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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.

Rare and diagnostically challenging, paraneoplastic syndromes can appear months to years before detection of their underlying neoplasms and are associated with rapidly progressive neurologic deficits, including cochleovestibulopathy and death. Less than 20 cases of paraneoplastic cochleovestibulopathy have been reported in the online database PubMed. We present three recent cases: one patient with a history of B-cell follicular lymphoma who developed dermatomyositis and hearing loss before detection of lymphoma recurrence in his anterior chest wall, a second patient with sudden asymmetric hearing loss, found to have a 12-cm renal mass before death, and a third with fluctuating bilateral hearing loss who was ultimately found to have a thymoma. Although characterized as type VI (non-immune rapidly progressive sensorineural hearing loss) within the Harris autoimmune inner ear disease classification system, the mechanism of paraneoplastic cochleovestibulopathy is not well understood. Although specific anti-neuronal antibodies such as anti-Hu may be associated with other paraneoplastic neurologic disorders, these antibodies have limited diagnostic utility with paraneoplastic cochleovestibulopathy. Steroids have limited efficacy with regard to hearing recovery, whereas intravenous immunoglobulin has been shown to be of benefit. These recent cases demonstrate how auditory and vestibular deficits may be indicative of a rare but potentially life-threatening occult neoplasm where timely diagnosis is critical. We believe that understanding paraneoplastic cochleovestibulopathy is of interest across a broad range of clinical practices.

Objective: Microvascular decompression (MVD) may be employed in the management of hemifacial spasm (HFS), wherein a pledget of polytetrafluoroethylene (i.e., Teflon, Chemours, Wilmington DE) is sometimes introduced to separate an offending vessel from the cisternal segment of facial nerve. Rarely, Teflon may cause a granulomatous reaction resulting in nerve palsy. We here present the first case series of facial palsy thought to be secondary to Teflon granuloma following MVD for HFS.Methods: A data repository of 1,312 patients with facial palsy was reviewed to identify individuals who had previously undergone MVD for HFS. Data collected include age at time of MVD, age at onset of facial weakness and at presentation, House-Brackmann scores, clinician-graded facial function using the Electronic Facial Paralysis Assessment scale, imaging findings, and therapeutic interventions and outcomes.Results: Six patients meeting criteria were identified. Average time between MVD with Teflon placement and onset of facial weakness was 16.1 (±4.9) years (range 9.3-23.3 years). Initial House-Brackmann scores were as follows: four patients with V/VI and one each with III/VI and IV/VI. Interventions included eyelid weight placement (n = 3), chemodenervation (n = 2), static suspension with tensor fascia latae (n = 2), dynamic reanimation with cranial nerves V to VII transfer (n = 1), and temporalis muscle transfer (n = 1).Conclusion: Teflon granuloma should be considered in the differential diagnosis for patients presenting with new onset facial weakness with a previous history of MVD for HFS. It remains unknown whether early granuloma extirpation is effective. Prompt diagnosis allows consideration of time-sensitive nerve transfer procedures to reanimate facial function.Level Of Evidence: 4 Laryngoscope, 130:1422-1427, 2020. [ABSTRACT FROM AUTHOR]

Objectives: Facial palsy causes variable facial disfigurement ranging from subtle asymmetry to crippling deformity. There is no existing standard database to serve as a resource for facial palsy education and research. We present a standardized set of facial photographs and videos representing the entire spectrum of flaccid and nonflaccid (aberrantly regenerated or synkinetic) facial palsy. To demonstrate the utility of the dataset, we describe the relationship between level of facial function and perceived emotion expression as determined by an automated emotion detection, machine learning-based algorithm.Methods: Photographs and videos of patients with both flaccid and nonflaccid facial palsy were prospectively gathered. The degree of facial palsy was quantified using eFACE, House-Brackmann, and Sunnybrook scales. Perceived emotion during a standard video of facial movements was determined using an automated, machine learning algorithm.Results: Sixty participants were enrolled and categorized by eFACE score across the range of facial function. Patients with complete flaccid facial palsy (eFACE <60) had a significant loss of perceived joy compared to the nonflaccid and normal groups. Additionally, patients with only moderate flaccid and nonflaccid facial palsy had a significant increase in perceived negative emotion (contempt) when compared to the normal group.Conclusion: We provide this open-source database to assist in comparing current and future scales of facial function as well as facilitate comprehensive investigation of the entire spectrum of facial palsy. The automated machine learning-based algorithm detected negative emotions at moderate levels of facial palsy and suggested a threshold severity of flaccid facial palsy beyond which joy was not perceived.Level Of Evidence: NA Laryngoscope, 130:32-37, 2020. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]