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Progressive fluctuant hearing loss, enlarged vestibular aqueduct, and cochlear hypoplasia in branchio-oto-renal syndrome.

OBJECTIVE: To study the results of petrosal bone imaging and audiometric long-term follow-up of two patients with branchio-oto-renal (BOR) syndrome and relate them to the clinical features, including caloric responses. STUDY DESIGN: Longitudinal case study. SETTING: Tertiary referral center. PATIENTS: A father and son with the BOR syndrome. MAIN OUTCOME MEASURES: Both patients underwent imaging studies to detect and evaluate inner ear anomalies. Longitudinal audiometric analysis of the hearing threshold data over the previous 23 years was performed. Caloric tests were performed at various ages. RESULTS: The son had a short, wide internal acoustic canal, a hypoplastic cochlea, a plump vestibule, and a wide vestibular aqueduct on both sides; the semicircular canals and endolymphatic sac were of normal size. He showed progressive fluctuant sensorineural hearing loss. Caloric tests disclosed hyporeflexia on the left side. The father had a plump internal acoustic canal and hypoplastic cochlea on both sides. The left vestibule was hypoplastic, and the left vestibular aqueduct was marginally enlarged. He showed severe hearing impairment, without substantial progression or fluctuation, and caloric areflexia on the left side. CONCLUSION: These findings suggest a correlation between progressive fluctuant sensorineural hearing loss with caloric hypofunction and the presence of an enlarged vestibular aqueduct in the BOR syndrome. Additional longitudinal case studies are needed to further evaluate such a correlation.

Adult↗

Vestibular neuropathy accompanying auditory and peripheral neuropathies.

OBJECTIVE: To define the incidence of measurable vestibular disorders in patients with auditory and peripheral neuropathies. DESIGN: Descriptive study of the case features of auditory neuropathy in 14 patients, 8 of whom had concomitant peripheral neuropathies. SETTING: University referral center. PATIENTS: Fourteen patients aged from 10 to 75 years and diagnosed as having auditory neuropathy, 8 of whom had concomitant peripheral neuropathies. MAIN OUTCOME MEASURES: Incidence of abnormal vestibular caloric test results and the relationship of such incidence to clinical variables including the ages of the subjects, the presence of a concomitant peripheral neuropathy, vestibular symptoms, and audiological findings. RESULTS: Abnormal vestibular caloric test results occurred in 9 of the 14 patients. These 9 patients were on average older (35.6 years) than patients with normal caloric responses (17.8 years). Seven of the 9 patients with abnormal caloric responses had concomitant peripheral neuropathies compared with only 1 of the 5 patients with normal caloric responses. None of the 14 patients experienced symptoms of vestibular disorder. CONCLUSIONS: Asymptomatic vestibular disorders are common in patients with auditory neuropathy when a peripheral neuropathy is also present. The reason for the abnormal vestibular test results is likely a neuropathy of the vestibular nerves. Arch Otolaryngol Head Neck Surg. 2000;126:1453-1456

Adolescent↗

Electronystagmographic findings in 147 patients with acoustic neuroma.

We report on the preoperative findings in 147 patients with surgically confirmed acoustic neuroma (AN). Gaze nystagmus, spontaneous nystagmus and positional nystagmus were present in 23.1%, 47.5% and 63.0% of the patient, respectively. Pathological findings in ETT, OKP and bithermal caloric test were found in 44.3%, 45.3% and 77.0%, respectively. The ratio of pathological findings in ETT and OKP was larger in the large tumor group than in the small tumor group. Gaze nystagmus to both directions and direction-changing positional nystagmus were also found in the large tumor group. While the correlation between tumor size and CP% in caloric test was slightly significant (correlation coefficient, 0.52) in all 122 patients, it was more significant (correlation coefficient, 0.65) in 16 patients whose tumor origin was surgically confirmed to be the inferior vestibular nerve. These findings suggest that reduced caloric response is caused by compression of the nerve by tumor mass. On the other hand, 6 patients whose tumor originated from the superior vestibular nerve showed no significant correlation between CP% and tumor size.

Adolescent↗

Balancing the caloric-induced nystagmus velocity with cold air and water.

