The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis.

BACKGROUND: A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment. Although MRI-compatible cochlear implants are now available, concerns about the feasibility of recoverable hearing with cochlear implantation in these patients as well as the practical difficulty of disease monitoring due to implant artifact must be considered. RESULTS: We present 3 recent cases from different institutions. The first is a 39-year-old man with a history of progressively worsening hearing loss, followed by visual loss, delusions, agitation, ataxia, and musical auditory hallucinations, diffuse leptomeningeal enhancement on MRI with a normal serum angiotensin-converting enzyme (ACE) level but elevated cerebrospinal fluid (CSF) ACE levels, suggesting neurosarcoidosis, was treated with corticosteroids, and underwent successful cochlear implantation. The second is a 36-year-old woman with rapid-onset horizontal diplopia, left mixed severe sensorineural hearing loss (SNHL) and tinnitus, diffuse leptomeningeal enhancement on MRI, and progressive palsy of the left CNs IV, VI, VII, IX, X and XI, with altered mental status requiring admission following high-dose intravenous corticosteroids. The third is a 15-year-old boy who presented with sudden, bilateral, profound SNHL, recurrent headaches, and left facial weakness refractory to antivirals, ultimately diagnosed with neurosarcoidosis following an aborted cochlear implantation where diffuse inflammation was found, and histopathology revealed Schaumann bodies; he was treated with methotrexate and later underwent successful cochlear implantation. CONCLUSIONS: Neurosarcoidosis is an elusive diagnosis and can cause hearing loss and psychiatric symptoms. Cochlear implantation for patients with severe hearing loss should be considered once the diagnosis is confirmed, as it is possible to achieve a successful level of hearing. Psychiatric symptoms can manifest with the onset of neurosarcoidosis, result from CN deficits, or develop as a side effect from long-term, high-dose corticosteroids, and should be monitored carefully in patients with neurosarcoidosis.

Evaluation of the utricular function with the virtual-subject visual vertical system: comparison with ocular vestibular-evoked myogenic potentials.

Introduction: The subjective visual vertical (SVV) is the most frequently assessed modality of verticality perception and has been measured in a variety of clinical situations, including peripheral vestibular lesions.Aim: The main objectives are (1) to collect normative data of Virtual SVV™ from healthy subjects, and (2) to study the correlation between Virtual SVV™ and ocular vestibular-evoked myogenic potentials (o-VEMP) on healthy subjects.Materials and methods: Forty-three healthy subjects were recruited. Air conduction (AC)-elicited oVEMPs and bone conduction (BC)-elicited oVEMPs were measured. BC stimuli were produced with a RadioEar B-81 High Output Bone Transducer. Virtual SVV™ were also measured.Results: Virtual SVV™ data from our healthy subjects were consistent with previously published normative SVV data. Normal Virtual SVV™ data did not correlate with normal AC-elicited and BC-elicited oVEMPs.Conclusions: Virtual SVV™ data from our healthy subjects were consistent with previously published normative SVV data. Virtual SVV™ data from our 43 health subjects only had weak correlation with c-VEMP, AC-elicited and BC-elicited oVEMPs. These data serve as a baseline for a future study of patients with unilateral utricular dysfunction.Significance: The Virtual SVV™ can be an attractive substitute for traditional SVV in clinical settings.