Cases reported "Hearing Loss"

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1/39. Sudden unilateral hearing loss with simultaneous ipsilateral posterior semicircular canal benign paroxysmal positional vertigo: a variant of vestibulo-cochlear neurolabyrinthitis?

    We describe 4 patients who all simultaneously developed a sudden total or partial unilateral sensorineural hearing loss and an unusual acute peripheral vestibulopathy in the same ear characterized by posterior semicircular canal benign paroxysmal positional vertigo with intact lateral semicircular canal function. Two patients also had ipsilateral loss of otolith function. The vertigo resolved in all 4 patients after particle-repositioning maneuvers. The findings of audiometry and vestibular tests indicated that the lesion responsible for this syndrome was probably located within the labyrinth itself rather than within the vestibulocochlear nerve and that it was more likely a viral vestibulocochlear neurolabyrinthitis than a labyrinthine infarction.
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2/39. Meniere's disease in the elderly.

    OBJECTIVES: To assess the prevalence, presentation, treatment, and evolution of Meniere's disease in elderly patients (> or = 65 yr old). STUDY DESIGN: Retrospective clinical study and case report. SETTING: A neuro-otology referral center at the university hospital in Bern, switzerland, and a neurotologic practice in Geneva, switzerland. patients: patients were selected from the hospital clinic and otolaryngologic practice registers. Among 8423 neurotologic checkups performed between 1988 and 1998, 432 (5.1%) patients had definite Meniere's disease and 66 (15.3%) of these patients were > or = 65 years old. INTERVENTION: The files of these 66 patients were analyzed. RESULTS: Meniere's disease in the elderly had 2 modes of presentation: reactivation of longstanding Meniere's disease (40.9%) and de novo Meniere's disease (59.1%), where the first dizzy spells, tinnitus, and hearing loss appeared after 65 years of age. Drop attacks occurred in 11.1 and 25.6% of the cases, respectively, and could be responsible for misdiagnosis of strokes of the brainstem. The patients were treated by mild antivertiginous drugs (betahistine, cinnarizine). Twenty-seven patients (41%) underwent surgery: transtympanic ventilation tubes (19 patients), sacculotomy (4 patients), vestibular neurectomy (3 patients), surgical labyrinthectomy (1 patient). The vertigo spells disappeared in 73.3 to 100% of the cases according to the type of surgery performed. CONCLUSION: Our study shows that Meniere's disease in the elderly is not at all uncommon. It can appear as a de novo disease or as a reactivated longstanding disease. Drop attacks are more frequent than reported in general patient populations with Meniere's disease and can mimic a stroke of the brainstem. Medical and surgical treatments have to be cautious because many of these elderly patients are fragile.
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3/39. Radical scavengers for Meniere's disease after failure of conventional therapy: a pilot study.

    OBJECTIVE: To perform a trial to assess the efficacy of radical scavengers, i.e. rebamipide, vitamin C and glutathione, for the treatment of Meniere's disease (MD). MATERIAL AND methods: Rebamipide (300 mg/day), vitamin C (600 mg/day) and/or glutathione (300 mg/day) were given orally for at least 8 weeks to 25 patients with poorly controlled MD. RESULTS: Of 22 patients, 21 showed marked improvement of vertigo; 12/27 ears showed improvement of hearing disorders; 17/27 ears showed improvement of tinnitus; and 18/25 patients showed improvement of disability. CONCLUSION: This study suggests that treatment using radical scavengers has the potential to become an effective new therapy for MD.
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keywords = vertigo
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4/39. Familial early-onset progressive vestibulopathy without hearing impairment.

    A family with early-onset vestibulopathy is presented. The 34-year-old father had experienced brief attacks of vertigo and currently suffers from unsteadiness and oscillopsia during head movements. His two young sons also experience brief attacks of spontaneous vertigo. In vestibular testing all three subjects showed reduced caloric responses. However, only the father showed reduced otolith function (as reflected by the vestibular-evoked myogenic potentials). Further, all three subjects had walked before the age of 1 year and none of them had had any auditory symptoms. It is suggested that they have familial early-onset progressive vestibulopathy affecting the canals before the otoliths but sparing cochlear function.
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5/39. Auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction.

