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1/114. Anti-labyrinthine antibodies in a patient with relapsing polychondritis.

    Relapsing polychondritis is a rare inflammatory disease that causes destruction of cartilaginous tissue in various anatomical regions. We report here about a 55-year-old female patient with relapsing polychondritis that involved the right auricle, both audiovestibular organs and both eyes. The patient presented with persisting inflammation of the right auricle, sudden lower-frequency hearing loss, acute moderate vertigo with nausea and mild ocular symptoms. Immunofluorescence assays were used for the detection of antibodies against the cochlea and the vestibular organ and demonstrated the presence of circulating antibodies against the audiovestibular organ. No staining for anti-corneal IgG was detected. Improvement of clinical disease was achieved by treatment with systemic steroids and vasodilator drugs, and long-term medication with low-dose corticosteroids.
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ranking = 1
keywords = vertigo
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2/114. Solitary plasmacytoma of the skull base presenting with unilateral sensorineural hearing loss.

    Solitary plasmacytoma of the skull base is a rare entity with only a few reported cases in the literature. We review the literature and present our experience with this lesion that produced ipsilateral sensorineural hearing loss, vertigo and ipsilateral sixth nerve palsy.
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keywords = vertigo
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3/114. Intraoperative mitomycin C in endolymphatic sac surgery for Meniere's disease: A pilot study.

    The long-term results of endolymphatic sac (ES) shunt surgery in Meniere's disease have been reported to be unsatisfactory compared to the short-term results, probably because of fibrosis and/or reclosure of the incised ES. To solve this problem, we tried to apply mitomycin C (MMC) intraoperatively to the incised ES as used in trabeculectomy for glaucoma. MMC has an antiproliferative effect as well as an antineoplastic effect. Thus, it is expected that MMC would have a benefit to prevent rapid fibrosis and/or reclosure of the ES. MMC did not show any ototoxicity in our animal study and we started a clinical trial after being approved by the ethics Committee for Human research at our university. Fourteen patients with Meniere's disease underwent ES mastoid shunt surgery with intraoperative application of MMC and they were followed up more than 6 months. No patients experienced vertigo, although some complained of slight dizziness. Five patients out of 14 showed remarkable hearing improvement by more than 10 dB, accompanied with decrease of tinnitus.
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ranking = 1
keywords = vertigo
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4/114. Cogan's syndrome: unsuccessful outcome with early combination therapy.

    Interstitial keratitis and vestibuloauditory symptoms (vertigo and hearing loss) are the typical signs of Cogan's syndrome, a rare inflammatory vascular disease. Signs of vasculitis in many organ systems may appear, among which neurologic problems are sometime predominant. The efficacy of glucocorticoids on the ocular and systemic symptoms is established, but their effect on hearing loss is unknown. We describe a case of Cogan's syndrome with neurological involvement in which early treatment with combination therapy (prednisolone and cyclosporin) failed to bring ear inflammation under control.
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ranking = 1
keywords = vertigo
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5/114. Haemorrhagic acoustic neuroma with features of a vascular malformation. A case report.

    A 55-year-old man with hearing loss presented with vertigo and vomiting. CT tomography and MRI demonstrated a cerebellopontine angle mass with foci of haemorrhage. An angiomatous tumour, with large abnormal veins adhering to the capsule, was completely removed. Histologically, the tumour was an acoustic neuroma with abnormal vascularisation and limited intratumoral haemorrhage.
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ranking = 1
keywords = vertigo
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6/114. Chemical labyrinthectomy and cochlear implantation for Meniere's disease--an effective treatment or a last resort?

    The surgical management of Meniere's disease presents a difficult and controversial problem and includes various procedures ranging from 'placebo operations' to vestibular nerve section. The situation is even more complex in the case where the only hearing ear is affected. We present a case of severe debilitating vertigo due to Meniere's disease that was treated by chemical labyrinthectomy and cochlear implantation. This unique management strategy allowed restoration of useful hearing and relief of vertigo.
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ranking = 2
keywords = vertigo
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7/114. An unusual complication of cochlear implant: benign paroxysmal positional vertigo.

    Three days after the initial fitting of the cochlear device a 40-year-old woman complained of severe rotational vertigo following head movements associated with neurovegetative symptoms. Otoneurological evaluation revealed a horizontal paroxysmal positional nystagmus beating towards the lowermost ear, induced by rolling the patient's head from supine both to the right or to the left lateral position suggesting the diagnosis of benign paroxysmal positional vertigo of the left horizontal semicircular canal. The nystagmus characteristics were the same whether the cochlear device was switched on or off. The hypothesis of an otolith dislodging due to the electrical stimulation during the initial fitting is discussed.
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ranking = 6
keywords = vertigo
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8/114. Neurotologic follow-up after radiation of posterior fossa tumors.

    OBJECTIVE: Stereotactic radiation treatment, also known as gamma knife surgery or radiosurgery, has come into acceptance as a treatment alternative to surgical removal for posterior fossa tumors. The purpose of this article is to describe the role of the neurotologist in the optimal management of neurotologic complications after stereotactic radiation, as illustrated by five patients. STUDY DESIGN: Retrospective chart review. patients: Five patients who underwent stereotactic radiation of posterior fossa tumors. MAIN OUTCOME MEASURES: Presence or absence of neurotologic complications (tumor growth, hearing loss, imbalance/ataxia, vertigo, and facial paralysis) or neurosurgical complaints (facial numbness, motor weakness, headache, hydrocephalus, and subarachnoid cysts). RESULTS: Postradiation neurotologic complaints included vertigo, imbalance/ataxia, and progressive hearing loss in four of the five patients. Continued tumor growth occurred in two patients; two patients had no growth; in one patient the tumor became smaller. The complications of facial nerve paralysis, facial numbness, motor weakness, headache, hydrocephalus, cerebellar edema, and posterior fossa arachnoid cyst formation occurred less frequently. CONCLUSIONS: Stereotactic radiation of posterior fossa tumors can produce significant neurotologic problems. It is imperative that neurotologists remain involved in the follow-up care of patients with posterior fossa tumors to offer optimal treatment alternatives for the neurotologic disorders.
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ranking = 2
keywords = vertigo
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9/114. 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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ranking = 2
keywords = vertigo
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10/114. What inner ear diseases cause benign paroxysmal positional vertigo?

    Benign paroxysmal positional vertigo (BPPV) originating from the posterior semicircular canal (pSCC) is a common vestibular disorder that is easy to diagnose and usually easy to treat. The majority of patients with BPPV have no known inner ear disease; they have "primary" or "idiopathic" BPPV. However, a minority does have objective evidence of an inner ear disease on the same side as the BPPV and this group has "secondary" or "symptomatic" BPPV. Previous publications differ on the prevalence of secondary BPPV and about the types of inner ear diseases capable of causing it. In order to determine what proportion of patients have secondary as opposed to primary BPPV and which inner ear diseases are capable of causing secondary BPPV, we searched our database for the 10-year period from 1988 to 1997 and found a total of 2847 patients with BPPV. Of these, 81 (3%) had definite pSCC-BPPV secondary to an ipsilateral inner ear disease. Sixteen had Meniere's disease, 24 had an acute unilateral peripheral vestibulopathy, 12 had a chronic unilateral peripheral vestibulopathy, 21 had chronic bilateral peripheral vestibulopathy and 8 had unilateral sensorineural hearing loss. It seems that any inner ear disease that detaches otoconia and yet does not totally destroy pSCC function can cause BPPV and that a case can be made for audiometry and caloric testing in all patients with BPPV.
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ranking = 5
keywords = vertigo
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