Wednesday, March 2, 2011

Kevin Frank, Ph.D.

"Testing the perceptual limits of acoustic and electric hearing in children using cochlear implants"

The scientific way of doing this project is to use psychophysics. Unfortunately, psychophysics are boring and because of this, typically only adults can be tested. We are developing a video game to attempt to do this testing in children. We hope to correlate other types of measures from children with cochlear implants with what we learn from this game. If we can, we might be able to imagine ways of improving on the design of the cochlear implants.

Claus-Peter Richter, MD., Ph.D.

Claus-Peter Richter, MD., Ph.D.

"The current distribution in the cochlea and coding of acoustic information"

(1) The current distribution in the cochlea:
Although cochlear implants are able to restore some auditory sensation, they are far from providing normal hearing. This project will provide for the first time fundamental data on the current path in the cochleae and will quantify changes of the current path that occur after neural degeneration.

(2) Coding of acoustic information:
For a cochlear implant user the speech signal has to be processed and encoded in electrical signals to be sent to the implanted electrode contact. This project will test a possible novel coding strategy that is based on a stochastic model (Hidden Markov Model, HMM) developed by the PI to describe the activity of a single auditory nerve fiber.

Tuesday, March 1, 2011

Zheng-Yi Chen, D. Phil. / Albert Edge, Ph.D.

"Identification and analysis of inner ear stem cell genes"

The major research focus of our laboratories is on hair cell regeneration. Mammalian inner ear hair cells do not regenerate. As a consequence of damage to hair cells, and lack of available treatment, deafness and balance disorders are permanent in general. In lower vertebrate such as chick and fish, however, inner ear hair cells are regenerated by cell cycle re-entry of supporting cells/progenitor cells, and by transdifferentiation from supporting cells to hair cells. One approach to regenerating mammalian hair cells would be to activate endogenous inner ear stem cells by manipulation of genes involved in cell cycle or fate determination. However, no inner ear stem cells (IESC) genes have been identified, severely limiting the use of IESC as a source for hair cell regeneration. In these studies we will identify genes that are critical for IESC self-renewal and differentiation to hair cells.









Project Name: "Identification and analysis of inner ear stem cell genes

Victor Pikov, Ph.D.

"Tinnitus - related pathophysiology in the cochlear nucleus"

Tinnitus, a manifestation of phantom auditory sensations, is a prevalent problem affecting 14% of the general population, with 3% severely affected by this intrusive condition. Despite high prevalence of tinnitus and significant associated financial and psychological costs, no effective treatment is currently available and its mechanisms remain largely unknown.







Project Name: "Tinnitus - related pathophysiology in the cochlear nucleus"

Paul A. Webster, Ph.D.

"Identifying and Treating Biofilm Infections Associated with Otitis Media"

Paul Webster - Even with the availability of a variety of antibiotics, otitis media remains one of the major causes of morbidity and the most common cause of hearing loss in children.

The House Ear Institute seeks to understand the causes of persistent otitis media infections in infants and children and to find ways of improving diagnosis and treatment.

Such persistent infections may well be the result of bacterial biofilms forming in the middle ear. Biofilms have been broadly defined as multicellular assemblages of microorganisms and extracellular products attached to an abiotic or biotic surface. The extracellular products form a matrix, typically rich in sugar moieties, that confers protection from host immune responses, phagocytosis and antibiotic treatment.

Project Name: "Inhibition of apoptosis as a means to mitigate hearing loss in mice"

Lisa V. Goodrich, Ph.D.

"Rewiring the Cochlea: A Genetic Screen for Regulators of Auditory Circuit Assembly"

As the sole conduit for sound information from the inner ear to the central auditory system, spiral ganglion neurons are important therapeutic targets for the treatment of deafness.  Indeed, the promise of hair cell regeneration is limited by whether new hair cells can be properly innervated by spiral ganglion neurons.  Thus, efforts to stimulate hair cell regeneration must be coordinated with efforts to induce neurite outgrowth in surviving neurons or to replace lost neurons with stem cells. The long term goal of the work in my laboratory is to dissect the genetic basis of auditory circuit assembly, focusing on the molecules that drive auditory-specific programs of differentiation in spiral ganglion neurons.  Towards this end, we have developed genetic tools that allow us to visualize the pattern of innervation in the cochlea at any stage of development (see figure).  We have also created a catalog of genes that are uniquely expressed in spiral ganglion neurons but not in the closely related vestibular ganglion neurons, which mediate the sense of balance.  Currently, we are elucidating functions for these auditory-specific genes, both by analyzing available mutant mice and by developing a new technique for performing a forward genetic screen.  Together, these studies will deepen our understanding of the normal developmental program for spiral ganglion neurons, information that is critical for future efforts to engineer naïve stem cells to re-wire the cochlea as a treatment for deafness.

Project Name:"Rewiring the Cochlea: A Genetic Screen for Regulators of Auditory Circuit Assembly"

Joseph Donaher Ph.D.

The SANDBOX is a virtual reality, computer based program which will allow educators, parents and other interested parties to enter a realistic school-based setting while having a disability like stuttering or a hearing loss simulated electronically. They will then be asked to maneuver through a set of activities similar to those routinely faced by children with disabilities. In this way, the families and professionals may gain a heightened sense of the obstacles faced daily by the child and the inherent difficulty of incorporating rehabilitation strategies into everyday experiences.

This tool will also allow clinicians and children with disabilities to practice strategies or techniques in a safe virtual environment prior to trying them in the classroom. The SANDBOX program will also allow researchers to explore situations commonly experienced by children with developmental disabilities. Thus, the Sandbox program is a powerful tool to increase carryover of new skills, to advance our knowledge base on developmental disabilities and to educate families and professionals.