Audiovestibular Involvement Research Articles

Phenotyping vestibulocochlear manifestations in Susac syndrome: a cohort study.

To characterize vestibulocochlear involvement in patients with Susac syndrome (SuS), a rare immune-mediated endotheliopathy of cerebral, retinal and inner ear microvasculature causing a triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. The electronic patient files of 21 patients with SuS are reviewed for data on demography, clinical presentation, disease course and audiovestibular testing. All 21 patients experienced some form of audiovestibular complaints during the disease course, with vertigo and instability being most frequently reported, followed by hearing loss, tinnitus and aural fullness. These audiovestibular symptoms did not always coincide. Fifteen patients had objectified predominant low- to midfrequency sensorineural hearing loss and 8 out of 18 patients showed abnormalities on vestibular testing, most frequently vestibular evoked myogenic potential-abnormalities, indicating otolith dysfunction. Treatment protocols consisted of uniformly extensive immunosuppressive therapy and hearing loss remained mostly mild. Audiovestibular involvement is very common in patients with SuS. Characteristic findings include a "reverse-slope" configuration on audiological testing and otolith dysfunction on vestibular testing. Aggressive immunosuppression may prevent severe audiovestibular dysfunction. Symptoms as aural fullness and otolith dysfunction may indicate an underlying hydrops. Further investigations are necessary to elucidate the histopathological mechanisms underlying these preferentially involved cochleovestibular areas. Early recognition and treatment of SuS are important to stabilize or decrease disease activity and might also have beneficial effects on inner ear outcome. THE SUBMITTED MANUSCRIPT REPORTS DATA DERIVED FROM CLINICAL OBSERVATIONS IN HUMANS: Consent for the research was provided by the Ethics Committee of Ghent University hospital (application number 2019/1443, registration date 31/12/2021, principal investigator Guy Laureys).

Audiovestibular Involvement in Patients With Systemic Sclerosis.

Audiovestibular dysfunction has been reported in many autoimmune connective tissue diseases, including systemic sclerosis (SSc). To assess the prevalence and features of audiological and vestibular disturbances in SSc patients and evaluate their relationship with disease duration, clinical features, nailfold videocapillaroscopy pattern, and immunologic profiles. A matched case-control study was conducted in a rheumatology clinic of a second-level hospital over 24 months. All patients underwent a detailed ear, nose, and throat examination, as well as audiometric and vestibular assessments, including pure tone audiometry, speech audiometry, immittance tests, and the Video Head Impulse Test. Thirty-five SSc patients and 24 healthy controls were included in the study. In the SSc group, subjective hearing loss was reported by 17.1% of patients, vertigo by 14.3%, tinnitus by 11.4%, and dizziness by 5.7%. Sensorineural hearing loss was identified in 42.9% of SSc patients, significantly higher than in the control group (p = 0.013). There was no correlation between audiological manifestations and clinical symptoms, organ involvement, immunologic characteristics, and treatment. Vestibular dysfunction was detected in 60% of SSc patients, significantly higher than the control group (p = 0.05). A significant correlation was found between abnormal Video Head Impulse Test and the presence of anti-RNA polymerase III and anti-Th/To antibodies (p = 0.05 and p = 0.034, respectively). Our study revealed an increased prevalence of sensorineural hearing loss and vestibulopathy in SSc patients.

Chronic inflammatory demyelinating polyneuropathy: evaluation of the vestibular system with cervical and ocular vestibular evoked myogenic potentials.

To investigate the possibility of vestibular damage in a group of patients suffering from chronic inflammatory demyelinating polyneuropathy (CIDP) using a diagnostic protocol including the caloric test, C-VEMPs and O-VEMPs. Twenty patients suffering from CIDP (mean age 58.5 years, range 33-80 years; 4 women and 16 men) were investigated. To assess any eventual audio-vestibular involvement, all patients of the study underwent pure tone audiometry, Fitzgerald-Hallpike caloric vestibular test, C-VEMPs and O-VEMPs. In 11 patients with CIDP values of both O-VEMPs and C-VEMPs were either absent or abnormal. An absent trace at O-VEMPs testing occurred in 36% of these pathological patients, whereas an increase of n10 latency and amplitude was present in the other 64% . A specific diagnostic protocol including the caloric test, C-VEMPS, O-VEMPS, could be useful when employed for identifying vestibular damage in CIDP patients.

