Chronic Pachymeningitis Presenting as Asymmetric Sensorineural Hearing Loss

Oghalai, John S.; Ramirez, Alexander L.; Hegarty, Joseph L.; Jackler, Robert K.

doi:10.1097/00129492-200407000-00033

Cited by 16 publications

(23 citation statements)

References 18 publications

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“…Nonneoplastic causes of asymmetric SNHL may include labyrinthitis, chronic pachymeningitis, intralabyrinthine hemorrhage, and otospongiosis. In a retrospective study on 3,000 patients with asymmetric SNHL, 3 were found to have chronic pachymeningitis based on MRI, either linear or hypertrophic T1 enhancement of the pachymeninges (dura mater) as opposed to the leptomeninges (pia and arachnoid mater); this is what is typically enhanced in neurosarcoidosis [Oghalai et al, 2004]. The 2 pathologies are not distinct, as chronic pachymeningitis may be caused by viral, bacterial, or fungal meningitis, parasitic and other infections, malignancy, idiopathic etiology, or autoimmune diseases including neurosarcoidosis [Oghalai et al, 2004].…”

Section: Differential Diagnosismentioning

confidence: 99%

“…In a retrospective study on 3,000 patients with asymmetric SNHL, 3 were found to have chronic pachymeningitis based on MRI, either linear or hypertrophic T1 enhancement of the pachymeninges (dura mater) as opposed to the leptomeninges (pia and arachnoid mater); this is what is typically enhanced in neurosarcoidosis [Oghalai et al, 2004]. The 2 pathologies are not distinct, as chronic pachymeningitis may be caused by viral, bacterial, or fungal meningitis, parasitic and other infections, malignancy, idiopathic etiology, or autoimmune diseases including neurosarcoidosis [Oghalai et al, 2004]. In 1 study, idiopathic chronic pachymeningitis causing bilateral SNHL and facial weakness was only distinguished from neurosarcoidosis once a dural biopsy had been performed and found negative for granulomas [Christakis et al, 2012].…”

Section: Differential Diagnosismentioning

confidence: 99%

See 1 more Smart Citation

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: Differential Diagnosismentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

The Protean Neuropsychiatric and Vestibuloauditory Manifestations of Neurosarcoidosis

Greene

Naumann²,

Poulik³

et al. 2017

Audiol Neurotol

View full text Add to dashboard Cite

show abstract

“…The case we reviewed is peculiar for the bilateral and almost symmetrical hearing and vestibular loss, that is in contrast with monolateral or asymmetrical cases previously reported in literature (Oghalai et al, 2004;Iwasaki et al, 2003). Furthermore, the typically slow progressive clinical manifestation of HP was even more insidious as a consequence of the repeated steroid treatments that were given before the correct diagnosis was made.…”

Section: Discussionmentioning

confidence: 82%

Symmetric sensorineural progressive hearing loss from chronic idiopathic pachymeningitis

Bovo

Berto

Palma

et al. 2007

International Journal of Audiology

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We present the case of a 68 year-old man with a diffused hypertrophic pachymeningitis (HP) involving both internal auditory canals. The clinical symptoms were headache, decreased vision in one eye, progressive bilateral and symmetrical sensory-neural hearing loss (PSNHL) responsive to steroid treatment. Although hearing loss is a frequent manifestation of HP, only few studies reported an adequate audiological assessment and follow-up. Mechanisms related to the auditory involvement are discussed on the basis of audiological data. Gadolinium enhanced MRI is the most adequate technique for HP detection and for the differential diagnosis. A delay in the diagnosis of HP seems to be quite common and the consequences may be severe, especially in cases of optic nerve involvement. For these reasons, a cerebral MRI should probably be included in the assessment of PSNHL, especially when neurological signs coexist or are reported in the medical history.

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“…50 Although noncontrast MRI may detect features of dural inflammation, gadolinium is required to assure proper diagnosis of the described patterns. 51 Corticosteroids are the treatment of choice.…”

Section: Other Inflammatory Lesionsmentioning

confidence: 99%