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[Each one case of Vogt-Koyanagi-Harada disease with vestibular and cerebellar ataxia, and multiple cranial nerve palsies].

Two cases of Vogt-Koyanagi-Harada disease with rare neurological complications were reported, which are one case with vestibular and cerebellar ataxia, and one with multiple cranial nerve palsies. The first case, a 32-year-old man, was admitted to our ophthalmologic department because of visual impairment. Two weeks later, he complained of gait disturbance. Limb and truncal ataxia, vestibular dysfunction and perceptive deafness were observed. Ophthalmologic examination revealed typical findings of Vogt-Koyanagi-Harada disease. CSF showed elevated protein and mononuclear pleocytosis. After treatment with oral steroid therapy, vestibular and cerebellar ataxia, and visual impairment improved. The second case, a 22-years-old man, showed the VIIth, VIIIth, IXth, and Xth cranial nerve palsies with visual impairment, and he was diagnosed to have cranial nerve palsies associated with Vogt-Koyanagi-Harada disease. After steroid therapy, there was a marked clinical improvement. Vogt-Koyanagi-Harada disease is an autoimmune disease against the melanocytes and involving tissues containing melanocytes such as uvea, meningea, skin and inner ear, etc.. Neurological complications except for meningitis and perceptive deafness have been rarely reported in Japan. Related to the present cases, possible pathogenesis was discussed.

Adult↗

The furosemid test for Menière's disease.

In a previous report it was suggested that intravenous administration of furosemid followed by a comparative caloric test (the furosemid test) could be utilized for detection of endolymphatic hydrops (Kitahara et al., 1973). As a result of the further application of the furosemid test upon 161 patients with vertigo, positive results were observed in 80% of patients with typical Menière's disease, 6% of atypical Menière's disease, 42% with labyrinthine syphilis and 27% with sudden deafness. In patients with labyrinthitis, results were negative. It is thus concluded that the furosemid test provides positive evidence for determining the etiology of vertigo.

Audiometry↗

Rotatory test in otoneurology.

A series of rotatory tests are described, by which it is possible to activate the ocolomotor system by visual as well as by vestibular stimuli, either separately or in combination. The visual-oculomotor system was studied by means of rotation with 10 degrees constant speed for 10 s in either direction. Pursuit movements and saccades were also studied. The vestibulooculomotor system was studied by acceleration behind closed eyes with 10 degrees/s2 up to 100 degrees, immediately followed by a similar deceleration. Normal and pathological cases are presented. A combined visual-vestibular stimulation was performed with the same kind of acceleration-deceleration but with eyes open. Acceleration demonstrated the summation effect of visual and vestibular input to the vestibular nuclei. The deceleration revealed the cerebellar ability to suppress the vestibular output. Normal and pathological cases are demonstrated. Finally, a combined rotation was introduced, informing us about the vestibular reaction, the visual-vestibular summation, the optokinetic reactions, and the cerebello-vestibular suppression. Normal and pathological cases are described.

Acceleration↗

Presentation of a posturographic test with loading of the proprioceptive system.

Postural control is maintained by sensory feedback from visual, vestibular and somatosensory receptors. Recently several methods for evaluating postural control have been devised, utilizing an imposed perturbation. Most of these methods use stimuli which simultaneously affect more than one of the sensory feedback loops. In the present paper a posturographic technique is presented with specific loading of the proprioceptive system, using a computer controlled vibratory stimulus and computerized analysis of the results.

Central Nervous System Diseases↗

Eighteen years experience in stapedectomy. The case for the small fenestra operation.

The postoperative findings in almost 800 stapedectomized ears were analyzed to evaluate the proposition that complication rates in stapedectomy were affected by the size of footplate fenestration. It was concluded that small fenestra stapedectomy (diameter 0.4 mm) provided similar hearing gains to those achieved with standard techniques, that articulation problems occurred to a similar extent as with wire loop prostheses, and that there was a significantly lower incidence of a) fistual and b) immediate and delayed severe sensorineural hearing loss than with any other technique. There was also significantly less deterioration in bone conduction thresholds at 4 kHz after three years postoperatively. The incidence of severe immediate sensorineural loss in large fenestra stapedectomy (half or more of footplate removed) was significantly influenced by factors such as age, preoperative bone conduction thresholds and oval window pathology. A retrospective analysis provided no information which might predict oval window pathology. Additional information gained from the analysis indicated that with all types of stapedectomy, bone conduction did not deteriorate significantly more rapidly in the operated as compared to the unoperated ear, whereas in unoperated ears, deterioration in bone conduction was significantly greater in ears with mixed hearing losses than when the loss was purely sensorineural. It was concluded that small fenestra stapedectomy was currently the operation of choice because with it, the threat of cochlear dysfunction both immediately, and in the long term, was significantly less.

Bone Conduction↗

The impulsive test in man.

The impulsive test is brief and comfortable to the patient. The SVM is the best discriminator of the variables examined. By using percent differences between CW and CCW responses in the same subject at the same stimulus magnitude, we can define a range for the normal population and indicate patients outside that normal range. The impulsive test gives directional information in patients with unilateral lesions. In patients with severe bilateral loss of vestibular function, measurable responses are still obtainable; while in patients with primary cerebellar disease, responses are increased. The test may also be useful in giving anatomic information about posterior fossa lesions.

Brain Neoplasms↗

Broad-frequency rotatory testing.

The method of broad-frequency-band rotatory testing with results from normal subjects and patients with peripheral uni- and bilateral loss as well as central vestibular disorders are reviewed. The following conclusions are drawn: adequate testing of the vestibulo-ocular reflex including side detection of unilateral loss in light or in darkness can only be done with either random or high-frequency (2.5-3 Hz) sinusoidal stimulation. Measurements of compensatory eye movements at lower frequencies where vestibular and non-vestibular signals interact are of interest for central vestibular diagnosis. A decreased ability to suppress vestibular nystagmus is not an uncommon finding in patients with large acoustic neuromas or pathology in the brainstem or cerebellum.

Central Nervous System Diseases↗

Treatment of streptomycin-susceptible and streptomycin-resistant enterococcal endocarditis.

Fifty-six patients with enterococcal endocarditis received 4 weeks of antimicrobial therapy with penicillin G and streptomycin (36 patients) or, if infections were streptomycin resistant, penicillin and gentamicin (20 patients). Compared with patients who had symptoms for less than 3 months, patients with symptoms for more than 3 months had a higher relapse rate (0% versus 44%; p less than 0.001) and mortality (2.5% versus 25%; p less than 0.001). Patients with mitral valve endocarditis had a significantly higher relapse rate (25%) than patients with aortic valve infections (0%) (p less than 0.01). Gentamicin-associated nephrotoxicity was more frequent (p less than 0.001) among patients treated with greater than 3 mg/kg d of gentamicin than among those treated with 3 mg or less (100% versus 20%). Relapse and mortality rates did not differ significantly between patients treated with low-dose or high-dose gentamicin regimens. Patients who have had symptoms of enterococcal endocarditis for more than 3 months or patients with mitral valve infection should receive at least 6 weeks of antimicrobial therapy, but patients without these high-risk factors can be treated for 4 weeks.

Adult↗