Inner ear dysfunction in patients with Behcet\u2019s disease

BackgroundBehcet’s disease (BD) is a rare immune-mediated small-vessel systemic vasculitis that often presents with mucous membrane ulceration and ocular problems. Alajouanine and colleagues first described inner ear involvement in BD in 1961. The cochlea is more frequently involved than the vestibular labyrinth or the central vestibular tracts. Hearing is commonly impaired in BD and rates vary between 24 and 62%.ObjectiveThe current study investigated the incidence, relationship, and characteristics of audiovestibular disturbances in patients with BD compared with age and sex-matched healthy individuals.Patients and methodsThe participants of this prospective study classified into two groups. The study group consisted of 15 patients (six men and nine women) with BD and the control group consisted of 15 healthy volunteers (five men and 10 women) without otological symptoms. A wide range of tests were conducted; all patients were evaluated by conventional pure-tone audiometry (0.25–8.0 kHz), high-frequency audiometry (8.0–16.0 kHz), and vestibular test battery in terms of bithermal caloric test and computerized dynamic posturography testing.ResultsThere was no significant differences between the two groups in terms of sex and age. Abnormalities of the pure-tone audiometry showed that 34% of patients demonstrated variable degrees of sensorineural hearing loss, while 60% of patients demonstrated abnormal audiograms at the high-frequency audiometry. In terms of vestibular assessment, 34% of patients demonstrated abnormalities at the caloric test, while 47% demonstrated abnormalities at the computerized dynamic posturography testing.ConclusionThe outcome of this study confirms the association between the inner ear dysfunction and the BD. Inner ear dysfunction in BD could be attributed to the presence of vasculitis or neuropathy that could happen on top of chronic inflammation process associated with BD. Therefore, patients with BD should be regularly subjected to otolaryngological evaluation to diagnose and for follow up of any audiovestibular involvement.

Study of audiovestibular dysfunction in children with systemic lupus erythematosus

ObjectiveInner ear dysfunction in systemic lupus erythematosis patients has been reported but audiovestibular involvement is not well documented especially in pediatrics. This study was designed to evaluate silent audiovestibular dysfunction among SLE children. MethodsCase control study examined in allergy and immunology clinic; pediatrics hospital and audiovestibular clinic; Ain Shams University from January 2009 to December 2010. Thirty-five systemic lupus erythematosus children (diagnosed according to American College of Rheumatology); age group 8–16 years, were randomly selected. Five of them were excluded due to one or more exclusion criteria (previous otitis media, stroke, lupus cerebritis, meningitis or encephalitis, audiovestibular symptom). Ten of them refused enrollment or could not complete full battery. Seventeen females and three males, mean age 12.9±2.6 years, completed the study. Control group included 20 normal subjects, age and sex matched. Full clinical assessment, basic audiological evaluation and vestibular testing (videonystagmography VNG and computerized dynamic posturography CDP) were conducted for children included in the study. ResultsFive systemic lupus erythematosus patients had sensorineural hearing loss strongly associated with +ve antiphospholipid antibody and two had conductive hearing loss. Two children in control group had conductive hearing loss (p=0.05). Abnormal VNG findings was significantly higher among systemic lupus erythematosus children (40%) compared to controls (0%) and associated with +ve antiphospholipid antibodies (χ2=10, p=0.002, Fisher exact test=0.003). Twenty-five percentage of systemic lupus erythematosus children had abnormal CDP findings reflecting impaired balance function associated with positive antiphospholipid antibodies showing significant statistical difference compared to controls (0% affection) (χ2=5.7, p=0.017, Fisher exact test=0.047). ConclusionSilent audiovestibular dysfunction is prevalent among systemic lupus erythematosus children especially those positive for antiphospholipid antibodies necessitating routine regular evaluation.

AB0495 Audiovestibular involvement in systemic sclerosis: our exprerince

BackgroundHearing and balance disorders may occur in systemic sclerosis (SSc) as a result of the immune-mediated inner ear damage, the etiology being vasculitis and fibrosis. Objective of our study was...

Audiovestibular Manifestations in Patients With Limited Systemic Sclerosis and Centromere Protein-B (CENP-B) Antibodies

