Cases reported "Vertigo"

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1/19. Familial progressive vestibulocochlear dysfunction caused by a COCH mutation (DFNA9).

    OBJECTIVE: To describe the decline of vestibulocochlear function in a man with vestibulocochlear dysfunction caused by a Pro51Ser mutation within the COCH gene on chromosome 14q12-13 (DFNA9). methods: A follow-up of more than 15 years was performed in a single case. Clinical investigations were supplemented by oculomotor, vestibular, and auditory tests. RESULTS: A 50-year-old man had had progressive sensorineural hearing loss and dysequilibrium for 15 years; he had been asymptomatic at the age of 35 years. He suffered from instability in the dark, head movement-dependent oscillopsia, paroxysmal positional vertigo, and vertigo with and without nausea. Hearing impairment started unilaterally, predominantly in the high frequencies. He also reported tinnitus. Disease progressed to severe bilateral high-frequency hearing impairment and vestibular areflexia. Fluctuation of vestibulocochlear function was documented and mentioned by the patient. CONCLUSIONS: Our patient proved to suffer from an autosomal dominant vestibulocochlear disorder caused by a COCH gene mutation. The remarkable medical history has some features in common with meniere disease; however, there are also different clinical and neurophysiological features. In the family, phenotypic variability is present.
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2/19. Vestibulo-ocular and vestibulospinal function before and after cochlear implant surgery.

    Vestibular function in cochlear implant candidates varies from normal to total absence of function. In patients with intact vestibular function preoperatively, invasion of the otic capsule places residual vestibular function at risk. speech-processing strategies that result in large amplitude electrical transients or strategies that employ high amplitude broad frequency carrier signals have the potential for disrupting vestibular function. Five patients were tested with and without electrical stimulation via cochlear electrodes. Two patients experienced subjective vestibular effects that were quickly resolved. No long-term vestibular effects were noted for the two types of second generation cochlear implants evaluated. Histopathological findings from another patient, who had electrically generated vestibular reflex responses to intramodiolar electrodes, indicated that responses elicited were a function of several variables including electrode location, stimulus intensity, stimulus amplitude, and stimulus frequency. Differential auditory, vestibulocolic, and vestibulospinal reflexes were demonstrated from the same electrode as a function of stimulus amplitude, frequency, and duration.
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3/19. Anti-neuronal nuclear autoantibody type 2: paraneoplastic accompaniments.

    We identified the IgG autoantibody ANNA-2 ("anti-Ri") in 34 patients in a 12-year period by immunofluorescence screening of sera from approximately 75000 patients with subacute neurological disorders that were suspected to be paraneoplastic. Detailed clinical information was available for 28 patients (10 men, 18 women). Cancer was diagnosed in 24 patients (86%); 21 had histologically proven carcinoma (10 lung, 9 breast, 1 cervical, 1 bladder), and 3 had an intrathoracic imaging abnormality. Cancer anteceded neurological symptoms in 4 of 28 patients. Cancer detection frequency increased with continued surveillance. Neurological disorders, in decreasing frequency, were brainstem syndrome (including opsoclonus, myoclonus, or both), cerebellar syndrome, myelopathy, peripheral neuropathy, cranial neuropathy, movement disorder, encephalopathy, Lambert-Eaton syndrome, and seizures. Four patients had laryngospasm and four had jaw opening dystonia (two with neck dystonia). Nine (32%) were wheelchair-bound 1 month after neurological symptom onset. Most improved neurologically after immunomodulatory or tumor-directed therapy. Accompanying autoantibodies, found in 73% of sera, included ANNA-1, ANNA-3, CRMP-5-IgG, P/Q-type and N-type Ca(2 ) channel antibodies, and muscle-type acetylcholine receptor antibody. Some neurological accompaniments of ANNA-2 may reflect potentially pathogenic humoral or cell-mediated responses to coimmunogenic tumor antigens, for example, Lambert-Eaton syndrome (P/Q-type Ca(2 ) channel antibody) and peripheral neuropathy (ANNA-1 effector T cells).
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4/19. susac syndrome: retinocochleocerebral vasculopathy.

