Investigating The Role Of SERPINB6 In Cochlear Function And Deafness
Funder
National Health and Medical Research Council
Funding Amount
$570,803.00
Summary
In 2010 a novel genetic mutation was identified that causes progressive hearing loss in humans, however, it was not established why this mutation leads to the disease. We propose that the mutation renders cells of the inner ear more susceptible to death caused by noise trauma. We will investigate this in a mouse model of the human condition. This could lead to the development of therapies that prevent hearing loss.
Protecting Hearing During Surgery -an Experimental And Clinical Study
Funder
National Health and Medical Research Council
Funding Amount
$723,048.00
Summary
Both hearing and balance are vulnerable during ear surgery, and the loss of either carries a heavy personal, social and economic cost. We have developed new ways of protecting the ear during surgery, though either the local delivery of medication to the inner ear or by injection. The clinical protocol will be developed in the laboratory and tested in a clinical trial. This research will stand to benefit all people undergoing major operation on their ear, and especially cochlear implant candidate ....Both hearing and balance are vulnerable during ear surgery, and the loss of either carries a heavy personal, social and economic cost. We have developed new ways of protecting the ear during surgery, though either the local delivery of medication to the inner ear or by injection. The clinical protocol will be developed in the laboratory and tested in a clinical trial. This research will stand to benefit all people undergoing major operation on their ear, and especially cochlear implant candidates with residual hearing.Read moreRead less
The research will investigate the mechanisms by which our brains are able to listen selectively to sounds of interest in competing background noise. This will be investigated in normal hearing subjects, those with partial deafness and in profoundly deaf patients who use a cochlear implant. If deaf patients can learn to use cues to enhance detection of sounds of interest this could have an impact on the effectiveness of hearing aids and cochlear implants in noisy listening situations
Delayed Loss Of Acoustic Hearing After Cochlear Implantation: Mechanisms And Clinical Identification With New Cochlear Implant Technology
Funder
National Health and Medical Research Council
Funding Amount
$1,040,157.00
Summary
Natural hearing can be combined with a cochlear implant to improve sound quality and speech understanding. However, natural hearing frequently deteriorates weeks to months after cochlear implantation. This seems to occur when cochlear fluid buildup (hydrops) or fibrosis within the inner ear disrupts cochlear mechanics. We plan to monitor these processes in patients by recording directly from the cochlear implant, and conduct laboratory experiments to find therapies to alleviate their effects.
Nanoengineered Drug Delivery To The Inner Ear To Prevent Progressive Hearing Loss
Funder
National Health and Medical Research Council
Funding Amount
$479,056.00
Summary
Hearing loss is a common sensory deficit and can get progressively worse over time, eventually requiring a cochlear implant. In this project we will examine the effectiveness of a new technique that uses nanoengineered particles to provide long term and controlled delivery of drugs in order to prevent progressive hearing loss and protect residual hearing following cochlear implantation. We will develop the next generation of nanotechnology to enhance drug delivery in pathological ears.
Bilateral Cochlear Implants: Restoring Binaural Processing By Experience And Training With Binaural Cues
Funder
National Health and Medical Research Council
Funding Amount
$968,030.00
Summary
Cochlear implantation in both ears is increasingly common and while there are benefits, performance falls short of expectations, likely due to the degradation of the long-term deaf brain’s sensitivity to small timing differences of sounds reaching each of the two ears. By confirming the hypothesis that experience with high-fidelity timing information will improve performance, this study will drive the technical innovations required to maximise the benefits and investment of bilateral implants.
Differentiation, Electrical Activity And Synapse Structure Of Human Stem Cell-derived Sensory Neurons
Funder
National Health and Medical Research Council
Funding Amount
$322,088.00
Summary
A major challenge in treating neurodegenerative conditions with stem cells, is to generate a population of donor cells which are capable of incorporating into the host nervous system and restoring function. This proposal will test the capacity of human stem cells to make functional connections in the auditory system, potentially enhancing hearing with a cochlear implant. These studies are critical in determining how stem cells may be used to promote functional regeneration of the damaged nervous ....A major challenge in treating neurodegenerative conditions with stem cells, is to generate a population of donor cells which are capable of incorporating into the host nervous system and restoring function. This proposal will test the capacity of human stem cells to make functional connections in the auditory system, potentially enhancing hearing with a cochlear implant. These studies are critical in determining how stem cells may be used to promote functional regeneration of the damaged nervous system.Read moreRead less
Gene Therapy For Preventing Progressive Sensorineural Hearing Loss And Restoring Hearing
Funder
National Health and Medical Research Council
Funding Amount
$549,848.00
Summary
This proposal aims to study the clinical feasibility and safety of gene therapy in the guinea pig cochlea for preventing progressive hearing loss and restoring hearing. Two cell survival genes (GDNF and BDNF) will be studied for preserving sensory (hair) cells and hearing nerves, while a gene called Atoh1 will be investigated for its ability to regenerate new hair cells and restore hearing after the onset of progressive hearing loss.
Effects Of Increased Endolymph Volume On Cochlear And Vestibular Function And Morphology Of Inner Ear Tissues
Funder
National Health and Medical Research Council
Funding Amount
$313,391.00
Summary
Our recent research suggests that the vertigo attacks associated with enlarged fluid volumes in the inner ear, in diseases such as Ménière's Disease, maybe triggered when the fluid pressure forces open tissue valves located in the fluid-ducts connecting the hearing and balance organs. This project aims to identify the functional role of these valves, and to develop an animal model of abrupt changes in vestibular activity due to hydrops, to allow future treatments to be developed.
Characterisation Of Eurl, A Novel Gene Implicated In The Etiology Of Abnormal Brain Development And Intellectual Disability
Funder
National Health and Medical Research Council
Funding Amount
$597,541.00
Summary
Intellectual disability affects around one per cent of Australians, and can arise from genetic abnormalities during fetal life, such as through abnormal regulation of gene expression. We have identified a novel gene, known as eurl, which controls brain assembly as well as the ability of neurons to form functional connections within the brain. We will investigate how this novel gene controls brain development, and characterise eurl as a potential therapeutic target for learning and memory.