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[The saccular function in Meniere's disease].

OBJECTIVE: To record and analyze the vestibular evoked myogenic potentials (VEMP) in Meniere's disease. METHOD: VEMP in 15 patients diagnosed as Meniere's disease were investigated and made out different kinds of manifestation of VEMP. The results of VEMP were compared with these caloric test. RESULT: The wave of p13 and n23 appeared 100% in normal subjects, but only appeared 30% in Meniere's disease. There were 3 kinds of manifestation of VEMP-normal response, bilateral no response and lower amplitude in hydrops side. CONCLUSION: Membranous hydrops in Meniere's disease may damage the saccular function and VEMP test may be helpful for diagnosis of Meniere's disease.

Adolescent↗

[Inner ear physiological function and pathological morphology in the offsprings of sensorineural hearing loss female guinea pigs induced by homogeneous inner ear antigens immunizing].

OBJECTIVE: To explore if the autoimmune of anti-labyrinth tissues acts as one of pathogenic cause by observing the inner ear physiological functions and pathological morphology changes of offspring of autoimmune sensorineural hearing loss (ASHL) female guinea pig. METHODS: The pregnant guinea pigs were immunized with homogeneous inner ear antigens (HIEAg), then, the hearing function were measured with EcochG [inspecting items including acoustic nerve compound action potential (cAP), summation potential (-SP) and cochlear microphone potential (CM)], while the vestibular function were measured with electronystagmography (inspecting items including spontaneous nystagmus and caloric test), inner ear Celloidin section with haematoxylin-eosin dyeing and being inspected under light microscope. The special antibodies in serum and special lymphocyte immune reaction were measured with ELISA and 3H-TdR intermingling lymphocyte transform test in all female guinea pigs and their offspring guinea pigs. RESULTS: In 7 offspring guinea pigs, 3 animals appeared sensorineural hearing loss. Immuno-inflammation pathologic changes happened in the labyrinth (including the number of bipolar cells reduced and some kind of inflammatory cells infiltrated in spiral ganglion and endolymphatic hydrops et al.), and rise of special antibodies against HIEAg in serum. There were no any obvious abnormity found in non-ASHL pregnant and normal contrastive pregnant guinea pigs and their offspring. CONCLUSIONS: In this study, some of ASHL female guinea pig's offspring demonstrated different grades of sensorineural hearing loss and inner ear inflammation, and the special humoral and cellular immune reaction against HIEAg, which could be induced by autoimmune inflammation against inner ear tissues antigens with special antibodies (maybe including special cellular immune reaction) from matrix through placental barrier. This result suggests that the factor of autoimmune against inner ear tissue antigens may be one of pathogenic causes inducing non-heritage congenital sensorineural hearing loss.

Animals↗

Assessment of vertigo.

Vertigo is defined as a subjective sense of imbalance and includes unsteadiness as well as a sensation of rotation. Vertiginous symptoms may arise from defects in the labyrinth, the visual system, or the central nervous system, although in most patients presenting with balance problems, the lesion lies within the labyrinth or its central connections. The most important step in the diagnosis is an unhurried and detailed history, bearing in mind that many patients will have difficulty in describing their symptoms. A detailed neurological examination is usually unnecessary but one must examine the ears, cranial nerves, cerebellum, and balance function, and look for the presence of nystagmus. The common error of carrying out investigations in place of a detailed history is to be avoided. In many cases, investigations are not required at all, although it is the author's practice to do a routine pure tone audiogram with basic assessment of speech discrimination. There is no indication for routine caloric testing, imaging or blood analysis, each of which should be carried out only when there are specific indications. In the routine clinical care of patients, it is only in exceptional situations that specialised vestibular testing is required, although these are often necessary in research projects.

Audiometry↗

Clinical examination of labyrinthine-defective patients out of the vertigo attack: sensitivity and specificity of three low-cost methods.

