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Vestibulo-ocular arreflexia in families with spinocerebellar ataxia type 3 (Machado-Joseph disease).

OBJECTIVE: To identify the presence of vestibulo-ocular arreflexia in patients with Machado-Joseph disease (MJD), which can easily be diagnosed at the bedside. METHODS: Seven patients with MJD from five unrelated families and 11 patients with sporadic or hereditary cerebellar ataxia other than MJD underwent a detailed neuro-otological and oculomotor examination. Six MJD and five non-MJD patients also underwent electro-oculographic recordings and caloric tests. RESULTS: Gaze evoked nystagmus, smooth pursuit, and saccade abnormalities were found in both MJD and non-MJD patients. However, in all seven MJD patients but in none of the non-MJD patients, sudden passively induced head thrust to both sides elicited pathological corrective catch-up saccades, indicating bilateral loss of the horizontal vestibulo-ocular reflex. This was further confirmed in six MJD patients who had absent vestibular response to both a standard caloric test and ice water ear irrigation. Nystagmus was induced by standard caloric irrigation in all non-MJD patients examined. There was no correlation between the loss of vestibular function and the severity of cerebellar impairment. CONCLUSIONS: The presence of vestibulo-ocular arreflexia, as measured by the head thrust test in a patient with dominant cerebellar ataxia, strongly suggests the diagnosis of MJD.

Adult↗

[Curative effect of transtympanic perfusion of gentamicin on unilateral Ménière's disease].

OBJECTIVE: To investigate the curative effect of transtympanic perfusion of gentamicin on unilateral Ménière's disease. METHODS: Twenty-three patients with unilateral Ménière's disease, 14 males and 9 females, aged 24 - 61, all with vertigo, tinnitus and ear fullness sensation to different degrees, were treated with transtympanic perfusion of gentamicin via the tympanostomy tube, 12 mg per 3 hours and 5 times a day. Pure tone audiometry and caloric test were performed every day before treatment. When destruction type of nystagmus was observed by Frenzel's glasses and the treated ear showed no response to caloric test, the treatment was ceased. After the treatment the patients were followed up for 9 - 11 years (10.2 years on average). RESULTS: The average amount of gentamicin used was 216 mg. The treatment lasted for 1.5 to 5 days (3.6 days on average). Vertigo disappeared in 21 patients (91%) and remained unchanged in 2. Hearing was improved or remained unchanged in 17 patients (73%) and became worse in 6. Tinnitus decreased or disappeared in 15 patients (66%), and increased in 2 patients. Ear fullness sensation decreased or disappeared in 21 patients (91%). and remained unchanged in 2. Twenty-one patients were completely recovered and returned to work (91%). CONCLUSION: Relieving intractable vertigo and preserving hearing, transtympanic perfusion of gentamicin is effective on unilateral Ménière's disease.

Adult↗

[Pupillometry in Menière's disease].

The autonomic dysfunction is one of the etiopathogenetic hypothesis of Ménière's disease. We have already described the presence of an anomalous pupillary contraction, induced by methacholine, during the attack stage of Ménière's disease, without cardio-vascular reflexes involving. We have now employed, in a larger number of cases, a new digital equipment with infrared lighting system, built by us. The pupillary area was measured in darkness by a personal computer in basal condition (time 0) and 10, 20, 30, 45, 60 minutes after methacholine instillation. We have observed 16 cases of Ménière's disease (during attack stage and free stage), 23 cases of other vertiginous disorders (during attack stage and free strage), 10 normal subjects (5 cases of these also during a caloric test). The pupillary contraction was always highest at 30 minutes after methacholine administration. Normal subjects didn't show anisocoria and the contraction was weak and symmetric. Caloric test modified only a little the pupillary response. We observed the same results in other vertiginous disorders. During free stage of Ménière's disease there was little basal anisocoria but the contraction was normal and symmetric. During attack stage of Ménière's disease the contraction was much more evident and higher on the affected side. Ménière's disease crisis is characterized by cholinergic pupillary hyperreactivity, that is not caused by labyrinthic reflexes. We suppose this autonomic dysfunction of central origin.

