Organization: The Feinstein Institute for Medical Research in conjunction with the Apelian Cochlear Implant Center at Northwell Health-Zucker School of Medicine at Hofstra/Northwell.
A study of immunologic mechanisms in Sudden Sensorineural Hearing Loss and Autoimmune Inner Ear Disease in Children: Implications for Intervention
Project Rationale: Immune mediated hearing loss is comprised of Sudden sensorineural hearing loss (SSNHL) and Autoimmune Inner Ear Disease (AIED). Both of these diseases respond to corticosteroids in approximately 70% of adult patients, however no alternative treatment options have been identified for those that do not. These diseases have been observed and treated in children, but are even more underappreciated than their adult counterparts. There is no data available as to the incidence or corticosteroid responsiveness of either AIED or SSNHL in the pediatric population. Although our clinical observations suggest they have reduced corticosteroid-responsiveness, this has not been rigorously studied. The proposed project will both define the incidence in this population and characterize the immunologic mediators in the peripheral blood of these patients. Whole exome sequencing may elucidate novel genetic markers that regulate immune mediated hearing loss. By comparing the blood of the child to the unaffected parent, we should be able to identify novel genetic targets if immune mediated hearing loss has a genetic risk factor.