Bilateral Vestibular Hypofunction in Neurosarcoidosis: A Case Report

Abstract

We describe the case of a 59-year-old woman who presented with progressive bilateral vestibular hypofunction and who was found to have bilateral granulomatous mass lesions of the mesial temporal lobe. Initially, her condition stabilized neurologically with corticosteroids, but a diagnosis of neurosarcoidosis was delayed because of the unusual presentation and persistently normal chest imaging results and serum angiotensin-converting enzyme (ACE) levels. Approximately 1 year after her initial presentation, the patient died of complications of a myocardial infarction and pulmonary embolism. Sarcoidosis should be considered in the differential diagnosis of idiopathic bilateral vestibular hypofunction even if the chest imaging and serum ACE levels are normal, particularly when there is evidence of a multisystem process.

Case report

“…[ 8 , 9 , 12 , 14 , 16 , 18 , 28 , 29 ] Chorea, cerebral vasospasm, progressive multifocal leukoencephalopathy, and vasculitis are rare. [ 1 , 2 , 4 , 16 , 18 , 20 , 22 , 25 , 30 – 33 ] The cranial neuropathies are not restricted, but nerves VII, II, and VIII are most commonly involved. Although generally unilateral, palsy of cranial nerve VII may be bilateral in up to one-third of patients.…”

“…[ 22 , 55 ] Useful parameters in establishing a diagnosis of “probable NS” are chest CT, elevated BAL CD4:CD8 ratio, and CSF CD4:CD8 ratio >5. [ 1 , 12 , 16 – 18 , 22 , 25 , 28 , 56 – 58 ] Gallium-67 scans, BAL samples, and ACE levels can be used to monitor disease activity. [ 35 , 57 – 62 ]…”

“…Sarcoidosis (also known as Besnier–Boeck disease) is a well-known inflammatory disorder of the lung and other organ systems, with a clear predilection for young or middle-aged adults. [ 1 – 3 ] Neurosarcoidosis (NS) is an uncommon manifestation of systemic sarcoidosis that occurs more often in middle-aged women. [ 3 – 7 ] Often undetected until autopsy, the 5% to 10% rate of neurologic involvement reported in the literature indicates that current diagnostic methods lack sensitivity and specificity.…”

“…Treatments may differ dramatically and be harmful if an improper diagnosis is made. [ 1 , 4 , 11 , 13 , 15 , 16 ] Isolated NS, devoid of other organ involvement, is a rare but often significant cause of morbidity and mortality. [ 2 , 5 , 11 , 15 , 17 – 23 ] Necrotizing NS is prone to vasculitic patterns.…”

Clinical Practice Guideline: Sudden Hearing Loss (Update)

“…SarcoidosisPulmonary symptoms, bilateral vestibular loss, elevated angiotensinconverting enzyme level, abnormal Gallium scan270,271 Hyperviscosity syndrome Mucous membrane bleeding, neurologic and pulmonary symptoms, associated retinopathy272 …”