Autonomic Nervous System Diseases↗

[Clinical research of vestibular autorotation test for patients with vertebrobasilar insufficiency].

OBJECTIVE: To explore the diagnostic values of vestibular autorotation test (VAT) for patients with vertebrobasilar insufficiency (VBI). METHODS: VAT and videonystagmography ( VNG) were performed on 73 patients with VBI and 48 patients with peripheral vestibular lesions (contrast group). Parameters analyzed included Gain, phase and asymmetry of VAT, as well as the canal paresis (CP) of caloric test and results of optokinetic-pursuit tests in VNG. Positive result of the test could be defined if anyone of the parameters was abnormal. RESULTS: For VAT test, Gain was enhanced in VBI group and was reduced in contrast group. In VBI group and contrast group, Gain enhanced showed in 47 (64.4%) cases and 5 (10.4%) cases, respectively (chi2 = 31.19, P < 0.01). Simultaneity, Gain reduced in 11 cases (15.5%) and 22 cases (45.8%), respectively (chi2 = 13.82, P < 0.01). But there was no statistics significant for results of the parameters of phase, asymmetry and integration between two groups. For VNG test, results with optokinetic-pursuit tests were more abnormal in VBI group than that in contrast group, which showed central lesions characteristics. Forty-four cases (60.3%) in VBI group and 10 cases (20.8%) in control group showed central lesions results with optokinetic-pursuit tests and visual fixation test (chi2 = 15.89, P < 0.01). Unilateral or bilateral CP showed in 33 cases (68.6%) in control group and 51 cases (69.9%) in VBI group with caloric test. CONCLUSIONS: Gain of VAT is mostly enhanced in VBI group, and Gain as a main characteristic is reduced in patients with peripheral vestibular lesions. The Gain parameter is availability for assessing characteristics of vestibular lesions. Phase and asymmetry can be used to assess the vestibular function but can not indicate the characteristics of vestibular lesions.

Adolescent↗

Edaravone protects the vestibular periphery from free radical-induced toxicity in response to perilymphatic application of (+/-)-alpha-amino-3-hydroxy-5-methyl-isoxazole-4-propionic acid.

Intracochlear infusion of (+/-)-alpha-amino-3-hydroxy-5-methyl-isoxazole-4-propionic acid (AMPA) was performed with a syringe pump in guinea pigs, and peripheral vestibular dysfunction was induced. Animals were administered edaravone systemically or topically. In the systemic application group, animals were administered edaravone once a day for 7 days after AMPA infusion. In the topical application group, edaravone-soaked gelfoam was placed on the round window membrane just after, 12 h after or 24 h after AMPA infusion. Spontaneous nystagmus was observed after AMPA infusion. Immunohistochemistry for 4-hydroxy-2-nonenal (4-HNE), a marker of free radical-induced lipid peroxidation, was performed 24 h after AMPA infusion. In addition, caloric tests were performed to evaluate vestibular function 1 week after AMPA infusion. Animals in both groups showed decreased spontaneous nystagmus, but results were not significant. Animals treated topically with edaravone within 12 h of AMPA infusion showed normal morphology of the ampullar sensory epithelia of the lateral semicircular canals and showed a good response to the caloric tests. 4-HNE immunoreactivity in the sensory epithelia was very low in these animals. In contrast, untreated animals and animals treated with edaravone systemically or topically 24 h after AMPA infusion showed morphologic hair cell damage, reduced caloric response and remarkable 4-HNE immunoreactivity in the sensory epithelia. These results indicate that topical application of edaravone within 12 h after damage protects the vestibular periphery from free radical-induced toxicity in response to intracochlear infusion of AMPA.

Aldehydes↗

Three-dimensional imaging of the inner ear by volume-rendered reconstructions of magnetic resonance data.

