[Peripheral vestibular syndrome intricated pathogenic issues. A case report]. / Sindromul vestibular--aspecte patogenice intricate. Prezentare de caz.

BACKGROUND: Ménière's syndrome appears to be the final common pathway of the mechanisms the inner ear responds to almost any injury. Although the autoimmune disease seems to play a major role, exposure to intense noise can also be a trigger in the appearance and/or aggravation of the disease. MATERIAL AND METHODS: The case of 41 years old musician with a history of ankylosing spondylitis, diagnosed with Ménière's syndrome 5 years ago, is presented. Recently the symptoms became more polymorphic, revealing the association between benign paroxysmal positional vertigo (BPPV), Ménière's syndrome and high frequency hearing loss in an autoimmune background. Besides general, neurological and ENT examination, the diagnostic workup comprised of tonal audiogram, brain stem auditory evoked potentials, computerized dynamic posturography and videonistagmography. RESULTS: The final diagnosis was acute noise trauma, Ménière's syndrome, left horizontal semicircular canal BPPV, bilateral sensorineural sudden aggravated hearing loss in high frequencies (above 9 kHz) and allegedly autoimmune inner ear disease. Treatment with an association of corticosteroids, vasodilators and vitamins combined with Vannucchi's maneuver were followed by a significant relief of the symptoms. CONCLUSION: Although no causal relationship was found between acoustic trauma and increased endolymphatic pressure, both literature data and the case presented show that intense and prolonged noise exposure may aggravate peripheral vestibular syndrome.

Mechanisms of hearing disturbance in an autoimmune model mouse NZB/kl.

A subline of the NZB mouse, NZB/kl, was found to develop severe hearing disturbances at high frequency sound at the age of 4 to 6 months. Deposition of IgG was observed on the capillary wall of the stria vascularis of the mice, but the concentration of circulating immune complex did not seem to be correlated to the deposition. Electron microscopic examination revealed that the capillaries had a thick basement membrane, and in severe cases the membrane contained foamy structures of various size. In some cases the base membrane was so thick that the capillary lumen was narrowed, and the intermediate cells seemed to be damaged. No pathological findings were found in other inner ear tissues. These results suggest that the changes in the stria vascularis were possibly caused by an autoimmune mechanism which resulted in hearing disturbance.

[Wegener's granulomatosis: otologic and clinico-immunologic aspects]. / Wegener'sche Granulomatose: Otologische und klinisch-immunologische Gesichtspunkte.

Clinical, audiometric and immunological data of 26 patients with histological proven Wegener's granulomatosis are presented. In 21 patients the middle ear was involved with serous otitis media, adhesive process or subacute otitis media. 28 of 36 audiometric examinations showed a mild to moderate sensorineural hearing loss. One ear did not recover from a sudden deafness in the early stage of the disease despite of immunosuppressive therapy. Beside clinical and morphological findings in Wegener's granulomatosis the detection of anticytoplasmic antibodies against neutrophil granulocytes (ANCA) is a reliable method in diagnosing the disease. In the present study, the serum of 19 out of 26 patients with histologically proven Wegener's granulomatosis was examined for ANCA by immunofluorescence test. In high activity stage of Wegener's granulomatosis all sera showed a positive ANCA-test. 39 patients with systemic vasculitis served as controls. Only two sera from patients with leucocytoclastic vasculitis and two with panarteriitis nodosa were ANCA positive. The follow-up (35 serum probes) of 8 patients showed no ANCA after immunosuppressive induced remission. In four histologically detected cases of recurrence ANCAs were positive again. ANCAs are very useful in primary diagnosis of Wegener's granulomatosis as well as in the follow-up. In addition, the analysis reveals, that immunosuppressive therapy with cyclophosphamide and prednisone facilitates complete remission and a long survival rate of patients with Wegener's granulomatosis.