Cases reported "Labyrinth Diseases"

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1/6. Radiologic diagnosis of labyrinthitis ossificans.

    labyrinthitis ossificans is the pathological ossification of the membranous labyrinthine spaces in response to processes which are destructive of the membranous labyrinth or the endosteum of the otic capsule. It has been primarily a histopathologic diagnosis. Complex motion tomography however, allows a detailed view of the osseous labyrinth and permits the diagnosis in the living state. Radiologic documentation of labyrinthitis ossificans is objective evidence of a process destructive of the membranous labyrinth. It supports the likelihood of an absence of cochlear and vestibular function. It alerts the surgeon to the possible obliteration of key inner ear anatomical landmarks.
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keywords = motion
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2/6. Vertical oscillopsia in bilateral superior canal dehiscence syndrome.

    A patient sought treatment for vertical oscillopsia and impaired vision during locomotion, and unsteadiness of gait. Positive fistula tests and CT of the temporal bones confirmed a diagnosis of bilateral superior canal dehiscence. An impairment of the superior canal vestibulo-ocular reflex, documented by three-dimensional search coil eye movement recordings for oblique (single) and downward pitch head motion (bilateral canal testing), is proposed to induce vertical rather than torsional-vertical oscillopsia during locomotion.
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keywords = motion
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3/6. Meyer zum Gottesberge's head-shaking test for the evaluation of jumbling.

    Jumbling consists of loss of vestibular eye movement reflexes and resultant oscillopsia during movement of the head. This results in a failure of clear vision during head movement. The head-shaking test for evaluation of the jumbling phenomenon was initially suggested by Meyer zum Gottesberge in 1952. In this test, binocular visual acuity is measured while the patient shakes his head at a rate of 2 or 3 movements per second, 10 to 20 degrees horizontally or vertically, and compared head still position. In normal subjects, only a slight decrease in visual acuity is noted due to this head-shaking. A diagnosis of jumbling is made when the visual acuity during head-shaking is less than half the visual acuity when the head is held motionless. When the visual acuity during head movement is expressed as a percentage of the value obtained while the head is motionless, a quantitative evaluation of jumbling is possible. This test should be done at regular intervals especially on patients who receive parenteral administration of ototoxic aminoglycosides for an early detection of jumbling and an appropriate discontinuation of the drugs, along with repeated auditory testing. The test is also useful in monitoring recovery from jumbling.
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keywords = motion
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4/6. 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 = motion
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5/6. Perilymphatic fistulas.

    The oft-quoted dictum that clinical suspicion rather than any specific test leads to the diagnosis is confirmed by our study. Further work is needed to define the vestibular findings more precisely and to work out relationships between CSF and perilymph pressures. temporal bone study will be necessary to document the double membrane break theory. Future study may include analysis of suspected fistula fluid to determine if it represents a mixture of perilymph and endolymph. From our study, fistulae may occur from minimal or no trauma. The presentation is usually subtle. Because no diagnostic test is available to assure correct diagnosis, one must maintain a high index of suspension. diagnosis usually cannot be made until the ear is surgically explored. The low morbidity of an exploratory tympanotomy, coupled with the high positive to negative ratio of exploration and the high degree of successful results, leads the authors to encourage exploration. Indeed, the overall concensus is that many active fistulae remain undiagnosed because of the lack of suspension and the reluctance to explore an ear without a concrete preoperative diagnosis. The duration of the fistulae and the recurrent nature of the problem poses another diagnostic dilemma. The first positive exploratory tympanotomy for perilymphatic fistula often leaves the surgeon with a dichotomy of emotion from pride of a correct diagnosis to fear that perhaps his observations of the minute clear fluid seepage was an error. Reversal of patient symptoms quickly erases such fears and presumptive diagnosis of perilymphatic fistula becomes easier to make. Eventually, one begins to worry, "How many have I missed."
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6/6. Positive Hennebert's sign in Meniere's disease.

    A positive Hennebert's sign was found in 24 of 81 (30%) of ears with Meniere's disease and in none of 22 cases of vestibular schwannoma or 100 normal controls. Negative pressure applied to the external auditory canal was the required stimulus in 14 of 24 (58%) ears. The character of induced eye motion was variable, although horizontal nystagmus was found in 22 of 24 (92%) ears. A positive Hennebert's sign is seen in a variety of labyrinthine lesions and gives corroborative evidence for a cochlear site of hearing impairment with hydrops and vestibulofibrosis are the underlying pathophysiologic condition.
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