    OBJECTIVES: To investigate the clinical and radiological features of patients presenting with an acute auditory syndrome as a prodromal symptom of anterior inferior cerebellar artery (AICA) infarction. methods: 16 consecutive cases of AICA infarction diagnosed by brain magnetic resonance imaging completed a standardised audiovestibular questionnaire and underwent a neuro-otological evaluation by an experienced neuro-otologist. RESULTS: Five patients (31%) had an acute auditory syndrome as a prodrome of AICA infarction one to 10 days before onset of other brain stem or cerebellar symptoms. Two types of acute auditory syndrome were found: recurrent transient hearing loss with or without tinnitus (n = 3), and a single episode of prolonged hearing loss with or without tinnitus (n = 2). The episodic symptoms were brief, lasting only minutes. The tinnitus preceding the infarction was identical to the tinnitus experienced at the time of infarction. At the time of infarction, all patients developed hearing loss, tinnitus, vertigo, and ipsilateral hemiataxia. The most commonly affected site was the middle cerebellar peduncle (n = 5). Four of the five patients had incomplete hearing loss and all had absence of vestibular function to caloric stimulation on the affected side. CONCLUSIONS: Acute auditory syndrome may be a warning sign of impending pontocerebellar infarction in the distribution of the AICA. The acute auditory syndrome preceding an AICA infarct may result from ischaemia of the inner ear or the vestibulocochlear nerve.
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6/39. Horizontal canal benign paroxysmal positioning vertigo with ipsilateral hearing loss.

    The etiology of benign paroxysmal positioning vertigo (h-BPPV) of the horizontal semicircular is unknown. Insight was obtained from two patients with h-BPPV and associated hearing impairment. Based on the different inner ear lesion patterns in neurolabyrinthitis contrary to ischemic labyrinthine infarction we assessed multiple vestibulo-cochlear functions for the first time in two patients who suffered from h-BPPV with sudden unilateral hearing loss. While in patient no. 1 the lesion pattern (posterior canal paresis in addition to the sudden hearing loss) could possibly be caused by ischemia of the common cochlear artery, the lesion pattern of patient no. 2 (hearing loss and ipsilateral paresis of the posterior and horizontal semicircular canal) exceeds the typical vascular labyrinthine territories and may indicate viral neurolabyrinthitis.
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keywords = vertigo
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7/39. Transcanal labyrinthectomy in childhood: report of a case.

    vertigo in children is caused by several factors. One such factor is viruses (e.g., mumps, measles, and rubeola) afflicting the inner ear by various mechanisms. In the majority of cases, affected patients improve with clinical therapy and vestibular rehabilitation. We present the case of a child with vertigo as a sequela of mumps virus infection that did not improve with clinical treatment and vestibular rehabilitation. Consequently, the child was submitted to a transcanal labyrinthectomy followed by vestibular rehabilitation, with significant postprocedure improvement of symptoms. We discuss surgical indications, the technique used in this case, and the importance of vestibular rehabilitation in the postoperative period.
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keywords = vertigo
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8/39. Labyrinth dysfunction 8 months after cochlear implantation: a case report.

    OBJECTIVE: Investigate whether the cochleostomy is a possible port of entry for pneumolabyrinth and a resulting vertigo in patients provided with a cochlear implant. STUDY DESIGN: Retrospective case review. SETTING: Ludwig-Maximilians University of Munich, Hospital Grosshadern. PATIENT: 62-year-old patient who underwent implantation of a HiFocus II cochlear implant with positioner from Advanced bionics (CLARION). Eight months postoperatively, the patient reported rotatory vertigo and right-side tinnitus after he had blown his nose harder than usual during an episode of rhinitis. INTERVENTIONS: Preoperative and postoperative testing of both the petrosal bone with a CT scan and of balance function. MAIN OUTCOME MEASURE: air inclusion in the labyrinth. RESULTS: In contrast to the preoperative high resolution computed tomography (CT) scan, air inclusion was seen in the labyrinth during the episode of vertigo. At the same time, balance function tests with Frenzel glasses revealed both spontaneous and provoked horizontal nystagmus to the right side. At follow-up 8 weeks later, the level of vertigo had significantly decreased. Twelve months later, the control CT showed the cochlear implant positioned correctly and no visible air in the labyrinth. CONCLUSION: It is known that placement of the HiFocus II with Positioner from CLARION requires a relatively large cochleostomy of 1.5 mm. Moreover, in the connective tissue seal between the electrode and the positioner, the latter reaches into the tympanic cavity, and this is possibly the weak point. Further investigation will be needed to determine whether the large cochleostomy with the HiFocus II with positioner increases the predisposition to labyrinth dysfunction.
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keywords = vertigo
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9/39. Differential grading of endolymphatic sac tumor extension by virtue of von hippel-lindau disease status.