Many reports have appeared in the medical literature concerning the clinical examination at the bedside of patients with vertigo and, even if few controversial opinions exist, the observation of one or more kinds of nystagmus is generally regarded as suggesting an organic aetiology. So far, the presence of nystagmus has been generally considered to be crucially important for clinicians who are daily asked to differentiate between an "organic" cause of vertigo (for example, a labyrinthine dysfunction) and a "non-organic" cause of vertigo, such as a panic disorder. Albeit, it should not be forgotten that the central nervous system is able to resolve the asymmetry of vestibulo-ocular reflexes, due to a peripheral vestibular failure, by means of compensatory mechanisms so that nystagmus is rapidly abolished after the acute attack of vertigo. In addition, visual fixation elicits sub-cortical inhibitory pathways to the vestibular nuclei so that spontaneous nystagmus is remarkably reduced by light. In order to more easily detect nystagmus, attempts have been made to minimize the interference of visual fixation by means of positive lenses (Frenzel's glasses) and light occluding masks with infrared cameras (videonystagmoscopy) which have in part replaced direct observation of the patient's eyes, albeit no systematic validation of the advantages has been reported yet. To investigate the usefulness of these 3 low-cost methods to detect nystagmus, 528 outpatients presenting peripheral vestibular hypofunction, diagnosed by a complete audiological and vestibular examination, including caloric tests, were enrolled in the present study, while 133 subjects with normal vestibular function acted as a control group. All patients and control subjects underwent a standardized clinical examination based on search for spontaneous, positioning and head-shaking nystagmus detected by direct observation of patient's eyes, Frenzel's glasses and videonystagmoscopy. Specificity of the three techniques were 35.6, 43.7 and 91.6, whilst sensitivity was 88.7, 88.7 and 84.2, respectively. Finally, discriminant analysis based on the presence/absence of at least one kind of nystagmus was computed for each technique and showed that videonystagmoscopy allowed the examiner to correctly classify both pathological and normal subjects more frequently (> 77% of cases) than the other two methods (about 50%). It is concluded that only videonystagmoscopy is an acceptable technique for screening a labyrinth defect in a population of outpatients with vertigo.

Adolescent↗

[Vestibular cochlear disturbances in multiple sclerosis].

To specify the character of vestibular cochlear disturbances in multiple sclerosis (MS) and mechanisms of their formation, the objective quantitative evaluation of experimental caloric nystagmus using the computer electronystagmographic method and a comparison of hearing impairment with asymmetry of vestibular reactions were conducted in 48 patients with MS. The peculiarities of vestibular disturbance found in the study suggest a predominance of central vestibular syndrome with both subtentorial and supratentorial traits. The influence of the heat stimulus of vestibular analyzer (hot water caloric test) in multiple sclerosis revealed during computer electronystagmography may have diagnostic value.

Adult↗

[False-positive diagnostic findings in acoustic neurinomas].

In the period from 1976 to 1990 Tos and Thomsen operated on 520 patients with acoustic neuromas using the translabyrinthine approach and five other patients were operated on via middle fossa approach. The diagnostic work up in all patients included: pure tone audiometry, tympanogram with acoustic reflexes, caloric test and brainstem audiometry. Since the late seventies, CT became the radiological investigation of choice to visualize the tumor. The first generation of CT failed to reveal tumors less than 1.5 cm in the extrameatal diameter, and pantopaque cisternography was necessary in some cases. The following generation of CT did not always reveal small intrameatal tumors, and false-negative results were reported. The incidence of false-positive CT findings in our series is calculated to be 0.6%. Three patients were operated on on account of false-positive CT. Peroperatively, adhesions in and around the internal porus were found in two cases while no pathology was found in the third case. Postoperatively, anacusis was observed in two cases. This could have been avoided if magnetic resonance imaging (MRI) with gadolinium contrast had been performed. In our opinion, MRI should be considered before a definitive surgical procedure is undertaken. Until MRI becomes more widely available, intravenous contrast-enhanced tomography followed by air cisternography is recommended in the diagnosis of small acoustic neuromas.

Adult↗

Audiometric and vestibular abnormalities in macular degeneration.

