Evaluation of Sensorineural Hearing Loss
Our previous work has set the standard for what types of evaluations should be performed on children with hearing loss to determine the etiology. We are currently evaluating the effects of unilateral enlarged vestibular aqueduct and studying the natural history of unilateral sensorineural hearing loss. We have just completed studies defining the risks of progression and asymmetry in patients with and without GJB2-related hearing loss.
Advanced Molecular Diagnostic Testing for Hearing Loss
As part of a long standing interdisciplinary effort, we are developing the first resequencing microarray to evaluate for genetic causes of deafness in young children with hearing loss. These advances will allow us to more accurately predict the clinical course of the child, appropriate treatment and secondary medical diagnoses.
Family Studies for Hearing Loss
Our center enrolls families interested in studies to determine the genetic cause of their hearing loss. Besides the US, families from India, Bangladesh and Singapore have been studied here. These studies help to identify novel genes the gives scientists new in roads to the understanding of hearing.
Outcome Studies in Young Children with Hearing Loss
We are studying the affects of early treatment on the clinical course of young children with hearing loss. Early identification and treatment is thought to be critical to the success of children with hearing loss, but the initial language, education and socially-based strategies to utilize on these children is ill defined. Our research should point early intervention specialists toward more effective therapy options.
Mitochondrial Genetics in Hearing Loss
Mitochondrial mutations play a significant role in the development of hearing loss in children, both nonsyndromic and in association with the administration of aminoglcyoside antibiotics. We have studied the molecular mechanisms of mitochondrial mutation in cell, animal and human models.
Functional Magnetic Resonance Imaging (fMRI)
Our Center is one of the largest research imaging groups dedicated to childhood diseases. fMRI allows our researchers to examine the direct effect of sound and the lack of sound on the brain pathways and processing. These findings are leading our researchers to better predict which children would be best served with a cochlear implant to restore hearing.
Molecular Development of the Inner Ear
With at least half of all causes of congenital hearing loss due to genetic or hereditary factors, it is very relevant to study the genes involved in normal development of the inner ear as well as the consequences to the ear and hearing when critical genes are perturbed.
By identifying the key genetic processes involved in forming a normal inner ear, we begin to understand the possibilities of either correcting mal-development of the inner ear or potentially finding novel ways to correct defects of the ear and deafness.
Congenital Cytomegalovirus-related Inner Ear Disease
Of all the cases of pediatric sensorineural hearing loss in the United States, a significant portion of the cases are due to inner ear infections caused by CMV. However, the ability to detect this infection clinically and our understanding of how the virus causes inner ear damage and hearing loss is limited.
Research is ongoing now to screen several thousand newborns for this congenital infection and to determine the natural history of hearing loss in these children. Concurrent research is directed at examining the potential benefits of delivering antiviral drugs directly to the inner ear and thereby avoiding the major side effects of using those antivirals when administered systemically.