Audio‐Vestibular Disturbance in Patients With Behçet's Disease

Choung, Yun‐Hoon; Cho, Min Jung; Park, Keehyun; Choi, Seong–Jun; Shin, You Ree; Lee, Eun‐So

doi:10.1097/01.mlg.0000237442.80711.65

Cited by 26 publications

(23 citation statements)

References 11 publications

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“…Also, vestibular involvement has been reported in 10 to 59% of cases. 9,[21][22][23] Gemignani et al found cochlear hearing impairment in 12 of 20 patients with Behçet disease, and they concluded that the cochlea is involved more frequently than is the vestibular labyrinth. 24 As another indicator of the inner ear involvement, we found no case of sudden hearing loss in our series.…”

Section: Discussionmentioning

confidence: 99%

Is There a Need for Audiologic Evaluation in Patients with Behçet Disease?

2012

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Behçet disease is known to be a multisystem condition. We conducted a study to determine the prevalence of hearing loss in patients with Behçet disease and to identify any associations between audiologic findings and other clinical manifestations and treatment. Our study group was made up of 41 adults with Behçet disease and 41 healthy sex-and age-matched controls. All patients and controls underwent a complete clinical otolaryngologic examination, which included pure-tone audiometry, acoustic impedance testing, and otoacoustic emissions testing. Audiology revealed that the prevalence of sensorineural hearing loss (SNHL) was significantly higher in the Behçet patients than in the controls-68.3 vs. 22.0% (p < 0.002). The duration of Behçet disease had no significant impact on whether patients did or did not experience hearing loss. Hearing loss was the fourth most common clinical finding in the Behçet group, after oral ulcers, genital ulcers, and skin lesions. We conclude that SNHL is present in a significant number of Behçet patients, and we suggest the need for an adequate investigation of hearing in the routine follow-up of these patients.

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Section: Discussionmentioning

confidence: 99%

Is There a Need for Audiologic Evaluation in Patients with Behçet Disease?

2012

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“…Hearing loss has been sporadically described in all of the systemic vasculitides, yet it is most frequently associated with WG with up to 52% of patients having evidence of OM and 8% developing SNHL, 5 whereas patients with CSS have a lower prevalence of otitis (around 10%), but similar rates of SNHL 13 . Although not as well recognized, SNHL in Behçet's disease is reported in 23–55% of patients, suggesting that hearing loss represents the fourth most prevalent clinical symptom after oral and genital ulcers and skin lesions 3,14 …”

Section: Discussionmentioning

confidence: 99%

“…13 Although not as well recognized, SNHL in Behçet's disease is reported in 23-55% of patients, suggesting that hearing loss represents the fourth most prevalent clinical symptom after oral and genital ulcers and skin lesions. 3,14 Less commonly, otologic manifestations form the presenting complaint of patients with systemic vasculitis. Fauci et al, in their report on the long-term follow-up of 85 patients with WG, noted that 25% of patients present with serous OM and 6% with hearing loss as the initial sign of the disease.…”

Section: Discussionmentioning

confidence: 99%

“…Otological manifestations, including chronic otitis media (OM), sudden or progressive sensorineural hearing loss (SNHL), and vestibular dysfunction, have been described in various autoimmune disorders and systemic vasculitides such as systemic lupus erythematosus (SLE), 1 rheumatoid arthritis, 2 Behçet's disease, 3 Sjögren's syndrome, 4 Wegener's granulomatosis (WG), 5 polyarteritis nodosa, 6 Churg–Strauss syndrome (CSS), 7 temporal arteritis, 8 Takayasu's arteritis, 9 Cogan's syndrome, 10 Kawasaki disease, 11 and antiphospholipid syndrome 12 . Hearing loss as the primary manifestation of a systemic vasculitis is an uncommon, frequently overlooked presentation.…”

Section: Introductionmentioning

confidence: 99%

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Hearing Loss as the Presenting Feature of Systemic Vasculitis

Lidar

Carmel

Kronenberg

et al. 2007

Annals of the New York Academy of Sciences

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Hearing loss, both sensorineural and conductive, is a well-recognized manifestation of systemic vasculitis, yet has only infrequently been described as its initial presentation. We describe three cases in which hearing loss preceded the diagnosis of systemic vasculitis by a period of a few weeks to more than 6 months. While steroid therapy was initiated immediately in one of the cases, comprehensive immunosuppressive therapy was given only after additional manifestations compatible with vasculitis appeared, allowing for a diagnosis of Wegener's granulomatosis in two of the cases and undifferentiated vasculitis, in the third. Hearing improved in all patients, albeit, residual dysfunction remained. Prompt referral for a complete rheumatologic workup in cases of unexplained hearing loss would allow for a timely diagnosis of a collagen vascular disease and facilitate early initiation of systemic therapy, possibly yielding better audiological results.

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“…Neurotologic manifestations have also been reported and cochlear and neural hearing loss are known to be not uncommon. Audio-vestibular disturbance 5) and vestibulo-ocular reflex abnormality have also been reported 16) . In Neuro-Behcet's disease, involvements of intracranial or extracranial arteries or veins have been reported.…”

Section: Clinical Features Of Neuro-behçet's Diseasementioning

confidence: 99%

Rapid Atypical Progression of Neuro-Behçet's Disease Involving Whole Brainstem and Bilateral Thalami

Lee

Choi

Kim

et al. 2011

J Korean Neurosurg Soc

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68called only a mild head trauma, due to a traffic accident, two weeks before. Brain MRI performed at the local hospital revealed abnormal signal intensity at the left tegmentum of the midbrain in Axial Fluid-Attenuated Inversion-Recovery (FLAIR) images (Fig. 1). In small portions of the brainstem, minimal peri-lesional swellings were noted; however sulci around the lesions were well recognized. Abnormal signal regions showed ill-defined margins. Neurologic and radiologic findings by the referring radiologist suggested lacuna brainstem infarction.The chief complaint of the patient was mild headache. His vital signs were stable and his complete blood count, routine biochemistry tests, urinalysis, liver function tests, thyroid function INTRODUCTIONBehçet's disease is a chronic relapsing inflammatory disorder of unknown etiology that involves many organ systems including the central nervous system (CNS). Neurologic deficits occur in 10% to 30% of patients and may be the presenting feature in up to 5% of patients 10) . Behçet's disease is characterized by recurrent oral and genital ulcers, relapsing uveitis, mucocutaneous, articular, neurologic, urogenital, vascular, intestinal, and pulmonary manifestations. In this article, we present our experience of a Behçet' s disease that showed unpredictably rapid respiratory distress and neurologic deterioration. We also discuss the clinical presentations, diagnostic tools, treatment options, and prognosis of Behçet's disease. CASE REPORTA 47-year-old man with a mild headache visited the neurosurgery department of our hospital as recommended by a local radiologist. His family history was unremarkable, and he re- We present a case of Neuro-Behçet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet's disease with CNS involvement. The patient was treated with highdose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, lowgrade glioma, multiple sclerosis, and occlusive venous disease.

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Audio‐Vestibular Disturbance in Patients With Behçet's Disease

Cited by 26 publications

References 11 publications

Is There a Need for Audiologic Evaluation in Patients with Behçet Disease?

Is There a Need for Audiologic Evaluation in Patients with Behçet Disease?

Hearing Loss as the Presenting Feature of Systemic Vasculitis

Rapid Atypical Progression of Neuro-Behçet's Disease Involving Whole Brainstem and Bilateral Thalami

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