A useful alternative to the traditional water caloric is to use an air stimulus. However, the caloric test has not been standardized and a range of parameters are being used in different audiology clinics. The aim of this study was to determine cold air parameters that resulted in a similar slow-component eye velocity to that for water irrigation. Twelve normal subjects underwent caloric testing using air temperatures in the range 18-33 degrees C. The duration and air-flow rate were held constant at 60 s and 5 l/min. A water irrigation at 30 degrees C for 30 s and delivering 150 ml resulted in a mean slow-component eye velocity of 17 degrees/s. An equal response was obtained with an air temperature of 21.0 degrees C. Further work is required to find equivalent air and water responses for other combinations of parameters.

Adolescent↗

Prediction of canal paresis using head-shaking nystagmus test.

OBJECTIVE: To compare the incidence of head-shaking nystagmus (HSN) with the value of canal paresis (CP) obtained from a caloric test and to propose the optimal value of CP that can be predicted by the presence of HSN. MATERIAL AND METHODS: HSN and caloric tests, together with other vestibular tests, were performed in 132 dizzy patients. HSN was recorded using electronystagmography with Frenzel's glasses. RESULTS: HSN was observed in 61/132 patients (46%). The sensitivity and specificity of the HSN test regarding the prediction of CP>20% were 66% and 77%, respectively. CP values were significantly higher in patients with than without HSN. Both the sensitivity and specificity of HSN were reasonably high when the limiting value of CP was set between 20% and 30%. CONCLUSION: The limiting value of CP that discriminates the presence of HSN may be approximately 20%. At this CP value, HSN shows an appropriately high sensitivity and specificity. The HSN test is not very sensitive but is still acceptable as one of the screening tests for detecting asymmetrical vestibular dysfunction.

Ear Canal↗

[Clinical study in vertiginuous patients suspected of having neurovascular compression syndrome of the eighth cranial nerve].

Neurovascular compression syndrome of the 5th and 7th cranial nerves has been recognized as the cause of trigeminal neuralgia and hemifacial spasm. On the other hand, it is still difficult to diagnose vertigo as neurovascular compression syndrome of the 8th cranial nerve. To detect some specific finding in this syndrome of the 8th cranial nerve, 5 patients with vertigo with hemifacial spasm were examined for the clinical course and neuro-otological features. In all patients MRI and/or angiography suggested vascular compression against the 8th cranial nerve. The clinical courses of these patients revealed various symptoms resembling benign paroxysmal positional vertigo, vestibular neuronitis and Meniere's disease. Audiograms showed two normal hearing patterns, bilateral high frequency hearing loss probably due to aging in one case, bilateral C5-dip in one and fluctuating unilateral hearing loss like Meniere's disease in one. The prolongation of IPL I-III on auditory brainstem response proposed as a criterion by Møller was detected in one case. No response in the caloric test was found in two cases. These abnormalities in the auditory brainstem response and caloric test appeared to be useful for diagnosis but were uncommon findings in all cases. Electronystagmographic examinations including the eye tracking test, optokinetic nystagmus and optokinetic pattern were all normal. We could not find any specific clinical findings valuable for diagnosis of neurovascular compression syndrome of the 8th cranial nerve. It is proposed that the indication of microvascular decompression should be decided carefully.

Cerebral Angiography↗

[Skull vibratory test in partial vestibular lesions--influence of the stimulus frequency on the nystagmus direction].

INTRODUCTION: Results of the skull vibratory test (SVT) in partial unilateral vestibular peripheral lesions (PUVL) are different from the results in total vestibular lesions (TUVL). AIM: To reveal a correlation between the results of the analysis of the skull vibratory nystagmus (SVN) horizontal component and the side of the lesion; to correlate these results with the stimulus frequency. To find out a predictive correlation between the SVN horizontal and vertical components and the topography of a vestibular lesion. To appreciate the degree of vestibular deafferentation (extended to high frequencies) provoked by gentamicin labyrinthectomy and its efficiency in Meniere's disease. PATIENTS AND METHODS: 53 patients with a SVN and a PUVL were included and compared with 10 TUVL and 10 normal subjects. Protocol included a HST (2 Hz), a SVT at 30, 60 and 100 Hz and a caloric test. Recordings were performed with a 2D and 3D VNG device. RESULTS: In PUVL, SVN at 30, 60 and 100 Hz was obtained in 80, 90 and 90% of cases respectively. SVN is correlated with the side of the lesion at 30, 60 and 100 Hz respectively in 65%, 63%, 80% of cases. SVN is not correlated with the side of the lesion in 20% of Meniere's disease, in 8% of vestibular neuritis and in 6% of vestibular schwannoma. In PUVL HSN is correlated with the side of the lesion in 69% of cases. The direction of the HSN and of the SVN was different in 23% when the nystagmus attended at the same time for both tests. In PUVL the direction of the SVN is different at 100 Hz and 30 Hz in 16% of cases when they are concomittant on the same patient. After Gentamicine labyrinthectomy, the coherence of the results in caloric test, HSN and SVN (areflexy and lesional nystagmus beating toward the safe side) was correlated with the efficiency of the therapy. A SVN vertical component was met in 10% of PUVL (essentially in anterior canal dehiscence and few cases of partial labyrinthitis). The horizontal SVN SPV is significantly slower in PUVL than in TUVL patients (p=0.0004). CONCLUSIONS: The SVT is a vestibular global and rapid test which explores high frequencies. In PUVL the direction of SVN is not always predictive of the side of the lesion and is sometimes depending on the stimulus frequency, the state of the vestibular lesion, the vestibular structure concerned (1/2 circular canals or otolithic organs) and the kind of sensory cells implicated in the lesion. In TUVL The direction of the SVN is always coherent with the side of the lesion (this is useful to predict the efficiency of a Gentamicine Labyrinthectomy). A SVN vertical component can mean a lesion of the vertical canal in PUVL.

