Cases reported "Endolymphatic Hydrops"

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1/10. A possible case of saccular endolymphatic hydrops.

    The vestibular-evoked myogenic potential (VEMP) is expected to be one of the examinations for saccular function. We report a case of dysequilibrium possibly due to a saccular disorder. A 27-year-old female came to our clinic complaining of severe recurrent dysequilibrium spells for few hours on September 21, 1998. The first attack was only vertigo with bilateral tinnitus for 8 days before. She did not show possible vestibular findings on a routine vestibular test. An electrochochleogram showed the dominant negative summating potential (SP) on both sides, and the furosemide test showed negative findings on both sides. A VEMP test indicated a low amplitude in the right ear, which improved to the normal response after furosemide injection. The amplitude was increased by furosemide administration even in the left ear. These findings suggested bilateral endolymphatic hydrops in the cochlea and saccule especially on the right side. The VEMP test may reveal saccular hydrops, which differs from the conventional Meniere type vertigo.
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2/10. Delayed endolymphatic hydrops: study and review of clinical implications and surgical treatment.

    Delayed endolymphatic hydrops (DEH) differs from Meniere's disease in that it occurs in pre-existing ear pathology in patients who have a profound unilateral or total deafness that was caused by infection, trauma, or unknown causes during childhood or adulthood. We performed a retrospective review of 160 patients with ipsilateral, contralateral, or bilateral DEH. Eighty-seven patients who did not respond to medical therapy underwent surgical treatment. Our findings indicate that the more conservative surgical procedures--endolymphatic sac surgery, cochleosacculotomy, and streptomycin perfusion--are all as effective as and less destructive than labyrinthectomy for controlling vertigo. The clinical results of this study would seem to support the observations of others that DEH and Meniere's disease are related disorders caused predominantly by cases of viral labyrinthitis with unknown etiology.
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3/10. 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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4/10. 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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5/10. role of autoimmunity in contralateral delayed endolymphatic hydrops.

    Contralateral delayed endolymphatic hydrops is described as the development of fluctuating hearing loss and episodic vertigo in a normal ear years following sensorineural hearing loss in the opposite ear. This condition is a variant of delayed endolymphatic hydrops in which new symptoms of severe episodic vertigo develop years later in an ear that has lost most or all of its hearing. The possible etiologies of these two conditions are unknown and may be different. This report presents seven cases of the contralateral form of this disease and experimental evidence that suggests that these patients may be suffering from the initiation of an autoimmune event directed against the remaining inner ear. In Western blot analysis, their serum was reacted against cow cochlear inner ear antigen preparations, and six of the seven cases had serum antibodies directed against a 68 or a 35-36 kilodalton (kd) molecular weight antigen in contrast to three of 43 normal controls (p < .001 Fisher's exact test), the significance of which had previously been reported for autoimmune inner ear disease.
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6/10. Traumatic endolymphatic hydrops.

    Traumatic endolymphatic hydrops is an accumulation of endolymph in the cochlear duct caused by traumatic insult. The causative mechanisms are: (1) fistulization of the bony labyrinth, which causes a disturbance in the normal perilymph-endolymph pressure relationship; (2) direct injury to the membranous labyrinth, which may be just a collection of fluid in the cochlear duct from irritation, resulting in endolymphatic hydrops that may not be progressive and may subside in a short period of time after injury and hearing loss may occur; and (3) injury to the endolymphatic fluid drainage system, including a temporal bone fracture in which the fissure happens to extend through the vestibular aqueduct, causing fibro-osseous blockage of the endolymphatic duct and surgical injury to the saccule with obstruction of the longitudinal flow of endolymph, resulting in endolymphatic hydrops that may be delayed in onset and is usually persistent. The diagnosis of traumatic endolymphatic hydrops is made by a history of trauma, such as barotrauma, a blow to the head, or perhaps a previous ear operation, such as stapedectomy; the presence of typical symptoms of endolymphatic hydrops, including fullness, tinnitus, fluctuant hearing loss, and episodic vertigo; and an elevated negative summating potential and an increased summating potential:action potential ratio by electrocochleography. Three patients are presented to demonstrate this clinical entity.
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7/10. endolymphatic hydrops associated with otosclerosis.

