Cases reported "Labyrinth Diseases"

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1/7. Traces of perilymph detected in epipharyngeal fluid: perilymphatic fistula as a cause of sudden hearing loss diagnosed with beta-trace protein (prostaglandin D synthase) immunoelectrophoresis.

    The incidence of perilymphatic fistula as cause of sudden hearing loss is not known. We present a case with sudden unilateral hearing loss associated with a positive beta-trace protein test of an epipharyngeal fluid sample. The patient presented with sudden sensorineural hearing loss on the right side. A stapedotomy had been performed nine months previously due to otosclerosis. Intravenous therapy for the treatment of sudden hearing loss was unsuccessful. At the time of sudden hearing loss, epipharyngeal fluid was collected using a Raucocel sinus pack. Investigation using rocket immunoelectrophoresis showed the presence of beta-trace protein. Upon repeating tympanoscopy there was no obvious labyrinthine fluid egress, but the oval window was sealed with fibrin sponge and fibrin glue. The patient's hearing improved over a period of five months.
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2/7. Inner ear extension of vestibular schwannomas.

    OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, italy, in this field. STUDY DESIGN: Case report and literature review. methods: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.
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3/7. 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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4/7. Occult otologic fistulas as a cause of recurrent meningitis.

    OBJECTIVE: Occult perilymph fistulas may be the cause of unexplained non-epidemic meningitis. MATERIAL AND METHOD: To review the case reports of 5 patients (3 females and 2 males aged 4-56) presenting with unexplained meningitis. All had sensorineural hearing loss of variable duration. RESULTS: All patients were submitted to CT, MRI and MRI cisternography. All underwent exploratory tympanotomy to seal the fistula. In all patients the fistula could be located and sealed. All had no further attacks of meningitis and those who had serviceable hearing did not show any further deterioration. CONCLUSION: In any case of recurrent meningitis an occult perilymph leak should be sought. A high degree of suspicion should exist if there are auditory or vestibular symptoms. Detecting and sealing of the defect will protect the patient against further attacks and deterioration of hearing.
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5/7. perilymph fistulae.

    A small series of 14 post-stapedectomy fistulae illustrates the varied aetiology. The long-term competence of the oval window seal may be ensured by making a small hole in the footplate. Contraction of ageing fibrous tissue contributes to late stapedectomy failures. Long-term follow-up is important, for any deterioration in hearing after stapedectomy may result from a perilymph leak.
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6/7. ataxia and hearing loss secondary to perilymphatic fistula.

    ataxia is rarely attributed to lesions of the peripheral vestibular system. In 1973, the first case of ataxia and hearing loss secondary to a labyrinthine fistula was reported. Until now, this syndrome has not been reported in patients under the age of 10 years. A case is presented of a 5-year-old boy with symptoms of ataxia and hearing loss as well as vertigo and tinnitus after head trauma. Three physical findings appear to be most characteristic of patients with perilymphatic fistulas: a positive fistula response, positive positional testing with the involved ear down, and evidence of vestibular ataxia when testing station and gait. The absolute diagnosis of perilymphatic fistula can only be established by exploration of the middle ear space. If a fistula is found, it may be sealed with soft tissue and, if this fails, actual stapedectomy may be required.
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7/7. Co-existing post-stapedectomy fistula and acoustic neuroma.

    A 66 year old woman had light-headedness and ataxia which increased slowly despite tissue seal of a post-stapedectomy oval window fistula. The cause was shown to be a large acoustic neuroma, ipsilateral to the side of the fistula. The history, physical, and laboratory findings are presented.
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