An Unusual Cause of Sudden Hearing Loss

Jardine, Dinchen A.; Recupero, William D.; Conley, George S.

doi:10.1001/jamaoto.2014.2785

Cited by 7 publications

(6 citation statements)

References 7 publications

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“…methylprednisolone is used for a few days [Hoitsma et al, 2010;Jardine et al, 2015;Loor et al, 2012;Rose et al, 2014;Zajicek et al, 1999]. In many cases, patients experience stable, improved, or even complete resolution of disease although they may require long term corticosteroids [Carlson et al, 2015;Jardine et al, 2015;Loor et al, 2012].…”

Section: Etiology/pathophysiologymentioning

confidence: 99%

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Greene

Naumann²,

Poulik³

et al. 2017

Audiol Neurotol

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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.

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Section: Etiology/pathophysiologymentioning

confidence: 99%

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Greene

Naumann²,

Poulik³

et al. 2017

Audiol Neurotol

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“…In a review of the literature, Colvin [2006] reported 50 cases. Since then, fewer than 30 adult and pediatric cases have been referenced in PubMed [Colvin, Color version available online DOI: 10.1159/000514479 2006; Agari et al, 2007;Cama et al, 2011;Loor et al, 2012;Szmulewicz, and Waterston, 2012;Dhanjal et al, 2014;Rose et al, 2014;Jardine et al, 2015;Shimizu et al, 2016;Greene et al, 2017;Svrakic et al, 2017;Taous et al, 2017]. In most cases, hearing loss was mild to moderate and reversible after corticosteroid treatment.…”

Section: Discussionmentioning

confidence: 99%

“…In a review of 305 patients diagnosed with neurosarcoidosis, Carlson et al [2014] reported that an optic neuropathy is the most prevalent cranial nerve lesion (30%) followed by trigeminal, facial, and audiovestibular nerve involvement (19, 18, and 17%, respectively). Audiovestibular manifestations are therefore relatively rare in sarcoidosis and have been described in a number of case reports [Colvin, 2006;Agari et al, 2007;Cama et al, 2011;Loor et al, 2012;Szmulewicz and Waterston, 2012;Dhanjal et al, 2014;Rose et al, 2014;Jardine et al, 2015;Shimizu et al, 2016;Greene et al, 2017;Svrakic et al, 2017;Taous et al, 2017]. The typical symptom is a mild to moderate sensorineural hearing loss usually bilateral (75%) and asymmetric (75%), slowly or rapidly progressive, sudden or fluctuating, and usually associated with signs of vestibular impairment [Colvin, 2006;Carlson et al, 2014].…”

Section: Introductionmentioning

confidence: 99%

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

et al. 2021

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Introduction: Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. Results: Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. Conclusion: Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

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“…The recovery of balance problems may be spontaneous or with medication while at least partial healing in the auditory functions is observed with high dose steroid treatment. 6,7 Here in this case report, a female patient with paresthezia in the left face half who afterwards experienced sudden hearing loss twice on the same side in only a week and responded to steroid treatment, diagnosed with possible NS will be presented. Written informed consent was obtained from patient who participated in this study.…”

Section: A Rare Cause Of Sudden Hearing Loss: Neurosarcoidosismentioning

confidence: 97%

A Rare Cause of Sudden Hearing Loss: Neurosarcoidosis

Öçal¹,

Öçal²

2018

KBB ve BBC Dergisi

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arcoidosis is an inflammatory disease with multi-systemic involvement. Symptoms related with the central nervous system occur in approximately 5-7% of patients with sarcoidosis which in that case the clinical picture is called neurosarcoidosis (NS). 1 In 80% of NS cases the most common symptom is cranial neuropathy, especially facial and optic nerve neuropathy. 1-3 The involvement of the vestibulocochlear nerve occurs in 1-7% of NS patients. 1,2,4,5 The histopathological correlation of audiovestibular dysfunction in NS was first described with the autopsy findings in a deaf NS patient in 1984. 6 Researchers observed perivascular lymphocytic infiltration resulting with significant axonal degeneration and demyelization especially in the acoustic, vestibular and facial nerves. The hearing loss and vestibular dysfunction in sarcoidosis were thought to be reversible initially. However in some patients it is thought that, irreversible damage occurrs as a result of the ischemic process secondary to vasculitis. 6

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An Unusual Cause of Sudden Hearing Loss

Cited by 7 publications

References 7 publications

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

A Rare Cause of Sudden Hearing Loss: Neurosarcoidosis

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