OBJECTIVE: To evaluate 3-dimensional inner ear visualization by volume rendering of high-resolution magnetic resonance data in patients with clinically suspected inner ear abnormality. DESIGN: Prospective comparative study of different postprocessing techniques, based on blinded film readings. SETTING: Tertiary referral hospital. SUBJECTS: Fifty patients (17 females and 33 males) aged 1 to 77 years (average age, 42 years) with sensorineural hearing loss, vertigo, and/or tinnitus. INTERVENTION: Postprocessing of magnetic resonance data to inner ear reconstructions by the use of volume rendering as well as maximum-intensity projection; caloric testing by electronystagmography. MAIN OUTCOME MEASURES: Film was read blindly by 4 radiologists using a 5-point parameter scale for image quality and diagnostic value. The assessibility of inner ear subsegments was evaluated. The specificity of volume-rendered reconstructions for detecting semicircular canal obliterations was assessed in a subgroup of 9 patients by caloric testing. The time required for data postprocessing as well as film reading was recorded by means of a stopwatch. RESULTS: Volume-rendered inner ear reconstructions were superior in image quality (P<.001), diagnostic value (P<.001), subsegment inner ear assessment (P<.01 to P<.001), and film reading time (P<.001) compared with maximum-intensity projections. The data postprocessing time was comparable for both techniques. Caloric weakness was noted in all patients assessed by electronystagmography. CONCLUSION: Volume rendering is the postprocessing technique of choice for 3-dimensional inner ear visualization, performing better than maximum-intensity projections with respect to various parameters.

Adolescent↗

Smooth pursuit neck torsion test: a specific test for cervical dizziness.

OBJECTIVE: This study aimed to determine how the smooth pursuit neck torsion (SPNT) test is affected by various diseases associated with disturbances in balance and arising in the neck, in the posterior intracranial fossa, and in the labyrinth in patients having such conditions, and to compare the findings with those in healthy subjects. STUDY DESIGN: This study was a consecutive, prospective, double-blind clinical study. SETTING: This study was conducted with ambulatory patients in a tertiary referral center (i.e., a county hospital). PATIENTS: Studied were 75 patients with whiplash-associated disorders (WAD) of at least grade II, according to the Quebec classification, all of whom had been injured in car accidents. Of these, 50 patients reported dizziness and 25 did not. CONTROL SUBJECTS: Twenty patients had vertigo of central origin with positive central nervous system findings, 20 patients had Meniere's disease, and 30 subjects were healthy. INTERVENTION: The SPNT test is a smooth pursuit eye movement test. The subjects are placed in a neutral position, and then they turn 45 degrees to the right and to the left. The difference between the average gain in neutral and torsion positions is the test parameter. In addition to the SPNT test, the authors performed saccade tests, auditory brain stem response, and the caloric test. MAIN OUTCOME MEASURES: In the two WAD groups, neck torsion reduced the SP gain (p < 0.001), but in control patients with central and peripheral vertigo and in the healthy control subjects, it did not. RESULTS: The sensitivity of the SPNT test in the WAD group with dizziness was 90% and the specificity was 91%. The sensitivity in the WAD group without dizziness was 56%. CONCLUSION: The SPNT test seems to be useful for diagnosing cervical dizziness, at least in patients with WAD having symptoms of dizziness, because it has a high sensitivity and specificity.

Adolescent↗

Vestibular neurectomy: a histological and clinical study of results.

Posterior fossa vestibular neurectomy has become one of the most effective surgical procedures for control of vertigo symptoms in Menière's disease. A small group of patients continue to have vertigo and demonstrable vestibular function by electronystagmography (ENG) post-operatively. Another group of patients may have no vertigo, despite residual vestibular function on caloric testing. In an effort to correlate histological findings with clinical outcome, nerve biopsies were taken from seven patients undergoing vestibular neurectomy. Fibre counts of nerve biopsies were compared with clinical, audiometric and ENG results. Results of this study demonstrate that complete reduction of vestibular response (on caloric testing) and clinical cure of vertigo can be obtained despite a variable number of nerve fibres at vestibular neurectomy.

Adult↗

Internal auditory canal vascular loops: audiometric and vestibular system findings.

