Cases reported "Endolymphatic Hydrops"

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1/6. Investigation of endolymphatic hydrops by electrocochleography in patients with Cogan's syndrome.

    We compared auditory and vestibular function between a patient with typical Cogan's syndrome and a patient with atypical Cogan's syndrome. Repeat audiograms demonstrated fluctuating sensorineural hearing loss in the affected ear. Brainstem auditory evoked response testing revealed no abnormalities. Hallpike caloric testing showed a decreased response in the affected ear in the typical case and responses within normal limits in the atypical case. Sinusoidal harmonic acceleration was normal in the typical case, and there was an abnormal phase at 0.01 Hz in the atypical case. endolymphatic hydrops was investigated by electrocochleography. In both patients, there was an enhancement of the summating potential, with an increased ratio of summating potential to action potential amplitude. This finding is consistent with endolymphatic hydrops.
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2/6. Changes in the Tullio phenomenon and the fistula sign in the course of endolymphatic hydrops.

    We performed electronystagmography with caloric stimulation and studied vestibular evoked myogenic potentials (VEMPs) associated with changes in the Tullio phenomenon and the fistula sign during the clinical course of a patient with endolymphatic hydrops. The Tullio phenomenon and the fistula sign disappeared in association with a reduction in the caloric response, which implicates the ampullary function of the lateral semicircular canal. Even when no VEMP could be detected for the affected ear, the Tullio phenomenon and fistula signs were observed; thus, either these phenomena had a lower response threshold than the VEMPs, or saccular receptors were not involved in the Tullio phenomenon.
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3/6. Three cases of cochleosaccular endolymphatic hydrops without vertigo revealed by furosemide-loading vestibular evoked myogenic potential test.

    OBJECTIVE: To describe possible cases of cochleosaccular endolymphatic hydrops without vertigo. STUDY DESIGN: Retrospective case report. SETTING: University hospital. patients: Three patients with possible cochleosaccular hydrops without vertigo were studied. The basis of diagnosis was positive result of the furosemide-loading vestibular evoked myogenic potential test, no canal paresis in the caloric test, and recurrent cochlear symptoms or fluctuating low-tone hearing loss. CASE REPORT: In case 1, a 47-year-old woman had recurrent left aural fullness and tinnitus and a few weeks later complained of a floating sensation and could not stand up. The furosemide-loading vestibular evoked myogenic potential test showed a positive result in the left ear. In case 2, a 24-year-old woman complained of a backward falling sensation lasting several seconds; subsequently, a severe floating sensation persisted and she could not stand up for several days. Audiography showed fluctuating low-tone hearing loss in the left ear, and the furosemide-loading vestibular evoked myogenic potential test showed a positive result. In case 3, a 41-year-old woman had a floating sensation while walking and subsequently complained of tinnitus in the left ear. She could not stand up because of a severe floating sensation and, moreover, complained of a sudden falling sensation lasting for several seconds. The furosemide-loading vestibular evoked myogenic potential test indicated a positive result in the left ear. CONCLUSIONS: The patients in cases 2 and 3 complained of a short-lasting sensation of falling down. Severe disequilibrium that prohibited standing up was noted in all cases. It was suggested that these symptoms were caused by saccular hydrops.
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4/6. Drop attacks in elderly patients secondary to otologic causes with Meniere's syndrome or non-Meniere peripheral vestibulopathy.

    Many neurologists are unaware of the drop attack that may occur from an inner ear dysfunction especially in elderly. We studied the clinical features and results of quantitative audiovestibular tests in six elderly patients (> or =65 years of age) who presented with drop attacks attributable to an inner ear pathology. Group was divided into Meniere's syndrome (4) or non-Meniere peripheral vestibulopathy (2). Standard dizziness questionnaire and quantitative audiovestibular function testing were performed. Episodes were described as a sudden push to the ground in four or a violent illusionary movement of environment leading to a fall in two. All cases gave a history of prior vertiginous episodes and vestibular testing revealed unilateral caloric paresis. Ipsilateral hearing loss was documented in four cases. Our results suggest that otologic causes should be considered in the differential diagnosis of the drop attack in elderly, even if the symptoms and signs were not consistent with Meniere's syndrome.
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5/6. Intratympanic gentamicin treatment: preliminary results in two patients with Meniere's disease.

    Meniere's disease is a vestibular disturbance characterized by episodic vertigo, tinnitus, and fluctuant hearing loss. The long-term effectiveness of available medical and nonablative surgical treatments has been variable, with frequent symptom recurrence. Vestibular neurectomy and labyrinthectomy, surgically ablative techniques, provide more permanent relief from vertiginous attacks. However, these procedures pose possible morbidity and cochlear risk. In this paper, preliminary results are presented for two patients who underwent intratympanic gentamicin application. Like surgical labyrinthectomy, intratympanic use of gentamicin is intended as an ablative procedure, but with potentially less risk to hearing. More vestibulotoxic than cochleotoxic, gentamicin initially disrupts the endolymph-secreting vestibular dark cells, thereby preventing endolymphatic hydrops development. Following gentamicin application, both patients demonstrated a significant change in peripheral vestibular function, as characterized by a reduction of caloric response, impaired posturography performance, and reduced low-frequency gain on rotary chair testing. Posturography performance subsequently improved, confirming functional compensation. Both patients reported relief from vertiginous attacks. However, word recognition ability was significantly worse in one of these two patients. Changes in pure-tone thresholds were minimal.
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6/6. mumps labyrinthitis, endolymphatic hydrops and sudden deafness in succession in the same ear.

    Acute sensorineural hearing loss, mostly unilateral and reversible, is a well-known complication to mumps. Secondary endolymphatic hydrops, Meniere's syndrome, has rarely been associated with a previous mumps infection. This paper presents the case report of a woman who experienced unilateral hearing loss, vestibular symptoms and a caloric depression on the same ear during mumps. The symptoms and findings were reversible. Twelve years later she developed Meniere's symptoms in the same ear. This continued for 2 years after which she suddenly had a sensorineural hearing loss. This was localized in the mid- and high-tone area and was almost identical with the initial hearing loss 14 years earlier. Viral damage to the resorptive structures of the inner ear seems to have caused the hydrops. It also seemed to have weakened the neuronal structures of the ear, letting the initial damage become overt after repeated attacks of pathological pressure changes.
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