Audiovestibular Disorders Research Articles

聴平衡覚障害をきたした再発性多発軟骨炎の２症例

Relapsing polychondritis (RP) is a multi-systemic inflammatory autoimmune disease that affects components of cartilage such as collagens and mucopolysaccharide. The most common findings of RP are auricular and nasal chondritis, whereas audiovestibular disorders are rarely noticed at first. We report two cases with relapsing polychondritis who first complained of audiovestibular symptoms. The first patient was a 19-year-old man, who suffered from repeated dizziness, vertigo and bilateral sensorineural hearing loss. One year later, he also had dyspnea caused by chondritis of the respiratory tract and otitis media with effusion. Results of a caloric test and vestibular evoked myogenic potential (VEMP) to clicks showed no response on either side, whereas VEMP to galvanic stimulation showed normal response on both sides. The second patient was a 58-year-old man with repeated vertigo and dizziness. He also had progressive bilateral severe sensorineural hearing impairment. Although he had undergone intravenous steroid therapy, his hearing did not recover. The results of neuro-otological examinations of our patients suggested that recurrent vertigo and dizziness in the patient with RP were mainly of labyrinthine origin.

Immune-mediated audiovestibular disorders in the paediatric population: A review

Recent studies show that several audiovestibular pathologies in the paediatric population may be immune-mediated. This is even more probable if the pathology is associated with a coexisting systemic autoimmune disorder. At this time, however, the current literature is limited to a few case reports, and little is known with regard to prevalence, diagnosis, and management of immune-mediated inner-ear disorders in children. This review aims to shed some light on clinical presentation, diagnosis, and treatment of paediatric immune-mediated inner-ear disorders. Sudden and progressive sensorineural hearing loss is discussed, in addition to some of the systemic autoimmune disorders commonly associated with immune-mediated audiovestibular pathology such as Cogan's syndrome, systemic lupus erythematosus, Behçet's disease, Sjögren's syndrome, and juvenile idiopathic arthritis.

Audiovestibular Disorders in Patients With Ankylosing Spondylitis

Audiovestibular disorders have been described in several autoimmune diseases but have not been studied in patients with ankylosing spondylitis (AS). The aim of this study was to evaluate the audiovestibular function in patients with AS. We prospectively evaluated 22 consecutive patients with AS. Clinical, radiologic, and immunogenetic features were analyzed. All patients underwent a complete ear, nose, and throat physical examination and audiologic evaluation that included pure-tone audiometry thresholds at octave frequencies of 250 to 8000 Hz, impedance audiometry (tympanogram, static compliance, acoustic reflexes, and reflex decay), and speech audiometry. Thirty-one healthy volunteers and 26 patients with rheumatoid arthritis (RA) were included as controls. Patients with AS had a median age of 45.5 years (interquartile range [IQR] 38-54) and a median disease duration of 20 years (IQR 12.5-26.2). Otosclerosis was observed in 2 patients with AS (9.1%), 3 patients with RA (11.5%), but not in any healthy controls (P = not significant). Sensorineural hearing loss (SNHL) was observed in 13 patients with AS (59%), 17 patients with RA (65.4%), and 13 healthy controls (41.9%) without statistically significant differences. Because age can influence audiometric results, patients and controls were divided into 4 age groups. A higher frequency of SNHL was observed in patients with AS from 45 to 59 years compared with healthy controls and patients with RA (87% versus 20%, P = 0.03, versus 70%, P = not significant, respectively). Middle-aged patients with AS had a significantly higher frequency of SNHL compared with controls. SNHL should be considered as a possible complication of AS as well as of RA and other autoimmune diseases. Possible toxic effect of nonsteroidal antiinflammatory drugs should also be considered in such patients.

Audiovestibular and neuropsychological outcome of adults who had recovered from childhood bacterial meningitis

A sample of 22 subjects was studied from a population of adults who had suffered from bacterial meningitis in childhood. Audiovestibular, oculomotor and neuropsychological investigations were performed and quality of life was assessed. An age-matched control group of 20 subjects was recruited. In the meningitis group, nine subjects had abnormal pure tone audiograms. One was previously undiagnosed and a progression was found in four. There was an overrepresentation of subclinical vestibular pathology (6 out of 9 (67%)) in this group. Audiovestibular test results showed a peripheral pattern and oculomotor tests were normal. The quality of life scores of those with hearing loss were significantly higher than those in the control group. Neuropsychological tests of brain dysfunction were abnormal in six out of 22 (27%) who had recovered from meningitis. The prevalence of such dysfunctions was not related to audiovestibular disorder. The quality of life scores of those with brain dysfunctions were similar to those of the control group. The findings of reduced auditory memory and tone level perception in four out of 22 (18%), suggest that lesions of central auditory pathways may follow from bacterial meningitis. The results support the idea that inner ear damage is the major cause of hearing loss after bacterial meningitis. Despite the absence of brainstem involvement, central nervous system lesions with disturbed auditory processing and language functions can be of significance. The high frequency of discrete brain dysfunctions indicate that a thorough neuropsychological investigation is required after bacterial meningitis.

Hearing loss in the Sjögren syndrome.