Prominent loops of the anterior inferior cerebellar artery in the cerebellopontine angle are found frequently during anatomic studies of this region. These vascular loops are suspected of causing hearing loss, tinnitus, and vertigo, and surgery has been advocated to separate the vascular loop from the eighth cranial nerve. Previous reports have described pathologic anatomy, surgical approaches, and results of treatment. In this study, we report the results of a uniform battery of audiometric and vestibular system test results administered to fifteen patients with prominent vascular loops in the internal auditory canal diagnosed by pneumo-CT. All patients were tumor suspects before CT because of unilateral (or asymmetric) tinnitus or hearing loss. Hearing losses ranged from mild to profound, and most were of a cochlear type with excellent speech discrimination. Only one-third of the patients had abnormal caloric tests, but spontaneous nystagmus was detected in all but one of the patients by photoelectric nystagmography. The wide range of audiometric and vestibular system test results probably reflects the complex interaction between the vascular loop and eighth cranial nerve, in which the loop exerts pressure on the nerve, and the nerve compromises inner ear circulation. Eighth nerve tumors and vascular loops produce similar symptoms, but a cochlear type of hearing loss with good speech discrimination and normal caloric testing should raise suspicion of a vascular loop. Pneumo-CT is an effective means of diagnosing vascular loops and differentiating them from other lesions of the cerebellopontine angle.

Adult↗

Inferior vestibular neuritis.

Sudden, spontaneous, unilateral loss of vestibular function without simultaneous hearing loss or brain stem signs is generally attributed to a viral infection involving the vestibular nerve and is called acute vestibular neuritis. The clinical hallmarks of acute vestibular neuritis are vertigo, spontaneous nystagmus, and unilateral loss of lateral semicircular function as shown by impulsive and caloric testing. In some patients with vestibular neuritis the process appears to involve only anterior and lateral semicircular function, and these patients are considered to have selective superior vestibular neuritis. Here we report on two patients with acute vertigo, normal lateral semicircular canal function as shown by both impulsive and caloric testing, but selective loss of posterior semicircular canal function as shown by impulsive testing and of saccular function as shown by vestibular evoked myogenic potential testing. We suggest that these patients had selective inferior vestibular neuritis and that contrary to conventional teaching, in a patient with acute spontaneous vertigo, unilateral loss of lateral semicircular canal function is not essential for a diagnosis of acute vestibular neuritis.

Adult↗

[Intratympanic gentamicin treatment for intractable Menière's disease].

BACKGROUND: Intratympanic gentamicin is an accepted mode of treatment for patients with Meniere's disease who suffer from recurrent vertigo attacks in spite of conservative medical treatment. Recently, it gained popularity as the primary treatment for intractable Meniere's disease, prior to surgical procedures. However, administration methods and treatment protocols still differ from one medical center to another. OBJECTIVES: We aimed to present outcomes of gentamicin treatment for vertigo attacks and functional capacity, based on our experience with 23 patients over 3 years and review the literature on the subject. METHODS: Three specially prepared gentamicin injections, were administered once a month, according to the state of vertigo attacks, functional capacity, and hearing status. Clinical observation, frequent hearing examinations and caloric tests were used for follow-up. RESULTS: Following treatment, 20 (87%) patients no longer experienced vertigo attacks, while 16 of them (69.6%) reported full functional recovery. Two patients (8.7%) did not respond to treatment, and therefore, underwent surgical procedures. One patient (4.4%) did not complete therapy. Post treatment caloric tests showed significant deterioration (P < 0.02). Severe vestibular weakness (51-100%) was found in the treated ear in 11 of the 14 patients with unilateral disease (78.6%). The mean change in hearing examination was non-significant. However, two of the 21 patients suffered a change from moderate to severe hearing loss to complete deafness (9.5%). There was no case of post treatment infection or permanent tympanic perforation. CONCLUSIONS: Intratympanic gentamicin injection is a simple and safe procedure for intractable Meniere's patients, with a high success rate and low risk for hearing deterioration. It is recommended for patients with continuous functional disability prior to surgical treatment.

Anti-Bacterial Agents↗

Familial hereditary progressive sensorineural hearing loss among Saudi population.

