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The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis.
Audiol Neurootol 2017; 22(4-5):205-217AN

Abstract

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.

Authors+Show Affiliations

Department of Otolaryngology - Head and Neck Surgery, Northwestern University, Chicago, IL, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

29166635

Citation

Greene, Jacqueline J., et al. "The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis." Audiology & Neuro-otology, vol. 22, no. 4-5, 2017, pp. 205-217.
Greene JJ, Naumann IC, Poulik JM, et al. The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis. Audiol Neurootol. 2017;22(4-5):205-217.
Greene, J. J., Naumann, I. C., Poulik, J. M., Nella, K. T., Weberling, L., Harris, J. P., & Matsuoka, A. J. (2017). The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis. Audiology & Neuro-otology, 22(4-5), pp. 205-217. doi:10.1159/000481681.
Greene JJ, et al. The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis. Audiol Neurootol. 2017;22(4-5):205-217. PubMed PMID: 29166635.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis. AU - Greene,Jacqueline J, AU - Naumann,Ilka C, AU - Poulik,Janet M, AU - Nella,Kevin T, AU - Weberling,Lindsay, AU - Harris,Jeffrey P, AU - Matsuoka,Akihiro J, Y1 - 2017/11/23/ PY - 2017/05/22/received PY - 2017/09/20/accepted PY - 2017/11/23/pubmed PY - 2019/5/10/medline PY - 2017/11/23/entrez KW - Autoimmune inner ear diseases KW - Cochlear diseases KW - Cochlear implantation KW - Cochlear implants KW - Labyrinthine diseases KW - Neurosarcoidosis KW - Psychotic disorders KW - Sarcoidosis SP - 205 EP - 217 JF - Audiology & neuro-otology JO - Audiol. Neurootol. VL - 22 IS - 4-5 N2 - 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. SN - 1421-9700 UR - https://www.unboundmedicine.com/medline/citation/29166635/The_Protean_Neuropsychiatric_and_Vestibuloauditory_Manifestations_of_Neurosarcoidosis_ L2 - https://www.karger.com?DOI=10.1159/000481681 DB - PRIME DP - Unbound Medicine ER -