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1/14. Unilateral deafness with subsequent vertigo.

    The association between the characteristic clinical picture of endolymphatic hydrops and pre-existent profound sensori-neural hearing loss of long duration, has not been previously reported. Several cases are reported to illustrate this clinical syndrome. Labyrinthectomy was found to be curative. A plea is made for better recognition of this clinical entity.
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2/14. Delayed endolymphatic hydrops and its relationship to Meniere's disease.

    Delayed endolymphatic hydrops (EH) can be characterized as having ipsilateral and contralateral types. They are similar in that both have early and late phases of otologic symptoms and that the early phase is a profound hearing loss in one ear. The late phases differ, however, in that the ipsilateral type develops the symptoms of EH (episodic vertigo) in the deaf ear and the contralateral type develops the symptoms of EH (fluctuating hearing loss and/or episodic vertigo) in the hearing ear. In more than half the cases of both types of delayed EH, the profound hearing losses in the early phase are simply discovered to be present in early childhood without a known time of onset. The temporal bones of two patients with contralateral delayed EH show pathologic changes in the deaf ears that are similar to those known to occur in mumps and measles labyrinthitis, whereas the pathologic changes in the hearing ears are similar to those known to occur in Meniere's disease. These observations support the proposition that Meniere's disease may occur as a delayed sequela of inner ear damage sustained during an attack of subclinical viral labyrinthitis occurring in childhood.
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3/14. Ultrastructure of the vestibular sensory organs in delayed endolymphatic hydrops.

    Vestibular sensory organs were examined ultrastructurally in two cases of delayed endolymphatic hydrops. The patients, two women, 27 and 16 years of age, suffered profound sensory hearing loss in childhood and experienced severe, recurrent vertigo. Specimens were obtained by a translabyrinthial vestibular nerve transection. We studied the utricular macula and the lateral and posterior cristae in one case, and the utricular macula, the anterior and posterior cristae, and the vestibular ganglion in the other. The otoconia and the otoconial membrane, the sensory epithelia, and the vestibular ganglion appeared fairly normal. Although the entire vestibular end organs were not studied in these cases, it was surprising that the ultrastructural findings did not conclusively identify vestibular end organ pathology as the cause of the vertigo attacks.
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4/14. Vestibular atelectasis.

    The temporal bone collection at the massachusetts eye and Ear Infirmary includes specimens from several cases in which the only reasonable explanation for vertigo is collapse of the walls of the ampullae and utricle, a disorder we have termed vestibular atelectasis. The clinical histories and temporal bone studies support the existence of a primary type that may have a paroxysmal or insidious onset, and a secondary type that occurs in association with other inner ear disorders. The principal clinical symptom is chronic unsteadiness, precipitated or aggravated by head movement, and sometimes associated with short episodes of spinning vertigo. It is presumed that the collapsed membranes interfere with the motion mechanics of the cupulae and otolithic membranes.
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keywords = vertigo
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5/14. Treatment of hydropic patients by immunoglobulin with methyl B12.

    Recently several investigations have been reported suggesting that the cause of endolymphatic hydrops might be an immunologic disorder of the endolymphatic space, including the endolymphatic sac. As the first choice in a conservative treatment by medication, the authors have used a combination of prednisolone and furosemide per os, which is rather safe and effective for hydrops patients in a subacute stage. However, some patients do not respond to this treatment or gradually become immune to this medication. With these patients, we have tried an intravenous administration of immunoglobulin g with methyl B12, expecting a curative effect on immunologic deficiency in the endolymphatic space. Compared with a group of patients without this treatment, the group receiving it showed rather good scores in hearing improvement; however, vertigo and tinnitus remained almost unchanged.
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6/14. Perilymphatic fistula: a histopathologic study.