    endolymphatic hydrops (EH) associated with otosclerosis has been noted for many years. However, the causal relationship of these two entities remains controversial. Having reviewed the records of patients with otosclerosis describing fluctuant hearing loss and vertiginous symptoms, the authors found the EH may coexist with otosclerosis preoperatively; they may be two separate diseases that exist coincidentally; or EH may be caused by the otosclerotic process. Secondly, EH may occur with a fistula after surgery. Occurring after stapedectomy, EH may be caused by fistulization of the bony labyrinth, which is effectively treated by surgery to seal the fistula, which may cause EH to subside and hearing to improve. Thirdly, delayed EH may occur months or years after stapedectomy, possibly as a result of otosclerotic foci or surgical insult to the labyrinth. dexamethasone, diuretics, and a room air rebreather can be used in the treatment of delayed EH. Hearing may be maintained or may deteriorate, but there usually is no dizziness. The clinical manifestations of EH associated with otosclerosis include a conductive or mixed type of hearing loss; the presence of fullness, tinnitus, fluctuation of hearing, episodic vertigo, an elevated negative summating potential (SP), and an increased summating potential:action potential (SP:AP) ratio shown by ECoG. This report presents five cases of EH associated with otosclerosis.
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8/10. The possible effect of pregnancy on Meniere's disease.

    There are few reports on the course of Meniere's disease during pregnancy. We report here the clinical course of Meniere's disease before, during and after pregnancy and discuss the possible effect of pregnancy on Meniere's disease and the treatment of Meniere's disease during pregnancy. In the present case, the vertigo attacks increased up to 10 times per month during early pregnancy, when the serum osmolality was significantly below normal at 268 mosm/kg. As the pregnancy proceeded, the serum osmolality normalized and the vertigo attacks decreased in frequency. The vertigo attacks were treated by oral isosorbide and intramuscular injection of low-dose diazepam. The coincidence of the decline in osmolality with the increase in vertigo attacks points to serum osmolality as a possible factor in the effect of pregnancy on Meniere's disease.
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9/10. Treatment of ipsilateral delayed endolymphatic hydrops.

    Nine patients with ipsilateral delayed endolymphatic hydrops were treated. All cases were initially treated by conservative therapy using isosorbide or other drugs and 4 patients have been doing well without surgical intervention, though they occasionally have slight dizziness. Five patients required surgery to control vertigo. Three patients first underwent cochleosacculotomy to maintain the vestibular function in the diseased ear, but vertigo recurred after 6 months in all 3 cases. These 3 patients and another patient who wanted definitive treatment right from the start underwent transmastoid labyrinthectomy which resulted in complete control of vertigo. One patient received chorda tympani nerve section because he did not want his vestibule destroyed. After the nerve section, he has had no vertigo though sometimes dizziness. Transmastoid labyrinthectomy seems to be the best treatment for ipsilateral delayed endolymphatic hydrops.
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10/10. diagnosis of cochlear Meniere's disease with electrocochleography.

    The existence of cochlear Meniere's disease, once considered a variant of classic Meniere's disease but without vertigo, has been questioned due to lack of objective evidence that endolymphatic hydrops is involved with the disease process. Transtympanic electrocochleography (TT ECoG) has emerged as a useful tool for electrophysiologic monitoring of the inner ear, and is especially valuable in assessing endolymphatic hydrops. A retrospective chart review was performed to identify those patients with a diagnosis consistent with cochlear Meniere's disease in order to determine the presence or absence of endolymphatic hydrops using TT ECoG. A total of 7 patients were identified with at least a 2-year follow-up. Using established norms for the summating potential to action potential ratio with click stimulus, 67% of the ears examined demonstrated values consistent with endolymphatic hydrops. Fluctuating aural pressure and tinnitus were present in all patients and medical therapy of diuretics and salt restriction seemed to stabilize or improve the condition in about 80% of the patients. Theoretical considerations are discussed, and a case history of 1 of the study patients is presented to illustrate a typical example of this variant of Meniere's disease.
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