Clinical and audiological studies of 234 Saudi patients from the central area of Saudi Arabia with progressive sensorineural hearing loss were carried out in Riyadh. One hundred and sixty-four came from 38 families, i.e. 70% of the total and 30% were sporadic cases. Consanguinity was found in 80.8%. Their hearing loss was characterized as being bilateral sensorineural, starting at 1 kHz frequency and gradually sloping the pure tone audiogram to severe and profound hearing loss at 8 kHz. Special tests for recruitment indicate hair cell lesions. Caloric tests were normal in 56 patients tested. No abnormal internal ears were found in a C.T. scan done for 84 cases. Serology for TORCH and non-TORCH agents were done for (124) selected cases. Syphilis was negative, however, positive immunoglobulins for cytomegalovirus, herpes simplex type 1 and 2, and toxoplasmosis were seen in 52 cases. All patients came from one area called Qassim where old customs and the tradition of consanguineous marriage were still practiced. Some of these families are now under molecular and cytogenetic study.

Adolescent↗

Neurotologic manifestations of the fibromyalgia syndrome.

The purpose was to assess otologic symptoms, and audiologic and vestibular findings in fibromyalgia (FM) syndrome. Twenty-four female patients with FM syndrome (FMS) were included in the study. The assessments were based on history, physical examination, audiometry, bithermal caloric testing and auditory brainstem response (ABR) testing. Dizziness was the most common complaint of the patients, and was followed by tinnitus, hearing loss and vertigo. Almost 50% of the patients had some sort of otologic symptoms.Dix-Halpike maneuver proved positional rotary vertigo in 5 (20.8%) patients. The audiometry results of 23 patients were normal. None of the patients had abnormal bithermal caloric testing. Although there were a variety of ABR abnormalities, the ABR results of the patients with and without cochleovestibular symptoms were not significantly different (p<0.05). In conclusion, FM patients can complain otologic symptoms even though they do not have any clinically or audiologically detectable ear disease. A neural disintegration or some other events related to neural mediators may be the mechanisms involved in the pathogenesis of otoneurologic as well as systemic manifestations of the disease that possibly leads to abnormal perception of the stimuli coming from internal or external environment.

Adult↗

[Functional state of the vestibular analyzer during lower body negative pressure tests].

With the aid of the caloric test the functional state of the vestibular analyzer of nine male athletes was investigated during their exposure to lower body negative pressure tests. The prolongation of the latent period of the caloric nystagmus and vertigo, shortening of the time of the nystagmus and vertigo were observed. It is concluded that the cerebral blood supply plays a major role in the development of vestibular reflexes.

Abdomen↗

[Clinical study on the mechanism of motion sickness--clinical examination based on trigger factors].

This report deals with factors which trigger motion sickness. The author has focused on the clinical examinations applied to motion sickness cases. Relationships between clinical data and susceptibility to motion sickness were investigated. The following results were obtained: 1. CV R-R(coefficient of variation of R-R intervals on EGG)in the frequent motion sickness group showed higher values than in the occasional motion sickness group. This result suggests a hyperfunction of the vagus nerve. 2. In caloric testing with ENG, the motion sickness group showed lower values of duration and eye speed than the occasional motion sickness group. On the other hand, the frequent group showed higher values of laterality in eye speed than the occasional group. 3. With the visual suppression test, the frequent motion sickness group showed higher values than the occasional motion sickness group. On the other hand, there was a significant difference between the frequent motion sickness group and the occasional motion sickness group in terms of laterality of visual suppression values. 4. In testing fluctuation of CV R-R in response to optokinetic stimuli, the frequent motion sickness group showed elevation of CV R-R values after optokinetic load. On the other hand, the occasional motion sickness group showed depression of CV R-R values after the same load. 5. Among clinical examinations designed to diagnose susceptibility to motion sickness, CV R-R, the caloric test, the visual suppression test and OKP testing can produce sufficiently accurate results for clinical investigation.

Electrocardiography↗

[Vestibular neurotomy versus chemical labyrinthectomy for disabling Menière disease].