    Over the last two decades, clinical criteria for perilymphatic fistulae have been defined to the extent that differentiation can be made between such fistulae and other balance-affecting disorders such as Meniere's syndrome. On the assumption that the specimens in the temporal bone bank of the University of chicago Medical School that had been obtained from patients having vertigo, hearing loss, or both, before those clinical criteria were so defined might have been classified incorrectly, we proposed a retrospective histopathologic study, with prediction of two independent variables: a clinical history and physical findings consistent with the diagnosis of perilymphatic fistula and communication between the vestibule and the middle ear adjacent to or via the fissula ante fenestram. Eleven pairs of temporal bones with the histologic diagnosis of idiopathic labyrinthine hydrops were evaluated before the clinical histories relevant to those specimens were reviewed. In one specimen, a communication between the vestibule and the middle ear space was identified. In none of the other specimens was there a similar communication. As this study continued, significance was given to the histologic details of the communication between the middle ear and posterior canal ampulla. The temporal bones without these communications did not have clinical histories consistent with the diagnosis of perilymphatic fistula.
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7/14. endolymphatic hydrops. An overview and classification.

    endolymphatic hydrops (EH) is a pathological condition which is the final common manifestation of a variety of otologic insults. In this paper we develop a classification which, on the basis of clinical and pathological data, distinguishes symptomatic and asymptomatic forms. Clinical case histories and temporal bone studies are presented to illustrate and substantiate this classification. The symptomatic form becomes evident by the hallmark symptoms of episodic vertigo and fluctuating hearing loss. The asymptomatic form is silent. Interconversion from one form to another may occur over time. Each of the forms can be subdivided, according to etiology, into embryopathic, acquired, and idiopathic types. The embryopathic type comprises those cases in which some noxious influence interferes with prenatal labyrinthine development. The acquired type includes those cases in which a documented insult, be it inflammatory or traumatic, is suffered by a previously normal labyrinth. The inflammation may be viral, bacterial, or spirochetal in nature, while the traumatic event may be either accidentally or surgically sustained. The idiopathic type includes cases in which the instigating event precipitating the EH is unknown. Meniere's disease represents merely one example of the EH group of disorders, namely symptomatic idiopathic EH.
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keywords = vertigo
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8/14. Meniere's disease and endolymphatic hydrops: clinical-histopathological correlations.

    The clinical-histopathological correlation between Meniere's disease and endolymphatic hydrops was done to explain, as much as possible, causes of symptoms of Meniere's disease. Twenty-three temporal bones with endolymphatic hydrops from 17 patients were reviewed and clinical and histopathological findings were correlated. Histopathological examination revealed frequent, severe deformities in the labyrinthine walls and permanent changes in Reissner's membranes. Evidence of rupture was difficult to assess. Of 21 ears of patients with the clinical diagnosis of Meniere's disease, 93% had endolymphatic hydrops. A statistical correlation between increased area of the cochlear duct and hearing loss was found. Some correlation was also found between frequency of vertigo and results of electronystagmography with histopathological findings. Consequently, the mechanical effect of endolymphatic hydrops seems to have greater significance in the production of symptoms of Meniere's disease than the biochemical effect of ruptures. In a review of ten unusual cases from this series of 17 patients, traumatic neuromas or remnants of vestibular structures were found after an incomplete labyrinthectomy. Histopathological findings of four patients who had undergone endolymphatic subarachnoid shunt surgery are described.
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9/14. endolymphatic hydrops with absence of vein in paravestibular canaliculus.

    Unilateral endolymphatic hydrops is described associated with absence of the vein in the paravestibular canaliculus (PVC), and with decreased vascularity of the vestibular aqueduct and endolymphatic sac. The venous return from the vestibule was normal as far as the junction of the branches forming the PVC vein. At this junction, a blind venous loop was formed with no continuation of venous drainage through the PVC. This probably represents a developmental anomaly. The decreased vascularity of the endolymphatic sac may be related to the absence of the PVC vein. However, anatomical and functional relationships of these vessels are not clear and need further study. Perisac fibrosis and endosteal bone formation are possibly secondary to the decrease in vascularity. A large chronic rupture of the inferior saccule wall probably accounts for the absence of vertigo and the relatively mild degree of cochlear endolymphatic hydrops.
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keywords = vertigo
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10/14. Delayed endolymphatic hydrops.

    Delayed endolymphatic hydrops is a disease entity that can be differentiated from Meniere's disease. Typically it occurs in patients who have sustained a profound hearing loss in one ear, usually from infection or trauma, and then after a prolonged period of time develop either episodic vertigo from the same ear (ipsilateral delayed endolymphatic hydrops) or fluctuating hearing loss, also sometimes with episodic vertigo, in the opposite ear (contralateral delayed endolymphatic hydrops). The ipsilateral form of the disease may be treated by labyrinthectomy but no satisfactory therapy is available for the contralateral form of the disease.
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keywords = vertigo
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