Polyarteritis nodosa: a human temporal bone study

Joglekar, Shruti S.; Deroee, Armin Farajzadeh; Morita, Norimasa; Cüreoğlu, Sebahattin; Schachern, Patricia A.; Paparella, Michael M.

doi:10.1016/j.amjoto.2009.02.006

Cited by 16 publications

(8 citation statements)

References 14 publications

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“…Consistent with pathological changes seen in humans diagnosed with PAN, tissue samples of the inner ear adjacent to the cochlear nerve of the three surviving NHPs display moderate subacute to chronic-active perivascular inflammation which multifocally surrounded smaller branches of the cochlear nerve ( Fig. 6B to D) (26). It is important to note that there was no viral staining evident in these sections, but it is impossible for us to determine whether the absence of viral staining is due to the harsh nature of the fixation and decalcification process necessary to remove the bony structures in the inner ear to enable mounting the tissues onto slides for analysis.…”

Section: Resultssupporting

confidence: 71%

“…Rapid-onset sensorineural hearing loss is a well-described complication of PAN and other autoimmune-associated vasculitides and is often the presenting symptom. Deafness associated with these vasculitis diseases is believed to be the result of inflammation and occlusion of the anterior inferior cerebellar artery (AICA) and downstream vessels, resulting in cochlear hypoxia (23)(24)(25)(26)(27)(28)(29). Consistent with pathological changes seen in humans diagnosed with PAN, tissue samples of the inner ear adjacent to the cochlear nerve of the three surviving NHPs display moderate subacute to chronic-active perivascular inflammation which multifocally surrounded smaller branches of the cochlear nerve ( Fig.…”

Section: Resultsmentioning

confidence: 87%

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Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset Sensorineural Hearing Loss following Lassa Virus Exposure in Cynomolgus Macaques

Cashman

Wilkinson

Zeng

et al. 2018

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Lassa virus (LASV) causes a severe, often fatal hemorrhagic disease in regions in Africa where the disease is endemic, and approximately 30% of patients develop sudden-onset sensorineural hearing loss after recovering from acute disease. The causal mechanism of hearing loss in LASV-infected patients remains elusive. Here, we report findings after closely examining the chronic disease experienced by surviving macaques assigned to LASV exposure control groups in two different studies. All nonhuman primates (NHPs) developed typical signs and symptoms of Lassa fever, and seven succumbed during the acute phase of disease. Three NHPs survived beyond the acute phase and became chronically ill but survived to the study endpoint, 45 days postexposure. All three of these survivors displayed continuous disease symptoms, and apparent hearing loss was observed using daily subjective measurements, including response to auditory stimulation and tuning fork tests. Objective measurements of profound unilateral or bilateral sensorineural hearing loss were confirmed for two of the survivors by brainstem auditory evoked response (BAER) analysis. Histologic examination of inner ear structures and other tissues revealed the presence of severe vascular lesions consistent with systemic vasculitides. These systemic immune-mediated vascular disorders have been associated with sudden hearing loss. Other vascular-specific damage was also observed to be present in many of the sampled tissues, and we were able to identify persistent virus in the perivascular tissues in the brain tissue of survivors. Serological analyses of two of the three survivors revealed the presence of autoimmune disease markers. Our findings point toward an immune-mediated etiology for Lassa fever-associated sudden-onset hearing loss and lay the foundation for developing potential therapies to prevent and/or cure Lassa fever-associated sudden-onset hearing loss. IMPORTANCE Lassa virus is one of the most common causes of viral hemorrhagic fever. A frequent, but as yet unexplained, consequence of infection with Lassa virus is acute, sudden-onset sensorineural hearing loss in one or both ears. Deafness is observed in approximately 30% of surviving Lassa fever patients, an attack rate that is approximately 300% higher than mumps virus infection, which was previously thought to be the most common cause of virus-induced deafness. Here, we provide evidence from Lassa virus-infected cynomolgus macaques implicating an immune-mediated vasculitis syndrome underlying the pathology of Lassa fever-associated deafness. These findings could change the way human Lassa fever patients are medically managed in order to prevent deafness by including diagnostic monitoring of human survivors for onset of vasculitides via available imaging methods and/or other diagnostic markers of immune-mediated vascular disease.

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Section: Resultssupporting

confidence: 71%

Section: Resultsmentioning

confidence: 87%

Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset Sensorineural Hearing Loss following Lassa Virus Exposure in Cynomolgus Macaques

Cashman

Wilkinson

Zeng

et al. 2018

mBio

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“…HBV/HCV infections can stimulate the production of inflammatory cytokines such as tumor necrosis factor-alpha, interleukin-1, and interleukin-6, which are injurious to the cochlear hair cells [26]. In addition, hepatitis virus infection has a well-documented association with polyarteritis nodosa, which is a life-threatening necrotizing vasculitis that may result in hearing loss [24, 27]. …”

Section: Discussionmentioning

confidence: 99%

Increased risk of sudden sensorineural hearing loss in patients with hepatitis virus infection

et al. 2017

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The etiology of sudden sensorineural hearing loss (SSNHL) remains unclear. Possible causes of SSNHL include vascular diseases, viral infection, and autoimmune disorders. Therefore, we investigated whether hepatitis virus infection is correlated with the risk of SSNHL. Using data from the Taiwan Longitudinal Health Insurance Database, we conducted a retrospective matched-cohort study to compare patients diagnosed with hepatitis B or C virus (HBV/HCV) infections from January 1, 2000, to December 31, 2010, (N = 170,942) with frequency-matched controls (N = 512,826) at a ratio of 1:3 by sex, age, and index year. We followed each patient until the end of 2010 and evaluated the incidence of SSNHL. At the end of the follow-up period, 647 (0.38%, 647/170,942) patients developed SSNHL in the HBV/HCV group compared with 978 (0.19%, 978/512,826) in the control groups, with a statistical significance of P < 0.001 (using the log-rank test). The incidence rate ratio of SSNHL was 5.743-fold higher in the HBV/HCV group than in the control group (283.17 vs. 49.31 per 100,000 person-years, P < 0.001). The risk of SSNHL increased with HBV/HCV infection, and an adjusted hazard ratio of 5.103 (95% CI, 4.585–5.678) was determined using Cox proportional hazards regression. This study contributes to the awareness of the increased risk of SSNHL in HBV/HCV-infected populations. Our findings suggest that an underlying viral infection contributes to the development of SSNHL.

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“…The hearing loss if detected early, can be reversed with immunosuppressive treatment (2,6,7) and is presumed to be the result of vasculitis. Several lines of evidence support the role of vasculitis in some patterns of hearing loss.…”

Section: Discussionmentioning

confidence: 99%