Annals of Otology, Rhinology & Laryngology 119(9):641-645. © 2010 Annals Publishing Company. All rights reserved. Neurosarcoidosis Affecting the Vagus Nerve Eran E. Alon, MD; Dale C. Ekbom, MD Objectives: Our objective was to review patients who presented to our medical center with a diagnosis of neurosarcol- dosis affecting the vagus nerve and to present symptoms, progression, treatments, and outcome. Metbods: We performed a chart review of patients who presented to our medical center in the past 10 years with a diag- nosis of neurosarcoidosis specifically affecting cranial nerve X. Results: A chart review of 53 patients revealed only 4 with findings suggestive of vagal neurosarcoidosis. All were male and had a mean age of 50 years (range, 42 to 57 years) at presentation of symptoms. 1\vo of the 4 patients presented ini- tially with cough, 1had recurrent syncope, and another presented with left facial pain. Vagus nerve involvement included vocal fold paresis or paralysis in all 4 patients, 2 of whom reported coughing with exposure to various odors and 2 of whom were found to have a unilateral. palatal weakness. All but 1 had positive findings on magnetic resonance imaging of the head. . Conclusions: Neurosarcoidosis involving the vagus nerve is a rare finding, but should be considered in the differential diagnosis of vocal fold paresis or paralysis. Key Words: neurosarcoidosis, sarcoidosis, vagus nerve. Sarcoidosis, a multisystem granulomatous dis- ten appearing in the first 2 years of the illness. Most ease, first described as a cutaneous disease, is now patients will have other organ involvement, which known to affect many organs of the body. Jonathan aids in making the diagnosis.' Sarcoidosis can af- Hutchinson was the first to describe the cutaneous feet any part of the nervous system. The neurolog- manifestations of sarcoidosis, at that time mistak- ic manifestations are divided into those with acute ing the disease for gout. The term sarcoidosis was symptoms and those with a more chronic onset. The coined from histologic features resembling sarco- acute setting usually presents with isolated crani- rna. Sarcoidosis most commonly affects young to al neuropathies or aseptic meningitis, whereas the middle-aged adults. The highest rates of sarcoidosis chronic picture is more diffuse in nature, involv- are observed in Swedes, Danes, and African-Amer- ing the brain parenchyma, hydrocephalus, multiple icans, with a prevalence as high as 50 per 100,000 cranial neuropathies, or peripheral nervous system reported in Scandinavian countries and among Afri- manifestations. The pathophysiology suggests an can-Americans in the United States.' intense inflammatory reaction, most often affecting Neurologic involvement in sarcoidosis is uncom- the meninges at the skull base. The initial symptoms mon, affecting 5% to 16% of patients clinically. Oc- may respond well to treatment, but a more chronic ular and cardiac involvement have an increased inci- picture suggests fibrosis with irreversible damage.' dence with neurosarcoidosis as compared to disease Cranial neuropathy remains the most common not involving the nervous system. The diagnosis of manifestation of neurosarcoidosis. Involvement of sarcoidosis is usually made with associated clinical, the 7th cranial nerve is the single most common radiologic, and histopathologic findings and absence neurologic manifestation, seen in 25% to 50% of pa- of exposure to organic or inorganic material capable tients. One third of patients will experience bilateral of inducing noncaseating granulomas. However, di- facial nerve involvement simultaneously or sequen- agnosis ofneurosarcoidosis is quite difficult because tially. The eighth cranial nerve is the second most of the challenges in obtaining appropriate tissue di- commonly affected cranial nerve, involving 10% agnosis, and unreliable findings on imaging studies. to 20% of cases. Symptoms may include vestibular In approximately 50% of patients, neurologic symp- and/or auditory involvement bilaterally with fluctu- toms are the initial presenting complaints, most of- ations in hearing. Other cranial nerve involvement From the Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota. Presented at the meeting of the American Broncho-Esophagological Association, Phoenix, Arizona, May 28-29,2009. Correspondence: Dale C. Ekbom, MD, Dept of Otolaryngology-Head and Neck Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905. 641