Ear involvement is not unusual in autoimmune diseases, but few data on this problem in the Sjögren syndrome are available. To determine whether the incidence of hearing loss is increased in patients with the Sjögren syndrome and to determine what factors might be involved in the pathogenesis of Sjögren syndrome-related hearing loss. Cross-sectional study. Secondary referral center in Italy. 30 women with the Sjögren syndrome were evaluated for evidence of audiovestibular disorder. Their results were compared with those of 40 age-matched healthy women. Evaluation techniques included pure tone audiometry thresholds at octave frequencies of 125 Hz to 8000 Hz, tympanometry, and the stapedial reflex test in both ears. The presence of vasculitis, the Raynaud phenomenon, nervous system involvement, and serum anticardiolipin antibodies was recorded. 14 patients with the Sjögren syndrome (46% [95% CI, 28% to 66%]) had sensorineural hearing loss. Only one control (2.5% [CI, 0.06% to 13%]) had a similar hearing impairment (P < 0.001). Nine of the 14 patients who had the Sjögren syndrome and sensorineural hearing loss (64% [CI, 36% to 87%]) had anticardiolipin antibodies compared with only 3 controls (18% [CI, 4% to 45%]) (P = 0.02). The high prevalence of hearing loss in the Sjögren syndrome supports the value of doing audiometric studies before excluding cranial nerve involvement in this disease. The correlation of sensorineural hearing loss with anticardiolipin antibodies must be investigated further.

Retrospektive und prospektive Untersuchungen an Patienten mit Cogan-I-Syndrom

Since August 1987 we documented the course of three patients with Cogan-I-Syndrome (CS) (interstitial keratitis and audio vestibular disorder). The patients (2 female, 1 male) received oral protease therapy in addition to the corticosteroids. Under oral treatment with proteases the corticosteroid therapy could be discontinued. Regression of inflammatory reaction and improvement of hearing was encountered in all patients. In two cases alpha-1-antitrypsin (AAT) serum levels were profoundly reduced, reaching normal range during therapy. Concomitant to the raise of this parameter improvement of hearing and general status could be encountered. In all 3 cases pathological leukocytosis normalized under treatment with proteases. All patients had increased IgA-levels against varicella zoster virus (VZV) indicating a reactivation.

II: A review of clinical drug trials on betahistine in audio-vestibular disorders

II: A review of clinical drug trials on betahistine in audio-vestibular disorders

RELAPSING POLYCHONDRITIS

Relapsing polychondritis (R.P.) is a disorder of unknown cause characterized by the destruction of cartilage throughout body. A 39-year-old female who suffered from sudden onset of hearing loss in both ears and vertigo during the course of R.P. was reported. By the way, we measured the antibodies to the type II (cartilage) collagen in the serum of the patient by an emzyme immuno assay in combination with electrophoretic transfer of sodium dodecyl sulfate- polyacrylamide gel electrophoresis-resolved collagen to nitrocellulose sheets. In result, antibodies to type II collagen were found in the serum of the patient and no antibodies to other collagen types were found. We demonstrated the antibody activity to type II collagen in the serum of the patient was decreased after plasmapheresis which was done as a treatment.Audiovestibular disorders observed in the patient was discussed with relation to the underlying R.P.. The onset and course of this audiovestibular disorders resembled that of Meniere's disease. If the type II collagen exists in the subepithelial connective tissues of the endolymphatic duct, which Yoo speculated, there is probability that endolymphatic hydrops was produced by immunologic reaction in the same place. In short, we suggest a possible role for type II collagen autoimmunity in the etiology of audiovestibular disorders with R.P..

Vestibular Findings in Sensorineural Hearing Disorders: Results of Caloric, Oculomotor and Hearing Tests in 205 Patients with Unilateral Hearing Dysfunction

In 205 patients with unilateral hearing loss, 117 of cochlear and 88 of retrocochlear origin, thorough audiovestibular examinations were performed to establish the occurrence and severity of vestibular dysfunction. The results were also analysed with the aim of determining the relationships between the severity of the hearing loss, the etiology and the topical location of the hearing disorder, on the one hand, and vestibular dysfunction, recorded as reduced caloric sensitivity and occurrence of oculomotor disturbances, on the other. No correlation was found between severity of hearing loss and recordable vestibular dysfunction, in either the cochlear or retrocochlear group of patients, or in the etiological subgroups of these main groups. With respect to the relations between the topic locations of the lesions and the results of vestibular tests, distinct characteristics were observed. In the affected ears, totally extinguished caloric reactions were more than six times as frequent in the retrocochlear group as in the cochlear group and appeared as a specific but rather insensitive sign of acoustic neurinoma. Oculomotor disturbances were only exceptionally observed in patients with cochlear lesions, but were noted in nearly half of the retrocochlear group; when only those patients with lesions of the brain stem or cerebellum were considered, all were found to exhibit such disturbances. The relations between audiometric hearing test pattern, caloric sensitivity and oculomotor disturbances seem to form interesting diagnostic paradigms of great value for the topical localization of audiovestibular disorders.

Automatic analysis of electro-oculographic (EOG) recordings. Application to audio-vestibular diagnosis.

An automatic, computerized system for analysis of the ocular movements in nystagmus, slow pursuit and saccades is presented. The performance of the system has been tested in healthy subjects, as well as in patients with peripheral or brainstem audio-vestibular disorders. The automatized procedure enables an extended application of opto-motor tests in the clinical examination of patients with suspected retrocochlear and brainstem involvement.