Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

Kim, Sang Hoon; Jung, Ah Ra; Kim, Su Il

doi:10.7874/jao.2016.20.1.55

Cited by 13 publications

(5 citation statements)

References 9 publications

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“…Unilateral PFP has been reported in advanced local disease [6,8] but it is extremely rarely the presenting fea-ture. Moreover, two case reports described ipsilateral facial nerve paralysis with bilateral sensorineural hearing loss as initial GPA presentation [10,11]. On the other hand, we showed the combination of ipsilateral PFP and unilateral severe MHL as presenting signs of the GPA.…”

Section: A B Csupporting

confidence: 49%

“…The standard treatment for the systemic form of GPA is an immunosuppressive such as CYC or rituximab, along with corticosteroids in high doses [1]. Kim, et al [10] described significant effect of rituximab on hearing loss and PFP. In our case, pulse doses of 1,000 mg/day IV CYC and 10 mg/kg/day IV methylprednisolone were given for the first 7 days.…”

Section: A B Cmentioning

confidence: 99%

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A Case of Unilateral Otologic Symptoms as Initial Manifestations of Granulomatosis With Polyangiitis

Batinović

Martinić²,

Durdov

et al. 2023

J Audiol Otol

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Presenting concernsA 36-year-old male patient arrived at the ENT Emergency Department (EED) with extreme pain in his left ear and a long history of left acute otitis media and noise exposure. The patient reported several weeks of persistent otalgia, left-sided hearing loss, and a sudden-onset left-sided facial weakness. Clinical findings and diagnostic assessmentsOn examination, only erythema of the external auditory canal and a purulent effusion from the perforated left eardrum was detected. A mild peripheral left-sided facial palsy was detected and categorized as House-Brackmann (HB) grade 2/6. The function of other cranial nerves was normal. The initial neurological screening done before presentation in the EED was unremarkable.With the preliminary diagnosis of acute otitis media compli-

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Section: A B Csupporting

confidence: 49%

Section: A B Cmentioning

confidence: 99%

A Case of Unilateral Otologic Symptoms as Initial Manifestations of Granulomatosis With Polyangiitis

Batinović

Martinić²,

Durdov

et al. 2023

J Audiol Otol

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“…As with immune-mediated sensorineural hearing loss, SSNHL with immune-mediated etiology is difficult to diagnose because of the lack of tests and biomarkers that can define the immunologic cause [ 4 , 5 ]. However, there are reports that such autoantibodies as anti-endothelial antibodies, anticardiolipin, anti-beta2-glycoprotein-1, and anti-heat shock protein-70 antibodies are involved in SSNHL [ 6 , 7 ], and the development of SSNHL in patients with systemic lupus erythematosus (SLE), psoriasis, and Wegener granulomatosis supports the immune-mediated etiology of SSNHL [ 8 , 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Psoriasis Increases the Risk of Sudden Sensorineural Hearing Loss: A Longitudinal Follow Up Study Using a National Sample Cohort

Choi

Park

Hong

et al. 2020

IJERPH

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Psoriasis is a well-known immune-mediated disease. Its autoimmune pathophysiology is consistent with the immune-mediated systemic vascular hypothesis regarding the pathogenesis of sudden sensorineural hearing loss (SSNHL). The purpose of our study was to investigate whether psoriasis affects the prevalence of SSNHL in all age groups matched by age, sex, income, and region of residence. Korean Health Insurance Review and Assessment Service-National Patient Samples were collected from 2002 to 2013. A 1:4 matched psoriasis group (n = 12,864) and control group (n = 51,456) were selected. The crude (simple) and adjusted (Charlson comorbidity index) hazard ratios (HR) for psoriasis and SSNHL were analyzed using the stratified Cox proportional hazard model. The incidence of SSNHL was significantly higher in the psoriasis group than in the control group (0.5% vs. 0.4%, p = 0.004). Psoriasis increased the risk of SSNHL (adjusted HR = 1.44, 95% confidence interval (CI) = 1.09–1.90, p = 0.010). In the stratification analysis, the incidence of SSNHL was significantly higher in the 30–59-year-old group than other group SSNHL (adjusted HR = 1.50, 95% CI = 1.06–2.12, p = 0.023). In addition, SSNHL occurred significantly more frequently in men with psoriasis (adjusted HR = 1.70, 95% CI = 1.17–2.49, p = 0.006). Psoriasis increased the risk of SSNHL, and SSNHL was more prevalent in between the age of 30–59-year-olds and men with psoriasis.

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“…In the described cases, apart from systemic treatment, patients also received local treatment. Intratympanic steroid injection is typically administered in the treatment of Meniere’s disease or idiopathic sudden sensorineural hearing loss [ 40 , 41 ] and has not been described as a standard procedure in GPA. In our case, due to massive inflammation in the temporal bone with progression in the base of the skull, we decided on a “rescue treatment” with dexamethasone intratympanic injections, theorizing that local injections with glucocorticoid steroids may be effective in limiting inflammation in temporal bone infiltration.…”

Section: Discussionmentioning

confidence: 99%

Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration

Bonek,

Brożek-Mądry,

Wroński

et al. 2023

Brain Sciences

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Objectives: To present a personalized approach in three cases of treatment-resistant, locoregionally aggressive forms of cANCA-positive granulomatosis with polyangiitis (GPA) and skull base involvement. Methods: Three patients with GPA and skull base involvement were described alongside a critical review of the current literature. Results: All presented patients suffered from GPA with an inflammatory tumor at the skull base, alongside cerebellopontine angle involvement, cranial nerve palsies, cerebellar disorders, concomitant hearing loss, and severe otalgia. Symptoms were associated with progressive granulomatous destruction of the temporal bone, laryngopharynx, and central nervous system infiltration. Treatment with cyclophosphamide and high doses of glucocorticoid steroids were ineffective but subsequent therapy with rituximab was successful in the presented cases. The literature review showed that the course of the disease with skull base involvement is associated with poorer clinical and radiological responses to standard pharmacotherapies. Conclusion: Granulomatous inflammation localized in the skull base is associated with a more aggressive disease progression and is less likely to respond to pharmacotherapy. Standard induction therapy with cyclophosphamide and glucocorticoid steroids may be ineffective. A better response may be achieved by using rituximab and concomitant local treatment with glucocorticoid steroid injections.

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Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

Cited by 13 publications

References 9 publications

A Case of Unilateral Otologic Symptoms as Initial Manifestations of Granulomatosis With Polyangiitis

A Case of Unilateral Otologic Symptoms as Initial Manifestations of Granulomatosis With Polyangiitis

Psoriasis Increases the Risk of Sudden Sensorineural Hearing Loss: A Longitudinal Follow Up Study Using a National Sample Cohort

Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration

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