Adolescent↗

The role of summating potential in the diagnosis and management of Menière's disease.

To assess the role of a negative summating potential (-SP) in the clinical diagnosis and management of Menière's disease, the relationship of -SP abnormality to clinical symptoms, hearing level and caloric test results was examined in 70 patients with unilateral Meniere's disease. The click-induced -SP and action potential (AP) were recorded by extratympanic electrocochleography. When SP/AP ratio exceeded 0.43, the -SP was considered to be abnormal. No significant relationship was found between abnormal -SP and clinical symptoms (the duration of cochlear and vestibular symptoms, recent hearing fluctuation and recent vertigo attack). Ears with abnormal -SP had a significantly worse hearing loss at high frequencies (2-8 kHz) than ears with normal -SP, whereas there was no significant difference in hearing loss at low frequencies (0.25-1 kHz) between both ears. No significant relation was found between abnormal -SP and abnormal unilateral weakness in caloric test. The results indicate that the click-induced -SP reflects the pathophysiology of Menière's disease basically in the cochlear partition at high frequencies.

Action Potentials↗

Vertigo and electronystagmography in uni- and bilateral Ménière's disease.

In this study we performed standardized measurements of spontaneous nystagmus (SN), caloric tests and velocity step tests on a well-defined group of 92 Ménière patients as part of a diagnostic protocol. The aim of the study was to look for results that could contribute to the diagnosis, but more specifically to a possible classification, of Ménière's disease. Relations with average hearing loss, shape of the audiogram, average hearing loss, duration and severity of perceived symptoms of disease and differences between uni-and bilateral Ménière's disease were studied. Directional preponderance (DP) in unilaterally affected patients and labyrinthine preponderance (LP) in both uni- and bilaterally affected patients were significantly more often directed towards the unaffected (uni) or least affected (bi) side (p = 0.007, p = 0.001, p = 0.002, respectively). DP was correlated with LP (uni: R = 0.615, p < 0.001; bi: R = 0.438, p < 0.01), which means that the DP can be seen as a latent SN, directed to the unaffected side. With the caloric test parameters LP and DP it was possible to find unilateral weakness. In unilateral disease a duration of disease <2 years resulted in small asymmetry in time constant (T(asym)), large LPs and LPs not only directed to the unaffected, but also to the affected side, whereas a longer duration of disease gave large T(asym) and small LPs that were only directed to the unaffected side. Patients suffered more severe vertigo in the first two years of the disease. Studying the shape of the audiogram, it was found that patients having an LP to the affected side, suggesting that this was a hyperactive labyrinth belonging to a beginning of the disease, never had a flat audiogram. In this study the LP was the best diagnostic parameter to find unilateral weakness in patients with Ménière's disease. Together with the duration of the disease, the severity of symptoms, the asymmetry in T and the shape of the audiogram, it was possible to find two subgroups in the group of patients with unilateral Ménière's disease. Vestibular data in association with other test results of the diagnostic protocol may contribute to a more sophisticated classification of Ménière's disease.

Adult↗

[Head-thrust test: its validity as a diagnostic clinical test].

