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Cogan's syndrome: acute vestibular and auditory dysfunction with interstitial keratitis.

Two patients with Cogan's syndrome had acute bilateral hearing loss and vestibular dysfunction. Interstitial keratitis was discovered by slit-lamp examination. Both cases responded to corticosteroid treatment. Immediate recognition of this syndrome and the institution of high-dose corticosteroid therapy should lead to resolution of the hearing loss in patients with Cogan's syndrome.

Acute Disease↗

Autoimmune reactivity in Cogan's syndrome: a preliminary report.

Autoimmune inner ear disease was first described by McCabe in 1979. The diagnosis is supported by cell-mediated immune responses to inner ear membrane antigen stimulation. Cogan's syndrome consists of vestibuloauditory dysfunction, ocular inflammation, and nonreactive serologic tests for syphilis. The ocular disease can be controlled by steroids; unfortunately, some patients ultimately become totally deaf. An autoimmune etiology has long been suspected but only recently confirmed by McCabe. In our study two patients with Cogan's syndrome were studied for autoimmune inner ear disease with the use of both cellular and humoral immune tests. Results were compared to normal control subjects. On the basis of test results, preliminary conclusions were that (1) the vestibuloauditory symptoms of Cogan's syndrome are autoimmune in origin; (2) the autoimmune process is mediated through cellular rather than humoral (antibody) pathways; (3) systemic steroids may suppress positive test results; and (4) test results are more likely to be positive when symptoms are acute. Therapeutic implications are significant: the addition of cytotoxic drugs to steroids in selected cases may help prevent total deafness that otherwise might be inevitable.

Antibody Formation↗

[Weather status and incidence of idiopathic facial nerve paralyses, vestibular disorders, Menière's attacks and sudden deafness].

The authors studied a possible relationship between the weather and Bell's palsy, acute unilateral vestibular disorder, Menière's disease and sudden deafness. These diseases definitely do occur more often at times of low pressure. This is most convincing when a stable high pressure zone is followed by a strong low, thus creating comparatively great pressure differences. Bell's palsy, sudden deafness and Menière's disease occur more often under the influence of the warm side of the low. Such atmospheric conditions could not be confirmed for acute unilateral vestibular disorder.

Facial Paralysis↗

Primary intravestibular lipoma.

An intravestibular lipoma was discovered during destructive surgery for disabling vertigo in a case diagnosed as delayed endolymphatic hydrops. The author recommends that in cases that resemble Meniere's disease but have unusual symptoms, the existence of other lesions within the inner ear should be suspected.

Adult↗

[The problems of electronystagmography in the clinical diagnosis of vestibular diseases (author's transl)].

The aim of a documentation in electronystagmography, that is to record as complete and definite as possible the information of a nystagmus relative to all its distinctive marks, hitherto has been reached just as little as the attempt, to develop a fine-quantitative investigatory method suitable for standardization for medical practice. The sources of error caused by the equipment and of physiological nature within a recorded nystagmus trace are exposed and illustrated by respective examples. Outgoing from the theory of the rotatory nystagmus based on the rotation test of the human vestibular system the fundamentels are developed for a complete evaluation method of an electronystagmogram including the elimination of artefacts by the authors' own research work in this field. However the pointed out problems of the electronystagmography are not yet answered in the clinical diagnostic of vestibular diseases, the systematic examination of spontaneous nystagmus and provoked nystagmus still represents the most important, most profitable and well-grounded part of the vestibular investigation in the medical practice.

Diagnostic Errors↗

Central auditory and vestibular pathology.

MR is the radiologic examination of choice when a patient's symptoms indicate the presence of some retrolabyrinthine pathology. CT dynamic studies now permit both heart and brain assessment for poor blood circulation problems of the hindbrain.

Auditory Cortex↗

Vestibular toxicity of gentamicin. Incidence in patients receiving long-term hemodialysis therapy.

Twenty-three patients on long-term hemodialysis regimens who received gentamicin sulfate were reviewed retrospectively to assess the incidence of ototoxicity and to identify potential risk factors. Dosage of gentamicin sulfate was 1.0 to 1.5 mg/kg intravenously three times weekly. Serum gentamicin levels were monitored in 21 cases. Seven patients developed signs and symptoms of vestibular dysfunction. Statistically significant differences were found between the ototoxic and nonototoxic groups with respect to age (P less than .001), total dose (milligrams per kilogram) (P less than .001), and duration of therapy (P less than .001). The total dose per kilogram of body weight contributed most heavily to ototoxicity, and regression analysis suggests that the critical cumulative dose is about 17.5 mg/kg. The two groups did not differ with respect to mean peak and valley serum levels. We conclude that this population is at high risk of developing gentamicin-related vestibular dysfunction specifically when the cumulative dose exceeds 17.5 mg/kg.

Adult↗

Neuro-ophthalmologic findings in vestibulocerebellar ataxia.

A young adult who presents with periodic vertigo, diplopia, and tinnitus, later followed by progressive ataxia, may not have multiple sclerosis as might initially be suspected, but rather may represent a type of familial spinocerebellar degeneration previously described by Farmer and Mustian as "vestibulocerebellar ataxia." We recently encountered a patient who presented with these symptoms, and who was found to have downbeat nystagmus, ocular dysmetria, skew deviation, optokinetic dissociation, and a vertical gain bias on horizontal eye movements. Although family involvement was specifically denied by the patient, seven members of her family representing four generations were examined, and found to have similar findings. The importance of a careful neuro-ophthalmologic examination in each available family member of any patient presenting with this constellation of symptoms is emphasized. A discussion of the spinocerebellar degenerations, differential diagnosis, and literature review are included.

Adult↗