Timothy C. Hain, MD. Page last modified: February 23, 2012
CNS sarcoidosis is a rare disorder in which noncaseating granulomas affect the brain. Only about 5% of patients with sarcoidosis have CNS lesions, but because the brain is a critical organ, CNS sarcoidosis causes a disproportionate amount of disability.
The inner ear is seldom involved by sarcoidosis (about 50 cases had been described through 1990) According to Moine and Frachet (1990), deafness is fluctuant in 50% of all cases, bilateral, and most often associated with facial palsy and uveitis, the vestibular reflexes being reduced.
The cause of sarcoidosis is unknown, but it is generally treated with agents that reduce inflammation. Damage to the inner ear is thought to initially begin as a neuropathy, and then progress due to vascular damage (Babin and Liu, 1984; Moine and Frachet, 1990)
Diagnosis is made through a combination of imaging studies showing enhancing lesions, sometimes involving the meninges, combined with other lesions in the body. A chest-Xray is the single most useful study. ACE levels may be elevated in the serum and IgG synthesis increased in the spinal fluid.
Facial nerve palsy (e.g. Bell's palsy) is the most common manifestation. (Chen, R. C. and J. G. McLeod ; 1989;May M., Fria T, et al. ; 1981)). Uveitis (inflammation of the iris) is possible. (Hybels, R. L. and D. H. Rice; 1976)
Vestibular involvement is rare but possible (e.g. von Brevern, M., T. Lempert, et al. (1997).
The author of this page has seen patients with pressure sensitivity accompanying neurosarcoidosis.
Treament is difficult as in spite of corticosteroids and oral immunosuppresants, the disorder often progresses. Like many other CNS inflammatory disorders, it is thought that tumor necrosis factor -- TNF alpha, might play an important role.
TNF inhibitors including Infliximab have recentlhy been used with success (Morovan and Segal, 2009).
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