The head-thrust test is presented as an ideal test to investigate the horizontal vestibulo-ocular reflex. We review its theoretical basis, and assess it as a diagnostic test with a 22 patient series. Its specificity accounts for 100% and the sensibility for 54%. Would this test substitute in the future the caloric test?

Caloric Tests↗

[Chemical labyrinthectomy: results and applications].

GOAL OF THE STUDY: Unilateral Menière's disease can be treated by chemical labyrinthectomy. The goal of this work was to report our clinical experience with chemical labyrinthectomy, and to discuss the respective value of labyrinthectomy and of vestibular neurotomy for treating unilateral Menière's disease. METHODS: Twelve patients underwent chemical labyrinthectomy using middle ear gentamicin infusion. Before treatment, patients had a pure tone audiogram and a vestibular caloric testing. After treatment, patients had a clinical assessment at 1 and 6 months after treatment (pure-tone audiogram, vestibular caloric testing), and answered a questionnaire at 1 and 2 years following the treatment (number of vertigo attacks, ataxia, tinnitus, subjective assessment of the method by the patient). RESULTS: At two years post-treatment, the vertigo cure rate was 91.6% (ABC using AAOO criteria), vestibular function was abolished in 70% of cases, ataxia was reported in 42% of patients, 16.6% of patients had hearing deterioration above 20 dB HL (C following AAOO criteria), tinnitus was decreased in 50% of cases. Of 12 patients, 8 were satisfied. CONCLUSION: Chemical labyrinthectomy cures vertigo in Menière's disease, with similar cure rate than vestibular neurotomy. Minor complications can occur with chemical labyrinthectomy. This method should be used in more patients.

Adult↗

[Horizontal canal benign paroxysmal positional vertigo--its affected side and horizontal semicircular canal function].

We report horizontal canal BPPV (HC-BPPV) targeting its pathophysiology, the affected side, and the function of the horizontal semicircular canal, together with a review of the literature. Subjects were 13 patients with HC-BPPV visiting our vertigo outpatient clinic at Nara medical university hospital and a related hospital in the 2.5 years from January 2000 to June 2002. Subjects were classified into 7 with canalolithiasis and 6 patients of cupulolithiasis after a neurotological examination. CP was positive in 54% of all patients, 71% of those with canalolithiasis, and 33% of those with cupulolithiasis. To determine the affected side in HC-BPPV, we used the affected side by using the law of Ewald in canalolithaisis patients and the detection of a neutral diminishing nystagmus in cupulolithiasis patients. CP positive in caloric testing indicated insignificant dysfunction of the horizontal semicircular canal in canalolithiasis patients compared to that in cupulolithasis patients. The mechanism behind caloric nystagmus was thought to be a convection of endolymphatic fluid interrupted consequently by an otolith in the semicircular canal in canalolithiasis patients. In contrast, CP was positive in cupulolithiasis patients regarded as having no convection of endolymphatic fluid. The mechanism causing a difference in caloric test results between canalolithiasis and cupulolithiasis patients thus requires a larger patient population and further examination to be conclusive.

Aged↗

Visual suppression test.

The visual suppression test is one of the visual fixation tests. It is performed by recording caloric nystagmus by ENG, and the maximum slow phase velocity of caloric nystagmus in darkness is compared with the slow phase velocity in light with eyes open. Visual suppression of slow phase velocity of caloric nystagmus is 54 +/- 12% in 52 normal adults ageing from 21 to 40. Visual suppression in normal subjects is not influenced by water temperature used for the caloric test and not changed by background illumination in light. Visual suppression is stronger when the target is closer to the eyes of the subjects. The following abnormalities have been diagnosed by this test: flocculus and nodulus lesions on the side of the lesion; inferior olive lesions; parietal lobe lesions; compensation after unilateral sudden loss of inner ear function.

Adult↗

[Isolated vertigo in vertebrobasilar ischemia--does it exist?].

An isolated vertigo may occur in vertebrobasilar ischemia, especially as the first symptom of cerebellar ischemia or basilar artery occlusive disease. Based on neuroanatomical findings, these short-lasting vertigo attacks result from transient insufficiency of the anterior inferior cerebellar artery (AICA), which supplies the inner ear and leads to ischemia of the upper parts of the vestibular labyrinth. Persisting vertigo with or without lateropulsion may occur in cerebellar infarctions with involvement of the medial inferior hemispheres due to occlusion of the medial branch of the posterior inferior cerebellar artery. Caloric testing with oculography can usually differentiate cerebellar infarction and peripheral vestibular disease. Since ischemic lesions with AICA insufficiency may cause pathological results in caloric testing, both clinical and neurophysiological analysis of associated oculomotor signs is essential for a correct diagnosis.