    OBJECTIVE: endolymphatic sac tumors are aggressive papillary tumors of the temporal bone frequently associated with von hippel-lindau disease. The goal of this study was to use a newly devised classification system as a means to analyze differences between endolymphatic sac tumor extension in von hippel-lindau disease and non-von hippel-lindau disease patients. methods: Previously reported cases of endolymphatic sac tumor and two new cases were retrospectively reviewed and assigned to a new classification system consisting of four grades based on tumor extent and location. RESULTS: Mean age of 103 patients without von hippel-lindau disease was 52.5 years, whereas in 46 patients with VHL the mean age was 31.3 years. patients with von hippel-lindau disease were more likely to be female (female-to male ratio of 2:1 for von hippel-lindau disease patients versus 1:1 for non-von hippel-lindau disease patients). Symptoms consisted of hearing loss (100% [mean duration, 10 yr] for VHL patients versus 97% [mean duration, 7.8 yr] for non-von hippel-lindau disease patients), facial weakness (38% versus 49%), and tinnitus or vertigo (41% versus 60%). Bilateral tumors were common in von hippel-lindau disease patients (28% versus 1%). Tumors in von hippel-lindau disease patients were significantly more likely to be lower grade than tumors in non-von hippel-lindau disease patients (Grade I, 40% versus 25%; Grade II, 50% versus 58%; Grade III, 8% versus 14%; and Grade IV, 2% versus 4%; p < 0.05). Before 1988, there were relatively fewer Grade I (15% versus 33%) and relatively more Grade II (69% versus 47%) endolymphatic sac tumors in non-von hippel-lindau disease patients than after 1988. CONCLUSIONS: Increased usefulness of intracranial imaging since 1988 has led to the diagnosis of sporadic endolymphatic sac tumors with lower grades. Surveillance imaging in von hippel-lindau disease may account for the greater proportion of endolymphatic sac tumors diagnosed with lower grades. endolymphatic sac tumors associated with a diagnosis of von hippel-lindau disease appear to affect a younger population of patients than non-von hippel-lindau disease cases and occur in women twice as often as in men when associated with von hippel-lindau disease. In addition, tumors are more frequently bilateral and less advanced in the von hippel-lindau disease patient as opposed to the non-von hippel-lindau disease patient.
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keywords = vertigo
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10/39. Subarcuate venous malformation causing audio-vestibular symptoms similar to those in superior canal dehiscence syndrome.

    OBJECTIVE: To present a patient with symptoms similar to those of superior canal dehiscence syndrome due to another cause. STUDY DESIGN: Case report. SETTING: University hospital, tertiary referral center. PATIENT: The 65-year-old woman had suffered for 4 years from hearing loss, tinnitus, and pressure-induced vertigo. INTERVENTION: Audio-vestibular testing, high-resolution computed tomography, and magnetic resonance angiography. MAIN OUTCOME MEASURE: The superior canal dehiscence syndrome is caused by failure of normal postnatal bone development in the middle cranial fossa leading to absence of bone at the most superior part of the superior semicircular canal. The typical features for this syndrome are sound and pressure-induced vertigo with torsional eye movements, pulse synchronous tinnitus and apparent conductive hearing loss in spite of normal middle ear function. We present a patient with very similar symptoms and findings, who instead had a superior canal dehiscence close to the common crus. Neuroradiologic findings suggested that the dehiscence was related to a venous malformation. CONCLUSIONS: Symptoms and findings suggesting superior canal dehiscence syndrome can have a different cause.
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