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Behçet's disease is a chronic autoimmune disease with vascular complications that are most frequently manifested as thromboembolism in veins and pseudoaneurysm in arteries. We report the case of a 13-year-old boy admitted for clinical and biological signs of rheumatic fever associated with chest pain. The clinical examination found heart sounds with a discrete systolic murmur of mitral regurgitation. The electrocardiogram showed a microvoltage with diffuse repolarisation disorder. Biologically, he had inflammatory syndrome. Transthoracic echocardiography showed circumferential pericardial effusion with anterosepto-apical hypokinesia of the left ventricle with systolic dysfunction, and a minimal mitral regurgitation. The patient was treated by corticotherapy and antibiotherapy. The outcome was marked by orogenital aphthous ulceration and decreased visual acuity related to intermediate uveitis. The retinal angiography showed a vasculitis. The late appearance of this symptom led to the right diagnosis of Behçet's syndrome. Transthoracic echocardiography showed a hypokinetic dilated cardiomyopathy left ventricular with septo apical and anterior akinesia and severe systolic dysfunction, with a defect of the inferior septal with a collar communicating the left ventricle with a giant pseudo aneurysm. Magnetic resonance imaging showed a giant pseudoaneurysm communicating with the left ventricle. The coronary computed tomography was normal. The patient had undergone surgical treatment for the pseudoaneurysm with good outcomes.
To investigate hearing loss in patients with Behçet's disease.
Materials and methods:
Twenty-four consecutive cases of Behçet's disease and 24 sex- and age-matched controls were included in this study. Pure tone and high frequency audiometric tests were performed and pure tone average hearing thresholds calculated for both groups. Transient evoked otoacoustic emission testing was also performed.
Results:
Pure tone audiometry showed a sensorineural hearing loss in 15 of the Behçet's disease ears. Hearing thresholds were significantly higher in the study group than in the control group, on both pure tone frequency (except 0.5 kHz) and high frequency audiometry. Significant reductions in transient evoked otoacoustic emission amplitude were found at 1.4 and 2 kHz in the Behçet's disease patients. There were no significant differences in reproducibility, stimulus intensity or stability, comparing the Behçet's disease patients and controls.
Conclusion:
Significantly lower mid-frequency amplitudes were found in Behçet's patients on transient evoked otoacoustic emission testing.
Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown etiology, and neurologic involvement is one of the major clinical features. When the clinicopathological and neuroradiological findings are combined, two different patterns of central nervous system (CNS) involvement in BD can be established: parenchymal and neurovascular. Neurologic involvement is one of the most devastating manifestations of BD. This involvement may occur primarily within the nervous parenchyma (n-BD) or secondarily in the cerebral vascular system (vasculo[angio]-BD). Thrombosis of cerebral large veins and sinuses is the most common feature of vasculo-BD, although thrombosis of the vena cava and portal vein may also occur in one third of these patients. BD is usually included among the systemic vasculitides but documented cerebral arteritis is extremely rare and even a debatable mechanism for CNS involvement. Cerebrovascular complications of BD are rarer than parenchymal involvement of the CNS and aseptic meningitis.
To review Behçet's disease and to describe its clinical features in the head, neck and upper respiratory tract.
Method:
A literature review was undertaken, following a Medline search of publications over a 30-year period, and utilising the expert knowledge of one of the authors (RJM) with a specialist interest in Behçet's disease.
Results:
Twenty-seven articles with ENT relevance were obtained. Otorhinolaryngological manifestations included symptoms and signs in the mouth, nose, sinus, larynx and ear.
Conclusion:
Behçet's disease is usually considered to be a condition affecting the oral cavity, eyes and genitals. This article shows that most patients will also exhibit other ENT symptoms, hearing loss in particular. Indeed, Behçet's disease may present with features other than the classic triad of symptoms. Raised awareness of the clinical features within the head and neck region will hopefully enable early diagnosis and treatment of this potentially serious condition.
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