8: Novel Immunologic-Related Approaches to Treating Sensorineural Hearing Loss

Immune-mediated inner ear disease (IMED) represents several disease processes, all of which can be characterized by a new, asymmetric sensorineural hearing loss that may be ameliorated by timely immunosuppression. However, not all patients are responsive to corticosteroids. As a result, there is a clear clinical need to identify the mechanisms by which the hearing loss occurs, thereby identifying new potential therapeutic targets for intervention. Furthermore, within IMED, although sudden sensorineural hearing loss (SSNHL) and Meniere’s disease (MD) can be clearly characterized, autoimmune inner ear disease (AIED) is a complex entity that presents challenges for both diagnosis and management. While the pathogenesis of disease remains to be fully delineated, proposed pathological mechanisms include immunological perturbations in both the innate and adaptive immune response, vascular compromise, and disturbances in cochlear physiology. First-line treatment for all patients with suspected IMED includes high-dose oral corticosteroids and/or intratympanic steroid injections. The injection alone may provide benefit for those who cannot tolerate systemic treatment. For those who fail to respond or become resistant to steroids, biological agents such as anakinra may offer promising results. Finally, patients with IMED with profound hearing loss should be considered for early cochlear implantation for the best long-term results although their hearing may continue to fluctuate even after implantation.