Sudden hearing loss as the sale manifestation of neurosarcoidosis CHARLES R. SOULIERE, JR., MD, MAJ. Me, CHARLES R. KAVA, DO, MAJ, Me, DAVID M. BARRS, MD. and ANN F. BELL, MD, Ann Arbor. Michigan. San Antonio. Texas. and Weisbaden, Germany

Sarcoidosis Is a chronic, Idiopathic granulomatous disease with frequent pulmonary, ocular, and lymphatic systemic manifestations. Central nervous system Involvement Is rare (1% to 5%), yet the most frequent presenting symptoms (48%) In patients with neurosarcoldosls are neurologic. Eighth cranial nerve abnormalities occur In 20% of these patients, typically In association with other cranial neuropathies or overt systemic disease. Isolated eighth nerve disease Isvery rare. Wereport two cases of neurosarcoldosls manifest as Isolated, sudden onset sensorineural hearing loss, one of which had a cerebellopontlne angle (CPA) granuloma that mimicked an acoustic neuroma. Both patients experienced marked steroid-Induced hearing Improvement that persisted after termination of therapy. Bralnstem evoked response audiometry (BSER) abnormality persisted despite normalization of audlologlc parameters. The Implications for diagnosis and treatment of sarcoidosis-Induced hearing loss are described. Neurosarcoldosls must be considered when evaluating patients with sudden hearing loss. Prompt steroid therapy offers hope for significant hearing recovery In these patients. (OTOLARYNGOL HEAD NECK SURG 1991;105:376.)

Sarcoidosis is a chronic granulomatous disease seen most commonly in young adults of black or Scandinavian heritage. Since its initial description by Boeck in 1899, the etiology has remained obscure, although several studies have suggested a possible infectious or immune causality.':' The diagnosis of sarcoidosis is made on the basis of a combination of clinical findings, laboratory tests, and histopathologic evidence. Patients often manifest nonspecific symptoms such as fever, weight loss, and malaise. Multiple-organ systemic involvement is typical, with pulmonary (88%), lymphatic (25% to 50%), ocular (15% to 25%), and dermatologic

From Tripier Army Medical Center (Drs. Souliere and Kava). Otologic Associates. San Antonio (Dr. Barrs). and Weisbaden USAF Hospital (Dr. Bell). The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as retlective of the views of the Department of the Army or the Department of Defense. Presented at the Annual Meeting of the American Academy of Otolaryngology-Head and Neck Surgery, New Orleans. La., Sept. 23-26. 1989. Received for publication April 6, 1990: revision received April 16, 1991: accepted April 25, 1991. Reprint requests: Charles R. Souliere, Jr., MD, 8e07 47th St. W., Tacoma. WA 98466. 23/1/30657

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(20%) manifestations seen most commonly. I To the otolaryngologist, sarcoidosis is of particular interest because of the less common head and neck involvement of salivary glands, larynx, nasal cavities, and central nervous system. Radiographic findings such as peritracheal hilar adenopathy, pulmonary infiltrates, or lytic lesions of the metacarpals and phalanges can support the clinical diagnosis, although none are pathognomonic. Serologic abnormalities seen in sarcoidosis include elevated transaminases, hypercalcemia, hypergammaglobulinemia, and elevated angiotension 1converting enzyme (ACE), which is believed to be a marker for the disease ..1 The diagnosis is best supported by examination of involved lymphatic tissue via peripheral biopsy or mediastinoscopy. Typical histopathologic findings consist of noncaseating granuloma composed of multiple epithelioid cells surrounded by mature lymphocytes. Langhan's giant cells with Schaumann and asteroid inclusion bodies are often present." These findings are not pathognomonic, however, and tuberculosis, fungal, or other infectious etiologies must be excluded before the diagnosis can be firmly established. In contrast to peripheral systemic involvement. central nervous system manifestations of sarcoidosis are rare, with a reported incidence of I% to 5%.5 Neurosarcoidosis is thought to result from basal granulomatous leptomeningitis with direct infiltration or compression of cranial nerves and adjacent structures. Though

Volume 105 Number 3 September 1991

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Sudden hearing loss in neurosarcoidosis

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controversial, most studies suggest that ncurosarcoidosis occurs within the spectrum of systemic disease. and not as an isolated entity.' Neurologic symptoms are, however, the presenting complaint in up to 48% of patients with neurosarcoidosis. I> The seventh nerve is the most common cranial nerve affected, followed in frequency by the second nerve, the ninth I tenth nerves. and finally, the eighth nerve." Eighth nerve involvement is usually associated with other cranial neuropathies and cerebellar or intraocular findings. Partial or total deafness, in the absence of other clinical signs. has not been previously reported as the initial presentation of sarcoidosis. We now describe two patients whose sole initial manifestation of neurosarcoidosis was sudden hearing loss. CASE REPORTS Case 1. A 24-year-old black male aviator went to an outside institution with sudden onset total right-sided hearing loss associated with constant, high-pitched tinnitus. He had expericnced a mild right-sided loss with tinnitus. dyscquilibrium (with tendency to full to the right), and night sweats during the 5 preceding weeks. Medical history was remarkable only for thalassemia trait. Ocular. otoscopic. and ncurologic examinations were normal except for Rhombcrg, pos-

itive with falling to the right. and Weber (512 Hz). lateralized to the left. A slight fullness was noted in the supraclavicular fossa, but no hepatosplenomegaly was present. Audiogram revealed a profound right sensorineural hearing loss (Fig. 1,A). with type A tyrnpanogram. Previous audiograms documented normal hearing. Electronystagmogram (ENG) showed no spontaneous or gaze nystagmus, but did indicate mild positional left-beating nystagmus. No response was noted to right cold or warm calories, whereas left calories wcrc normal. Head C1' scan with contrast demonstrated widening of the right internal auditory canal (lAC) without mass or lesion. At this point, the patient was referred to our institution for evaluation of a possible cercbellopontinc angle (CPA) tumor, Admission physical examination and audiogram were consistent with that noted above. Brainstcm evoked response (BSER) at maximal stimulus (100 to 120 dB) showed no response in the right car. Magnetic resonance imaging (MRI) was not available, so an air contrast C1' was performed, because the routine enhanced CT was normal. This study was remarkable for a large non-enhancing mass tilling the CPA, with widening of the lAC (Fig. 2). Additional tests ordered to evaluate a possible inflammatory Icsion demonstrated only an elevated erythrocyte sedimcntation rate (ESR) of 61 mm/hr (nl