Audiovestibular dysfunction has been reported in patients with connective tissue disease. Systemic sclerosis (SSc; scleroderma) is a rare connective tissue disease of unknown etiology. In the current study we assess whether audiovestibular involvement is present in patients with limited scleroderma (lSSc). To answer this question we studied a series of 35 consecutive patients who fulfilled well-established classification criteria for lSSc and had antibodies against the major centromere protein-B (CENP-B), and 59 matched controls. Individuals with a history of cerebrovascular complications, syphilis, Ménière and other vestibular syndromes, infections involving the inner ear, barotrauma, or in treatment with ototoxic drugs were excluded. The majority of patients with lSSc were women (94%). The mean age at time of study was 64.5 years, and the mean age at time of disease diagnosis was 56.9 years. Besides Raynaud phenomenon, most patients with lSSc had other typical features of CREST (calcinosis, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia) syndrome. Twenty-seven (77%) patients showed abnormal hearing loss in the audiogram compared with only 15 (26%) of the controls (p < 0.001). Values of audiometric tests (pure-tone average and speech reception threshold) yielded significant differences between patients and controls (p < 0.001). The typical pattern of hearing impairment in our series of lSSc patients was a bilateral and symmetrical sensorineural hearing loss with a flat pattern in the audiogram. Abnormal tympanogram and abnormal stapedial reflex were more commonly observed in patients than controls (p < or = 0.001). Similarly, a significantly increased frequency of abnormal oculocephalic response (10 patients, 29%) and head-shaking nystagmus (9 patients, 26%) was observed in patients compared with controls (p < 0.001 for both comparisons). Finally, a significantly increased frequency of abnormal caloric test and clinical test of sensory integration and balance was observed in lSSc patients (31% and 46% of patients, respectively) compared with controls (0% and 12%, respectively) (p < 0.001 for both comparisons). The current study demonstrates strong evidence for inner ear compromise in patients with lSSc.

Hearing loss in Behçet syndrome

To determine the prevalence and characteristics of hearing loss in Behçet syndrome. This study included 27 patients with Behçet syndrome and 35 sex-and age-matched controls. A complete audiological evaluation was performed. The average pure-tone audiograms from both groups showed a statistically significant hearing loss in the Behçet group. Sixteen patients (59.26%) showed some degrees of sensorineural hearing loss (SNHL), with the high-frequency type (4, 8, 10, and 12 kHz) being the most common pattern (93.75%). Hearing loss was the fourth most common manifestation. Although the patient's age, sex, and the duration of the disease were not related to hearing loss, there was a significant correlation between a negative pathergy test and hearing loss in patients with Behçet syndrome. We should consider audiovestibular involvement in Behçet syndrome as a common finding.

Audiovestibular Manifestations of Sarcoidosis: A Review of the Literature

Sarcoidosis is a multisystem disease of unknown etiology. Audiovestibular involvement is rare but has been reported in a number of cases. The objective of this review is to provide an evidence-based summary of the audiovestibular manifestations of sarcoidosis by collating the findings of these case reports. Retrospective review of 48 published case reports and 2 cases recently encountered in audiovestibular medicine clinics. Case reports were identified using a key word search of Medline database. Clinical details, audiovestibular test results, and radiological findings were recorded for each patient when available. When type of hearing loss (HL) was recorded, all losses were sensorineural, with only two patients found to have an additional conductive loss. The severity of HL ranged from mild to profound. Median thresholds were moderately raised. The HL was bilateral in 75% of patients and asymmetrical in 75% of these cases. Seventy percent of HLs demonstrated some recovery. Many of the patients with recovery were treated with corticosteroids, but no statistically significant association between treatment and HL outcome was observed. Symptoms of vestibular impairment were common. Vestibular testing was performed in 24 cases and was abnormal in 23. Eighty-one percent of patients had additional features of neurosarcoidosis. Six patients had radiological evidence of a retrocochlear lesion. In taking into account the evidence from the clinical features, audiovestibular testing, radiological investigations, and postmortem findings, it is concluded that the audiovestibular manifestations of sarcoidosis are likely to be primarily a result of vestibulocochlear nerve neuropathy.

Audio-vestibular evaluation in patients with Behçet's syndrome.

A prospective controlled clinical study was carried out at the Department of Ophthalmology and ENT, Inönü University Medical Faculty, Turgut Ozal Medical Center, Research Hospital, to evaluate the audio-vestibular involvement in patients with Behçet's syndrome compared with controls. Twenty-five consecutive patients with Behçet's syndrome (mean age +/- SD, 34.96 +/- 8.50) and 20 age- and sex-matched healthy volunteers (hospital staff) as control subjects (mean age +/- SD, 34.45 +/- 9.16) were included in this study. Behçet's patients were divided into two groups according to the number of criteria, complete (all four major criteria) and incomplete (three major criteria without ocular involvement). The groups were compared with each other or controls regarding inner ear involvement. Audiometric pure-tone thresholds at 125 to 8000 Hz were obtained in all subjects in both groups, and pure tone average (PTA) hearing thresholds were calculated for the middle, high and low frequencies. In addition, short increment sensitivity index (SISI), tone decay and BERA examinations were performed in all Behçet's patients. Sensorineural hearing loss (SNHL) was present in six of 25 patients with Behçet's syndrome. Two Behçet's patients had unilateral total SNHL, two had bilateral moderate level SNHL, one had bilateral low-frequency SNHL and one bilateral high frequency SNHL. In two, BERA, and in five SISI, examination disclosed inner ear involvement. In control subjects, the past medical history was normal and there was no consistent audio-vestibular complaint. Their PTA thresholds were all in the normal range. Otoscopic examination findings were normal, with intact, mobile tympanic membranes in both groups. The present study showed that audio-vestibular involvement is not infrequent in Behçet's syndrome compared with age- and sex-matched healthy controls, and it is under-estimated. All Behçet's patients should regularly be followed by an otolaryngologists and be given information about the possibility of inner ear involvement. According to our results, hearing loss occurs more often in older patients and also in the complete form of Behçet's syndrome.