OBJECTIVE: To evaluate the efficiency on the vertigos and the impact on the audition of the vestibular neurotomy (VN) and the chemical labyrinthectomy (CL) in patients with severe Menière's disease, in order to determine the precise place for each technique in treatment algorithm. METHOD: Retrospective study of 71 VN (performed between 1986 and 2003) and 35 CL (performed between 1997 and 2003). These two treatments both aim at obtaining a vestibular deafferentation in order to free definitely the patient of vertigo manifestations. Vestibular results have been assessed by caloric tests performed before and 6 months after treatment. Subjective success was evaluated by searching for recurrent attacks of vertigo and by the AAO-HNS (American Academy of Otolaryngology-Head and Neck Society) scale of subjective evaluation of vertigos (follow-up of 6.4 years after VN and 2.4 years after CL). Pure tone audiometry before treatment and then 6 months later was performed. Complications of two types of treatment were recorded and evaluated. RESULTS: In 90% of the cases after VN and 86% of the cases after CL, caloric tests indicated a strong vestibular hyporeflexy (hypovalence > 90%). Attacks of vertigo reoccurred in 5.6% of the cases when the hyporeflexy was strong and 85.7% of the cases when it was weak. The quality of the vestibular results on vertigo depends on the degree of hypovalence after treatment. An improvement of the AAO-HNS scale have been obtained in 93% of the cases in the NV group and in 81% of the cases in the LC group. Attacks of vertigo were recurrent in 7% of the patients operated on by VN and 11.4% of the patients treated by CL. Mean pure tone auditory thresholds changed from 50.24 dB HL to 55.64 dB HL (p=0.003) in the VN group and from 69.11 dB HL to 74.51 dB HL (p=0.41) in the CL group. Comparison of the variations of the mean pure tone auditory thresholds before treatment and 6 months after the end of the treatment doesn't show any significant difference between the 2 groups (p > 0.05). Impairment of the audition superior to 20 dB HL was observed in 8.5% of the patients of each group. The auditive results are similar in the 2 groups. CONCLUSION: These two methods of treatment can not be strictly compared due to several bias in this study. Indeed more patients were treated by VN with a longer follow-up and only the patients with a class C or D audition according to the criteria of the AAO-HNS could be treated by CL. The VN provides a better control of the vertigos than the CL which is an efficient method of treatment in invalidating Meniere's disease. In all the cases, the quality of the results on vertigos depends on the degree of hypovalence after treatment. The auditive results are similar. Since the results of the two treatments are similar, the simplicity of CL encourages us to broaden the indications and to modify the place of the VN in the therapeutic algorithm of Menière's disease.

Adult↗

Vestibular testing in hearing loss.

The basic electonystagmography battery (saccade, gaze, position, caloric tests, and spontaneous nystagmus) are described along with rotational testing and dynamic posturography. Emphasis is placed on interpretation, relative benefits, and limitations as an aid in diagnosis.

Caloric Tests↗

Transmastoid labyrinthectomy: surgical management of vertigo in the nonserviceable hearing ear. A five-year experience.

We have continued to employ transmastoid labyrinthectomy since reporting our results for the first 15 patients. Further experience has confirmed our earlier findings that the vestibular system rapidly accommodates to complete unilateral surgical vestibular ablation, regardless of age and vestibular activity of the ear operated upon as measured by bithermal caloric testing. Patients are able to ambulate in one to three days and typically return home three to eight days postoperatively. Only 7 percent of sixty patients retained occasional unsteadiness and only two individuals stated that they suffered constant unsteadiness. Once compensated, there was no instance in which vertigo or unsteadiness returned during the follow-up period. In the individual with unilateral sensorineural hearing loss and incapacitating dysequilibrium, the ear with the hearing loss can be reliably implicated as the one producing the vertigo, regardless of the vestibular activity in that ear as measured by the bithermal caloric test. As we gain more experience, this has continued to be a valid assumption. No individual has lost hearing in the contralateral ear during the period of observation. Dysequililbrium with all its variants--rotary vertigo, instability, veering , ataxia, and lightheadedness--is one of man's most disabling and incapacitating afflictions and affects an individual's ability to pursue normal life and work. We continue to be impressed with the length of time many individuals have borne this incapacitation; one-third of our patients have been incapacitated for over five years.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