Updates on the Management and Classification of Inner Ear Schwannomas

PROGRAM DESCRIPTION: The detection rate of inner ear schwannomas has significantly increased in the post-MRI era, now exceeding 4 per 100,000 person-years among age groups at highest risk – incidence rates that exceed the historical rates attributed to vestibular schwannoma. The growing prevalence of inner ear schwannomas underscores multiple ongoing controversial aspects of patient care, from optimal tumor management to rehabilitation with cochlear implantation and its attendant implications on tumor surveillance with MRI. Complicating these issues, multiple different classifications of inner ear schwannomas have been used since the original tumor description in 1917. Often referred to as, “intralabyrinthine schwannomas,” prior classification systems have been cumbersome and potentially misleading (e.g., the term “labyrinth” or “labyrinthine” is sometimes used to refer to the vestibular component of the inner ear only, such as in labyrinthectomy or the translabyrinthine approach).

In Spring 2023, an international working group met in Bergen, Norway to update and simplify the classification of inner ear schwannomas. Following that working group, an international cohort study encompassing 11 institutions across three countries collaborated to study outcomes of ipsilateral cochlear implantation in over 100 patients with sporadic inner ear schwannoma. The current panel submission will cover the rationale and development of an updated nomenclature and classification system for inner ear schwannomas. Within this context, this panel will cover recent advances surrounding tumor management, imaging surveillance, and clinical outcomes associated with rehabilitation with cochlear implantation.