Diagnosis Investigation And Discussion

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Behget's syndrome was diagnosed on the basis of the negative biochemical and immunological results, positive pathergy test, recurrent oral ulcerations, and the presence of both recurrent genital ulcerations and skin lesions. Additional periodontal disease and dysphonic laryngitis were diagnosed (laryngoscopy showed glottis oedema and a 1 -2 mm white lesion on the right ventricular band). On the basis of synchronous symptoms we investigated two associations: ear involvement with hearing loss and rhinitis with sinusopathy.

Rhinitis with sinusopathy was diagnosed based on rhinoscopy showing hyperaemia with no focal lesion and perinasal sinuses CT-scan images compatible with chronic pansinusopathy of ethmoido-maxilar predomination, with destructive characteristics (amputation of the conchae and sept perforation). ENT findings were: normal otoscopy; positive hearing tests for mixed conductive and sensory hearing loss of light degree on the left side and of moderate degree on the right side. CT scan of the temporal bones showed bilateral middle ear involvement; right side occlusion of the oval and round windows by liquid collection; no signs of cholesteatoma or of ossicle destruction, compatible with bilateral medial chronic otitis, particularly on the right ear. It is known that sensory hearing loss is common

in Behget s syndrome as part of neural involvement and that in several diseases, external, middle or inner ear structures are subject to immunological injury4. What seems strikingly new in this case is the existence of a chronic bilateral middle ear otitis, especially on the right ear, which could explain the conductive component of the patient hearing loss. CT-scan images, hearing tests and clinic show a good correlation, especially on the right side. We cannot yet prove that Behcet's syndrome is the cause but it seems acceptable that part of these complaints can be attributed to this non-sensory hearing loss component. To our knowledge there is no published material about destructive sinusopathy in Behcet's syndrome, and one of the few diseases that show these features is Wegener's granulomatosis which is excluded in this patient. Without a reliable histological marker for the nasal sinus biopsy histological evaluation, a clear documentation of the association between Behcet's syndrome and this entity will have to be postponed.

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