    susac syndrome is a rare microangiopathy of cochlea, retina, and brain. We report a case of a 30-year-old man with susac syndrome. The patient initially suffered from unilateral hearing loss associated with peripheral vestibular syndrome, and followed with recurrent arterial retinal occlusions and encephalopathy. The patient underwent clinical, laboratory, and neuroradiological examination. Laboratory tests were negative for systemic inflammatory or infectious disease. Signs of encephalopathy and vestibular syndrome regressed after 6 weeks, retinal obstructions were partially improved, and deafness remained unchanged. Two unexplained epileptic seizures had been documented 7 years before the development of typical clinical course. The etiology is still unknown and diagnosis was suggested by the clinical triad of bilateral sensorineural hearing loss on low frequency on audiology, recurrent bilateral retinal branch artery occlusions, and small multiple areas of signal hyperintensity in the white and gray matter on brain magnetic resonance T2-weighted images. The clinical course is self-limited and treatment options are not codified. Epileptic seizures, as those in our patient, may extend the clinical spectrum of susac syndrome. This case also documents the possibility of multiphasic disease course.
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5/19. Audiometric findings of patients with migraine-associated dizziness.

    OBJECTIVE: The objective of this study was to determine whether the audiometric findings of migraine-associated dizziness could be used to better distinguish migraine-associated dizziness from Meniere's disease. STUDY DESIGN: A retrospective chart review. SETTING: Tertiary, otology/neurotology practice. patients: Two groups of patients were studied, a migraine-associated dizziness and a Meniere's disease group. There were 76 and 34 patients in the migraine-associated dizziness and Meniere's disease groups, respectively. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Initial and follow-up pure-tone average and low-frequency pure-tone average were recorded for both groups. Independent samples t tests were used to test for mean differences in pure-tone average and low-frequency pure-tone average. RESULTS: Pure-tone average and low-frequency pure-tone average were significantly worse for patients in the Meniere's disease group at both the initial and follow-up assessments. Three patients in the migraine-associated dizziness group had an elevated pure-tone average (>/=26 dB) and/or low-frequency pure-tone average at initial and/or follow-up assessment. The remaining 73 migraine-associated dizziness patients had normal hearing. In the Meniere's disease group, only two patients had a normal pure-tone average and low-frequency pure-tone average at both initial and follow-up evaluations. The hearing difference between the two groups was significant even when controlling for age and duration of dizziness symptoms. CONCLUSION: Audiometric findings of patients with migraine-associated dizziness are most often normal. Unlike Meniere's disease, the sensorineural hearing loss in migraine-associated dizziness rarely progresses. These audiometric findings may help to distinguish migraine-associated dizziness from Meniere's disease when diagnostic ambiguity exists between these two diagnoses.
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6/19. Symptomatic high frequency/acceleration vestibular loss: consideration of a new clinical syndrome of vestibular dysfunction.

    CONCLUSION: Symptomatic high frequency/acceleration vestibular loss is a distinct clinical entity that can be missed on conventional ENG with caloric testing. Under certain circumstances, symptomatic patients with a high frequency/acceleration vestibular loss should undergo an MSSC study for confirmation, if required. OBJECTIVE: To document that normal electronystagmography (ENG) with conventional bithermal caloric testing is inadequate for diagnosing clinically significant high frequency/acceleration vestibular loss. MATERIAL AND methods: patients with clinical symptoms and signs of persistent peripheral vestibular dysfunction despite normal conventional bithermal caloric testing on ENG underwent high frequency/acceleration horizontal magnetic scleral search coil (MSSC) eye movement studies. The clinical findings and results from audiometric tests, conventional ENG with bithermal caloric tests and MSSC tests were reviewed. RESULTS: Eleven patients were identified as having an abnormal MSSC study, indicating a high frequency/acceleration vestibular loss consistent with their clinical history despite normal or equivocal bithermal caloric responses on conventional ENG. Although valuable, ENG caloric testing evaluates lateral semicircular canal function and should be considered a non-physiological test primarily of low frequency vestibular function. High frequency/acceleration head thrust testing clinically detected a "high frequency/acceleration vestibular loss" in 8/11(72.7%) cases.
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keywords = high frequency, frequency
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7/19. Management of superior canal dehiscence syndrome with extensive skull-base deficiency.

    Superior canal dehiscence syndrome is a recently described condition resulting in noise- or pressure-induced vertigo. We review the case of a 50-year-old woman who presented with debilitating pressure and noise-induced vertigo as well as a low-frequency conductive hearing loss. Imaging was consistent with superior semicircular canal dehiscence syndrome. An extradural middle fossa approach was used to approach the dehiscent superior canal. Intraoperatively, our patient was found to have extensive idiopathic skull base dehiscence of the temporal floor. Middle ear and mastoid mucosa was exposed with focal areas of dura prolapsed into the mastoid cavity. Because of these findings, temporalis fascia and bone pate were used to cover the dehiscent canal as well as a large area of the temporal floor. Additionally, a temporalis muscle flap was rotated between the dura and the dehiscent temporal floor to reconstruct the middle fossa skull base and prevent encephalocele.
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8/19. Posturography can be used to screen for primary orthostatic tremor, a rare cause of dizziness.