Cerebellum↗

Vestibular decruitment in central nervous system disease.

The differential caloric test consisting of a weak and of a strong stimulation may provide evidence of certain vestibulo-oculomotor abnormality. The phenomenon of vestibular decruitment serves as an indication of central nervous system or intracranial pathology. A classification of the degree of decruitment was attempted in 139 patients exhibiting this phenomenon. Ample evidence is provided that vestibular decruitment is indicative of a variety of intracranial lesions predominantly in the posterior fossa. Decruitment was encountered with hypo- and hyperactive vestibular responses alike and also in patients in whom there was no deficit in end organ sensitivity. The armamentarium of neurotologic testing is enriched by the differential caloric stimulations providing an indication of the "site of lesion."

Adolescent↗

Does intratympanic gentamicin treatment for Meniere's disease cause complete vestibular ablation?

OBJECTIVE: To determine changes in vestibular function following intratympanic gentamicin (ITG) treatment for Meniere's disease and to correlate changes with the need for further treatment. STUDY DESIGN: Prospective case series. PATIENTS: One hundred and three patients with disabling unilateral Meniere's disease who had failed a minimum of 6 months medical treatment. MAIN OUTCOME MEASURES: Vestibular function was measured by electronystagmography (ENG) caloric testing before and serially following treatment. Caloric responses were classified as normal (excitability difference [ED] < 25%), bithermal response (ED > or = 25%), positive response to ice water only, and absent ice water response. RESULTS: Twenty-one percent of patients had a bithermal caloric response, 62% had an absent ice water response, and 17% had an ice water response only 1 month following treatment. Mean follow-up was 27.3 months (range = 1-106 months). Eighty-four patients had one treatment course only. Fourteen patients required a further course of treatment due to recurrence of vertigo; 38% of these had recovery of caloric function. Significantly more patients with normal caloric function prior to initial treatment required further treatment compared to those with initial reduced caloric function (p < .05). Patients rendered absent ice water responsive were significantly less likely to require further treatment than those with a persistent caloric response (p < .0001). CONCLUSION: An absent ice water response is highly predictive of adequate vertigo control. Regimens of ITG that aim to completely ablate vestibular function are recommended.

Adult↗

VEMPs and dynamic posturography after intratympanic gentamycin in Menière's disease.

In this study, we investigated whether vestibular evoked myogenic potentials (VEMPs) and dynamic posturography (DP) are useful in the evaluation of the vestibular function in patients affected by active monoaural Menière's disease (MD) treated with intratympanic gentamycin. Twelve subjects affected by monoaural MD were treated with weekly intratympanic injections of 0.5 ml of gentamycin at a concentration of 20 mg/ml. VEMPs were used to test saccular function, while postural control was analyzed by DP. The results obtained with these two techniques were compared with those obtained by using bithermal caloric test. The mean follow-up was 15.3 months (6--28 months). Therapy resulted in complete absence of the caloric response in six subjects (50) and in caloric test-induced asymmetry in the remaining individuals, ranging from 83 to 27. At follow-up, eleven patients (91.6%) were free of vertigo, while one patient had two vertigo spells 9 months after treatment. Before treatment, VEMPs were present in the affected ear of eleven patients. After treatment VEMPs were absent in all the patients. At the end of follow-up, reappearance of VEMPs was observed in two patients, with no changes in latency values and amplitude ratio. DP demonstrated a reduction of the Composite Score (CS) one week after therapy, with a prevalent reduction of the vestibular component. After 6 months, there was an improvement of the CS and, in particular, of the vestibular component. The present study demonstrates that bithermal caloric test and VEMPs allow for the functional evaluation of both the horizontal semicircular canal and the sacculus, suggesting that these techniques might be used together to monitor the efficacy of intratympanic gentamycin therapy. In addition, our data indicate that DP might provide important information on compensation phenomena and show that intratympanic gentamycin can improve postural control in MD patients.

Adult↗

Vestibular assessment in a patient with confirmed lateral medullary syndrome.

The results of vestibular investigations in a patient with confirmed lateral medullary syndrome are presented. This patient showed a unilateral weakness on caloric testing which has not been reported previously in a patient with lateral medullary syndrome. The case for the possibility of a 'central' canal paresis on caloric testing is presented.

Electronystagmography↗