Hearing and Vestibular Disturbances in Behçet's Syndrome

Gemignani, G; Berrettini, Stefano; Bruschini, Paolo; Sellari–Franceschini, Stefano; P, Fusari; Piragine, Franco; Pasero, G; Olivieri, I

doi:10.1177/000348949110000606

Cited by 52 publications

(64 citation statements)

References 10 publications

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“…The authors concluded that audiovestibular involvement in Behçet's syndrome is frequent and generally underestimated, the cochlea being more frequently involved than the vestibular labyrinth. 9 The data obtained in our series are consistent with these reports, although differences in patient selection hinder exact comparisons. Our series contains a relatively large proportion (42 per cent) of patients with neurological abnormalities and this may represent more aggressive or advanced pathology and, thus, an increased prevalence of audiovestibular involvement.…”

Section: Discussionsupporting

confidence: 90%

Labyrinthine involvement in Behçet's syndrome

2001

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We report the neuro-otological ndings in 26 consecutive patients with de nite and probable Behçet's syndrome unselected for audiovestibular complaints. Auditory and/or vestibular abnormalities were found in 19 (73 per cent) patients, with auditory involvement in 14 (54 per cent) and vestibular in 10 (38.5 per cent) of patients. Peripheral involvement was more common than central involvement for both auditory and vestibular lesions. Bilateral cochlear hearing impairment was the most common audiological nding, whereas unilateral peripheral dysfunction was the prevailing vestibular abnormality. No correlation has been found between audiovestibular lesions and other organ lesions, disease duration or age or sex of the patients. Moreover, there was a lack of interdependence between cochlear and vestibular labyrinthine lesions. We conclude that a full neuro-otological assessment in patients under investigation for Behçet's syndrome may reveal labyrinth involvement in a substantial proportion of patients. In view of the absence of a speci c diagnostic test for Behçet's syndrome, audiovestibular lesions may provide further diagnostic support for this disorder.

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Section: Discussionsupporting

confidence: 90%

Labyrinthine involvement in Behçet's syndrome

2001

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“…As demonstrated by studies of several authors [22,23] and confirmed by our experience in a large series of mixed cryoglobulinemia [24], systemic lupus [25], and Behfet disease [26,27], some autoimmune diseases may cause inner and middle ear damage and, in our opinion, SSC must be considered one of these. For this reason we pro pose an otoneurologic study in all SSC patients with audiovestibular symptoms.…”

Section: Discussionsupporting

confidence: 69%

Audiovestibular Involvement in Systemic Sclerosis

Berrettini¹,

Ferri

Pitaro

et al. 1994

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In order to evaluate the nature and association of audiovestibular disturbances and systemic sclerosis (SSC), 37 unselected SSC patients were studied with a detailed audiological and vestibular examination since November, 1987. Pure-tone audiometry, speech audiometry, impedance audiometry, brainstem response audiometry and vestibular function using electronystagmographic recording were performed. We found a rather frequent audiovestibular involvement (41 %). A hearing loss was found in 14 SSC patients; hearing loss was sensorineural in 10 cases and mixed in 4 cases. The latter revealed a finding similar to tympanosclerosis. Four patients showed altered vestibular test values and only one of these had normal hearing. Sensorineural deafness was the more frequent pathological finding and in all cases the site of lesion was cochlear. SSC appears to be directly responsible for audiovestibular damage, since in 12 out of 15 patients with such involvement, no other apparent cause could be revealed. SSC may be included among the autoimmune diseases which may cause audiovestibular disturbances.

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“…In Behçet's disease, the rate of hearing loss ranges from 12 to 80% in different studies [9,10,[32][33][34] . However, the reports on inner ear involvement in Behçet's disease are contradictory.…”

Section: Discussionmentioning

confidence: 99%

“…In reality, the involvement of the nervous system in Behçet's disease is a heterogeneous condition that may be subclassified into two major forms: (1) a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement and (2) isolated cerebral venous sinus thrombosis and intracranial hypertension [39] . Secondly, there may be retrocochlear pathology, particularly in the vestibulocochlear nerve, and a latent brainstem abnormality, which are possible in Behçet's disease [33,34,40] . Finally, in the organ of Corti in the inner ear, there may be degeneration in the efferent fibers at their synaptic terminals on the outer hair cells or alterations in neuromediators at their synaptic region.…”

Section: Discussionmentioning

confidence: 99%

Distortion Product Otoacoustic Emission Findings in Behçet’s Disease and Rheumatoid Arthritis

Bayazıt¹,

Yılmaz²,

Gündüz³

et al. 2007

ORL

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Background and Objective: This study aimed to assess cochlear functions in Behçet’s disease and rheumatoid arthritis (RA) using otoacoustic emission testing (OAE), which objectively assesses outer hair cells in the cochlea. Methods: Patients with Behçet’s disease (n = 16) and RA (n = 11) as well as 20 controls were tested using pure tone audiometry and transiently evoked (TEOAEs) and distortion product OAEs (DPOAEs). Results: Pure tone results in the Behçet’s group were not significantly different from controls (p > 0.05). Pure tone results in the RA group were significantly different than in controls at 250, 500 and 6,000 Hz (p < 0.05). TEOAEs could be obtained in all participants. DPOAE amplitudes in the RA group and controls were similar (p > 0.05). DPOAE amplitudes were significantly higher in the Behçet’s group than in controls at 1 and 2 kHz (p < 0.05). Conclusion: A subtle deterioration in hearing can be seen at low and high frequencies in RA, which is not related to outer hair cell dysfunction as detected by DPOAE testing. In the patients with Behçet’s disease who have hearing thresholds within the normal limits, outer hair cell functions seem spared with an increased activity in the apical regions of the cochlea.

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Hearing and Vestibular Disturbances in Behçet's Syndrome

Cited by 52 publications

References 10 publications

Labyrinthine involvement in Behçet's syndrome

Labyrinthine involvement in Behçet's syndrome

Audiovestibular Involvement in Systemic Sclerosis

Distortion Product Otoacoustic Emission Findings in Behçet’s Disease and Rheumatoid Arthritis

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