Audiometric (pure tone audiometry and tone decay) and vestibular examinations (bilateral caloric test and electronystagmography) were carried out in 10 cases of macular degeneration of different types and 10 normal individuals as control. Out of 10 cases of macular degeneration of various types, 3 (30%) showed hearing impairment, while on vestibular examination 3 (30%) also had vestibular abnormalities. Only one case was associated with both audiometric as well as vestibular abnormalities. In the control group none of the individual showed either audiometric or vestibular changes.

Adolescent↗

[Electrophysiological studies of a case of startle disease].

A 53-year-old female with startle disease (major form) was reported. An abnormal startle response was the most prominent clinical feature. Physical examination revealed left lateral gaze palsy and left extensor plantar response. The caloric test evoked no responses bilaterally. Blood examinations including lysozomal enzymes and radiological examinations including MRI of the brain were all normal. A pathological startle reflex was elicited by the tap on the upper lip, causing the marked extension of the head with the elbow, hip, and knee joints slightly flexing. The earliest reflex activity in a surface-EMG study was recorded in the masseter muscle and the reflex then spread down the brain stem and the spinal cord. The duration of the discharge varied from 16 to 30 ms. The onset latencies of these responses from the tap were 11.2 ms, 12.7 ms, 14.5 ms, 25.7 ms, 38.5 ms, and 47.5 ms in the masseter, sternocleidomastoid, posterior-neck, biceps brachii, quadriceps femoris, and tibialis anterior muscle, respectively. An averaged electroencephalogram triggered by the taps showed no abnormal EEG activity preceding the pathological startle response, although a negative peak, which was thought as a normal early component of the trigeminal somatosensory evoked potentials, was followed by the reflex. High amplitude SEPs and long loop reflexes were observed following stimulation of the posterior tibial nerve but not of the median nerve. Blink reflexes and auditory evoked potentials were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Electrophysiology↗

Diagnosis of unilateral otolith hypofunction.

Asymmetric vestibular function is the basis of vertigo. Whereas caloric tests can identify unilateral peripheral loss or impairment of horizontal semicircular canal function reasonably accurately, there is as yet no single accepted test of unilateral otolith hypofunction. In some advanced vestibular laboratories around the world, new and perhaps diagnostically useful tests of otolith function are being devised. The physiologic basis and the present clinical applications of these tests are reviewed.

Animals↗

[Vestibular function in severely retarded children in relation to their motor disabilities].

We examined vestibular functions of thirty-one severely retarded children with caloric test and rotation nystagmus test, in combination with auditory brainstem response (ABR) recordings and measurement of brainstem width on cranial CT scans. The laterality of vestibular response was over 20% in most of the bedridden cases and was under 20% who could move. Abnormal findings in ABR had no relationship with the degree of motor disabilities, but decreased width of brainstem on CT scan showed some relationship with the degree of motor disabilities. We conclude that vestibular function tests are useful to predict future motor development in severely retarded children.

Adolescent↗

[Benign paroxysmal torticollis in infancy: case report].

Since Snyder reported first twelve cases of benign paroxysmal torticollis in infancy (BPT) in 1969, about 30 cases have been reported mainly in the North America and Europe, but not in Japan. The authors experienced such a case of one-year-old boy. The attack occurred at the age of 2 months for the first time and continued to repeat every 2 weeks periodically. The head tiltings were always noticed when he woke up in the morning and lasted for about 5 to 6 days, but they disappeared during sleep. He tilted his head to the lateral side more often to the left than to the right. There were associated symptoms and signs including irritability, vomiting, gait disturbance, and abnormal truncal posture during the attacks. He did not demonstrate any neurological abnormalities between the attacks. The study of cerebrospinal fluid, caloric test, computed tomography, electroencephalography and brainstem auditory evoked responses were also normal. The frequency of the attacks gradually decreased after the age of 2 years and disappeared at the age of 3 years, and he has not complained of severe headache or vertigo up to 6 years of age. This case is reported to emphasize that BPT has to be included in the differential diagnosis of acquired torticollis. And it is required to accumulate such cases to elucidate its etiological factors.

Diagnosis, Differential↗

[A case of ischemic disturbance of inner ear].

