. Source: Jonathan Gale

Understanding how the brain processes sound

Mice with ectopias have an unusual auditory temporal processing deficit, this “offset-deafness” could underlie poorly understood hearing difficulties in human patients with developmental disorders.

SIGNS OF STRESS IN THE INNER EAR

A 3D reconstruction from a scanning laser confocal image stack showing the organ of Corti, the sensory epithelium of the cochlea. The green particles are “stress granules” that are present in the sensory hair cells, the surfaces of which are outlined by staining with phalloidin (red). The stress granules are composed of RNA-binding proteins, RNA-translation machinery and RNA, and they have been implicated in neurodegenerative diseases. We are investigating stress granules in the inner ear as a potential target for protecting sensory cells during ageing.