Translational Research

A common clinical scenario is that a child is initially identified with a partial hearing loss, which then progresses to profound hearing loss over a period of months to years. Genetic defects are responsible for over half of these cases, however the specific mechanisms of how many of these mutations cause progressive sensorineural hearing loss is unclear. Right now, all we can tell a patient with hearing loss is that we know they have hearing loss, and that it is because of a problem in the cochlea. There are no more detailed tests available.

Because of the difficulty in performing auditory research in humans, we often study normal and transgenic mice that have hearing loss. We strive to perform comprehensive evaluation of the pathophysiology that creates the hearing loss. For example, cochlear function is monitored with measurements of the compound action potential, the auditory evoked brainstem response, distortion product otoacoustic emissions, and the cochlear microphonic. Furthermore, we have developed novel optical technology, termed Volumetric Optical Coherence Tomography and Vibrometry (VOCTV), for in vivo imaging of the cochlea. This allows us to measure how the various tissues within the cochlea respond to sound, and we perform this imaging non-invasively, peering through the bone.

Clinical Research

Our clinical research is focused on improving what we are currently doing to help patients, particularly children, with hearing loss. Cochlear implants (CI) are the most common treatment for deafness. While many factors influence the ability of a deaf child who is hearing through a CI to develop speech and language skills, an important factor is to properly program the CI. However, implementing the optimal CI program is a challenging, individualized, and iterative process with variable success.

One difficulty in CI programming is obtaining behavioral measurements from the young children in which CIs are usually implanted. Therefore, we are developing the technique of near-infrared spectroscopy (NIRS) to functionally image activity within the auditory cortex of children hearing through a cochlear implant.

As well, we are finishing up a multi-site, prospective randomized clinical trial of deaf children with special needs. The goal of the study is to determine the best treatment options for children that require such complex and individualized care.

Clinical Interests

• Cholesteatoma
• Cochlear implantation
• Facial nerve diseases
• Hearing loss
• Neurotology
• Otology
• Otosclerosis
• Skull base surgery
• Vestibular schwannomas (acoustic neuromas)