We reported a 51-year-old male with ischemic disturbance of right inner ear resembling Meniere's disease. The patient had a sudden-onset episode of vertigo, right severe hearing disturbance, nausea, vomiting and gait disturbance. Two days after, he had hypersomnia, vertical gaze palsy, double vision, left Horner's sign, and sensory disturbance of pain and temperature of right half body involving face. Brain MRI disclosed high intensity area in T2-weighted image and proton density in bilateral paramedian thalamo-mesencephalic region and right cerebellum (area of the anterior inferior cerebellar artery). Cerebral angiography showed 90% or more stenosis of the right vertebral artery, 50% stenosis of the left vertebral artery before the posterior inferior cerebellar artery (PICA), and 60% stenosis of distal portion of the basilar artery. Furthermore, stem portion of the posterior cerebral artery, and the right anterior cerebellar artery and the left vertebral artery after the PICA were absent or occluded. Right deafness was evaluated to be Jerger type II, namely disturbance of inner ear. Caloric tests showed no response, and right auditory brainstem response showed no waves. Main cause of this vertigo and right deafness was considered to be disturbance of inner ear due to ischemia of right labyrinthine artery, though this patient was not a typical case of the anterior cerebellar artery syndrome. Ischemic disturbances of inner ear have been reported only in patients with the anterior cerebellar artery syndrome, therefore this patient who had only acute ischemic disturbance of inner ear and did not have disturbance of caudo-lateral portion of the pons was considered to be very rare.

Cerebral Angiography↗

[Spontaneous and experimental vestibular activity following total removal of neurinomas of the auditory nerve].

Spontaneous and experimental vestibular activity in 84 patients after total resection of neurinomas of the acoustic nerve is analysed. In the immediate postoperative period the frequency of marked manifestations of spontaneous vestibular activity reduced sharply, but spontaneous nystagmus persisted in 97.5% of patients. In some patients spontaneous nystagmus to the side from which the tumor had been removed become more conspicuous. In the remote postoperative period spontaneous nystagmus was unilateral in 45.5% of patients, bilateral in 40.3%, and not recorded at all in 14.2% of patients. In the early postoperative period vestibular hyperreflexia was recorded in 93.7% of cases, in the remote period it was found in 76.6% of cases. Data obtained by comparing the results of caloric tests with cold and hot water in these patients are also analysed.

Adolescent↗

Otoneurophysiologic diagnosis of vascular disturbances in the central nervous system.

103 consecutive patients attending the Visby County Hospital due to vertiginous symptoms were included in the present study. All patients underwent conventional audiometric investigation, brainstem audiometry (ABR), electronystagmography including spontaneous, -gaze-, and positional nystagmus in darkness and under fixation, caloric tests with and without ocular fixation, pendular smooth pursuit test and saccade analyses. Optokinetic nystagmus-test and visual evoked potential recording were performed in some of the patients. Half of the patients underwent CT brain-scan. Abnormal otoneurophysiologic findings suggesting a central origin (pons, mesencephalon, cerebellum) was noted in 55% of the cases. CT brainscan displayed acoustic neuromas in three patients and a cerebellar affection in three patients. Otherwise the CT-scan was normal in the examined patients. In the present investigation the most sensitive tests for diagnosing disorders of central origin resulting in vertiginous symptoms were found to be the brainstem audiometry and smooth pursuit testing. In only two patients the results of the otoneurophysiologic examinations was found to be normal despite a convincing history suggesting a central nervous system disorder.

Adolescent↗

The investigation of unilateral sensori-neural hearing loss.

The site of the lesion in unilateral sensori-neural hearing loss (SNHL) may be confined to the receptor organ (labyrinthine) or may involve the auditory nerve including its central connections (retrolabyrinthine). The primary objective of the investigation of such a loss would be to define the probable site of the lesion. The protocol that we have found most productive and cost effective is as follows: 1. Audiometric evaluation consisting of a well masked pure tone audiogram, speech discrimination score and immitance studies. 2. Vestibular evaluation using photo-electric nystagmography and the Torok Monothermal Differential Caloric Test. Imaging studies of the temporal bone and brain are obtained on the basis of these tests. If the findings from auditory and vestibular tests are equivocal the patient is advised auditory brain stem response (ABR) testing. In our practice serologic tests for syphilis are obtained if there is the slightest suspicion of exposure. In this paper we report 2 cases which illustrate the value of our protocol and reliability of our technique.

