Cochlear Implantation of a Patient with Definitive Neurosarcoidosis

N oncaseating epithelioid granulomas are found in the central nervous system in 5% to 26% of patients diagnosed with systemic sarcoidosis. The most common presentation of central nervous system sarcoidosis (neurosarcoidosis) is cranial neuropathy, followed by meningeal disease, including aseptic meningitis and mass lesions. Neurosarcoidosis has a predilection for basal meninges that surround the cranial nerves with infiltrative, perivascular granulomas. In the case of cranial nerve involvement, eighth nerve symptoms occur in up to 20% of patients and are associated with other cranial nerve neuropathies or overt systemic disease. The incidence of sensorineural hearing loss (SNHL) is only 5% to 9% among those diagnosed with neurosarcoidosis. The likely mechanism of injury is vasculitis that leads to transient ischemia and neural damage. Approximately 70% of patients will recover at least some hearing, either spontaneously or with corticosteroid therapy. Progression to profound hearing loss is exceedingly rare. Consequently, little is known about cochlear implantation as a rehabilitation option in this group of patients.