Cogan-I-Syndrom: Zu oft zu spät erkannt? - Ein Beitrag zur Frühdiagnose des Cogan-I-Syndroms

Cogan's syndrome is an uncommon disease characterized by ocular inflammation, vestibuloauditory dysfunction and symptoms of systemic disease. The etiology is unknown, however there is evidence for an autoimmune pathogenesis. The "typical" Cogan's syndrome presents as bilateral interstitial keratitis and progressing vestibuloauditory dysfunction. The presence of other inflammatory manifestations in addition of keratitis has been termed as "atypical" Cogan's syndrome. We report on six patients presenting with typical as well as atypical ocular manifestations between 1982 and 1994. Typically, the illness was accompanied by systemic symptoms. Each patient had audiovestibular involvement, that was the initial presentation in 4 cases. Vestibular dysfunction often preceded hearing loss. Five of our patients not only presented with keratitis but also demonstrated signs of ocular inflammation diagnosed as scleritis or episcleritis. In two patients these ocular symptoms were the first signs of Cogan's syndrome, recurred periodically and did not respond to corticosteroids. In 3 patients that were diagnosed early and treatment with corticosteroids was initiated early, hearing could be stabilized, in the remaining patients total bilateral deafness could not be prevented. The importance of being aware that vestibuloauditory dysfunction may occur in patients with ocular inflammation, and the fact that early immunotherapy may prevent the risk of deafness, has to be emphasized.

Audiovestibular involvement in systemic sclerosis.

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.

C 33 Audio vestibular involvement in Behçet's disease (BD)

C 33 Audio vestibular involvement in Behçet's disease (BD)

Hearing and Vestibular Disturbances in Behçet's Syndrome

In order to evaluate the prevalence of audiovestibular disturbances in Behçet's syndrome, we submitted 20 consecutive patients and 20 control subjects to detailed audiologic and vestibular examination in the last 3 years. A sensorineural hearing loss was found in 12 patients, 2 of whom revealed sudden deafness. Two other patients with neuro-Behçet's syndrome showed a vestibular function deficit, and 3 others exhibited altered caloric stimulation test results. Two of these last patients also revealed a simultaneous bilateral auditory deficit. HLA typing showed the presence of the B51 antigen in 10 of the 14 patients with ear involvement, while only 3 of the 6 patients without ear involvement were HLA-B51-positive. Results suggest that audiovestibular involvement is common in Behçet's syndrome: sudden deafness may be the first sign of ear disturbance; vestibular lesions may represent an early sign of neuro-Behçet's syndrome; and the HLA-B51 antigen is associated with ear involvement. Otoneurologic study can reveal hidden brain stem lesions in Behçet's patients during flare-ups of the disease, even without obvious signs of neurologic deficits.

Cogan's syndrome: audiovestibular involvement and prognosis in 18 patients.

The experience at the Mayo Clinic with 18 patients who had Cogan's syndrome is reviewed. Typically, the illness began with systemic symptoms. The ocular and audiovestibular symptoms included ocular discomfort and redness, photophobia, and fluctuating sensorineural deafness with imbalance. Each patient had interstitial keratitis; the ocular symptoms periodically recurred but responded to therapy. Thirteen patients who had fluctuating bilateral sensorineural deafness subsequently suffered total bilateral deafness. Two patients who are unilaterally deaf have good residual hearing. Two other patients regained hearing within normal limits after treatment with corticosteroids. No hearing symptoms were observed in one patient who had severe vertigo and whose condition is stable. Seventeen patients had vertigo: 11 have no labyrinthine function, 1 had absent responses on vestibular testing initially but currently demonstrates good responses, and the 5 others have remained stable with diminished vestibular function. If Cogan's syndrome is diagnosed early and treatment with corticosteroids is initiated promptly, hearing can be stabilized; otherwise, the prognosis for auditory function is poor.