Adult↗

[A family of von Recklinghausen's neurofibromatosis complicated by mononeuritis multiplex, bilateral acoustic neurinomas, and falx and spinal meningiomas].

A family of von Recklinghausen's disease complicated by multiple diverse primary brain tumors was reported. Case 1. The proband, born in 1923, was admitted to the Nagasaki University Hospital on March 11, 1974, for evaluation of headache and hearing loss. Neurological examination disclosed: decreased visual acuity on the right: bilateral choked discs; anisocoria, right pupil wider than left; right blepharoptosis; artificially fixed right eye; right facial palsy; markedly impaired hearing with negative vestibular responses to caloric test; paralysis of the right soft palate and vocal cord; atrophy of the right side of the tongue; right claw hand with positive Froment's sign; left drop foot; loss of deep reflexes on the left arm and legs; positive Babinski on the right. Nerve conduction studies revealed failure to evoke muscle action potential in response to electric nerve stimulation on the left ulnar and right superficial peroneal nerves. Needle electromyography showed no motor unit potentials in the left first dorsal interosseus and right anterior tibial muscles. Sensory nerve action potentials could not be evoked on any nerves tested. X-ray films showed enlargement of the internal auditory passages, falx tumor on brain scan and carotid angiography, and spinal tumor on myelography. At craniotomy, a 7 X 5.5 X 4 cm falx meningioma was removed. At suboccipital craniotomy performed five weeks later, right acoustic neurinoma measuring 1.5 cm in diameter was removed. Case 2. This relative, born in 1945, was the son of the proband. A spinal meningioma at C 7-T 1 was removed in 1957. However, he could not walk after operation and died of pneumonia two years later.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Vestibular results before and after neurectomy of the vestibular nerve in cases of vertigo resistant to medical treatment (author's transl)].

25 patients suffering from vertigo due to peripheral-vestibular disorders resistant to medical treatment underwent neurectomy of the vestibular nerve. Präoperatively 24 showed a pathological result in the neurotological examination. In 83% of the cases an abnormal caloric reaction could be seen in the frequency-calorigram, which graphically represents the caloric test. The postoperative objective vestibular results always correlated with the subjective feeling of vertigo. We could see a decrease and finally a disappearance of the horizontal unidirectional spontaneous nystagmus. It is of value to examine the gaze nystagmus in none directions of gaze. In the positiogram, a quantitative scheme of the positional test, one could recongize a decrease of the nystagmus intensity in the follow-up examination. The same was found by testing the vestibulo-spinal reactions. Although the neurectomy of the vestibular nerve involves a serious operation it is nevertheless the first choice of treatment. 96% of the cases are free from vertigo. Postoperative there is a very low complication quota.

Adult↗

[Cerebral disturbances following isolated whiplash injury (author's transl)].

During the last decades, possible sequelae of so-called whiplash-injuries have been intensively explored and described by traumatologists and orthopedists. In regard of the neurological symptoms radicular lesions, impairments of the autonomic nervous system at the neck and spinal cord injuries are well known and accepted. Symptoms of cerebral disturbances (e.g. headache, dizziness, disability of concentrating, abnormal irritability and fatigability) often still are considered as "psychoneurotic". In contrast to this opinion, some experimental and clinical neurophysiological research has shown in the meantime, that there may be cerebral damage even in the presence of a normal clinical examination. At first we want to give a review of this findings, then we derive as a consequence for the clinical practice, that in the future all patients should not only be examined neurologically, but the following investigations should also be done: electroencephalography (EEG), otorhinolaryngological examination (with caloric testing of the labyrinth function) and if possible electronystagmography (ENG) and auditory evoked brainstem potentials (AEP).

Brain Injuries↗