    OBJECTIVE: Primary orthostatic tremor is a rare neurologic condition of unknown origin characterized by a 10- to 20-Hz tremor in the legs while standing. patients with primary orthostatic tremor usually complain of dizziness and unsteadiness that is relieved if they sit down or start to walk around. These patients might be referred to neurotology clinics. Previously, the only way to make the diagnosis has been by means of surface electromyographic recordings from the lower limbs during standing. The authors wanted to study whether posturography can be used to screen for primary orthostatic tremor. STUDY DESIGN: Retrospective case review. SETTING: Balance clinic at a tertiary referral center. patients: From September 2000 to August 2002, 701 patients were investigated at the authors' balance clinic. INTERVENTION: Static posturography on a force platform with 50-Hz sampling frequency. Recordings were made during 120 seconds of quiescent stance with open or closed eyes. MAIN OUTCOME MEASURE: Fast-Fourier transformation (FFT) analysis of recorded torque in the fore-to-aft direction to quantify the spectral power distribution in the range of 0 to 25 Hz. Visual inspection of power spectrum plots was performed. RESULTS: Five patients (0.7%) were identified (1 man and 4 women; mean age, 56 yr; age range, 36-73 yr) with narrow peaks at 8.5 to 18 Hz in the power spectra of their posturographic recordings. Surface electromyographic recordings during standing confirmed their diagnosis of primary orthostatic tremor. CONCLUSION: Analysis of power spectra from posturographic recordings is a simple, quick method to screen for primary orthostatic tremor. The condition might be more common than previously assumed. It is important to identify patients with primary orthostatic tremor because they might be misdiagnosed and left without treatment.
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9/19. Behavioral treatment of dizziness secondary to benign positional vertigo following head trauma.

    Benign positional vertigo (BPV) represents a challenge to rehabilitation due to the subjective nature of the complaint of dizziness, frequent failure of pharmacologic intervention, and complicating psychologic factors. Behavioral therapy was used to treat a 26-year-old woman who complained of debilitating dizzy spells after mild head injury sustained in a motor vehicle accident. During a three-week baseline period before treatment, the patient reported a weekly average of 48 dizzy spells, which prevented participation in independent activities and kept her homebound and psychologically distressed. Nine-week behavioral treatment included biofeedback-assisted relaxation training, psychologic counseling, gaze-fixation practice, desensitization exercise, and generalization training. This protocol has been used successfully to train aviators to combat vertigo and nausea in flight. Our regimen included hourly recording of physical activity, notation of frequency of dizzy spells, and use of behavioral methods during nine weeks. The patient reported 90% reduction in dizzy spells and full resumption of independent activities including driving and athletics. Results are discussed in the context of behavioral problems associated with BPV and application of behavioral methods to the complaint of dizziness.
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10/19. water may cure patients with meniere disease.

    OBJECTIVES/HYPOTHESIS: We examined whether sufficient water intake is effective in the long-term control of vertigo and hearing activity in patients with meniere disease (MD) for whom conventional therapy has proven unsuccessful. STUDY DESIGN: The authors conducted a time-series study with historical control. methods: Eighteen patients with MD in group 1 drank 35 mL/kg per day of water for 2 years. Twenty-nine patients with MD treated with the conventional dietary and diuretic therapy for more than 2 years during 1992 to 1999 at the same hospital were enrolled in a historical control of group 2. RESULTS: patients in group 1 dramatically relieved vertigo and significantly improved in the hearing of the worst pure-tone average of three frequencies (0.125, 0.25, and 0.5 kHz) (low PTA) during the last 6 months of the study period. In contrast, patients in group 2 became worse in both the four- (0.5, 1, 2, and 4 kHz) frequency PTA and the low PTA, although their vertigo did improve. The number of patients whose hearing were improved, unchanged, and worse were 4, 12, and 2 in group 1 and 2, 11, and 16 in group 2, respectively. CONCLUSION: Deliberate modulation of the intake of water may be the simplest and most cost-effective medical treatment for patients with MD. Larger studies will be needed to confirm these results in a larger patient cohort.
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