Research Projects

Cochlear implants (CI) are successful in most sensorineural hearing loss (sNHL) patients, but there is still variability in speech and language outcomes due to differences in neuronal relay at central auditory centers. Genetic mutations in peripheral auditory pathway genes have been linked to CI performance variation, but no strong genetic basis is available to predict cochlear outcomes. The hypothesis is that a possible co-relation of central auditory pathway genes/variants (CAPGs) with auditory comprehension outcomes in cochlear implantees is possible. However, CAPGs have not been extensively explored in the context of establishing a genetic basis of CI outcomes. The proposed study aims to develop a novel panel of CAPGs based on mechanistic studies aimed at establishing potential novel cochlear-brainstem organoids (CBOs) from patients with congenital sNHL disease. Hereditary congenital sNHL patients with poor clinical cochlear implant outcomes will be tested for genetic mutations by whole exome sequencing. Novel patient CBOs will be established and compared against unaffected healthy controls/carrier parents for constructing deregulated peripheral-central auditory pathway networks (PCAPNs) following transcriptomics. A potential panel of altered genes related to ectodermal patterning lineage intermediates of PCAPNs will be established and labeled as Ectodermal Patterning Panel (EPP) to establish the genetic basis of cochlear implant outcomes.