Cases reported "Labyrinth Diseases"

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1/104. The leaking labyrinthine lesion resulting from direct force through the auditory canal: report of five cases.

    The leaking labyrinthine lesion is treated by conservative methods or surgical procedures. With respect to the stapes, the surgical treatment is controversial. Five cases of middle ear injuries accompanying oval window rupture are reported herein. In each case, direct force through the auditory canal damaged not only the ossicular chain but also the oval window. Initial symptoms were sudden hearing loss with significant conductive disturbance and various degrees of unsteadiness. Spontaneous horizontal nystagmus directed toward the uninvolved ear was observed in each case. Tympanic cavities were promptly explored under general anesthesia and oval window injuries were confirmed. In each case, the damaged stapes was temporally removed from the oval window. Perilymphatic leakage was recognized in each case. Two patients had subluxation of the stapes with a paucity leakage. Three had complete luxation of the stapes with a relatively huge oval window fistula. Disrupted oval windows were repaired with temporalis muscle fascial grafts that were inserted under the middle ear mucosae surrounding the oval windows. The stapes were replaced in the repaired oval windows, and the ossicular chains were reconstructed without artificial grafts. Vestibular dysfunctions disappeared within 7 days, and satisfactory audiologic results were obtained in each case.
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keywords = hearing loss
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2/104. Labyrinthine involvement in Langerhans' cell histiocytosis.

    BACKGROUND: Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature. patients: We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy.
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keywords = hearing loss
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3/104. Haemorrhage in the labyrinth caused by anticoagulant therapy: case report.

    We report a patient who experienced a severe vertiginous episode with bilateral tinnitus and progressive right-sided hearing loss. She had Marfan's disease and was on anticoagulant treatment. The fluid in the labyrinth gave higher signal than cerebrospinal fluid on T1-weighted images, suggesting haemorrhage. The radiological follow-up is discussed.
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ranking = 1
keywords = hearing loss
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4/104. Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management.

    OBJECTIVES: The objective of this study was to identify factors correlated with the CT outcome and to examine the contribution of the CT scan in the aetiological diagnosis and management of unilateral sensorineural hearing loss in childhood. methods: The records of 35 consecutively investigated patients by the audiology Department of Great Ormond Street Hospital between January 1996 and June 1998 were reviewed. The CT results, population sample characteristics, initiation of further investigations after the CT results and management decisions based on the CT results were tabulated and analysed. RESULTS: In a series of 35 consecutively investigated children with unilateral sensorineural hearing loss, 11 CT scans were identified as abnormal. The CT findings were: labyrinthitis ossificans (3), unilaterally dilated vestibular aqueduct (2), bilaterally dilated vestibular aqueduct (2), unilateral deformity of the cochlea ('Mondini') (1), unilateral severe labyrinthine dysplasia (1), unilateral markedly narrow internal acoustic meatus (1), bilaterally dilated lateral semicircular canals (1). The presence of progressive hearing loss was a significant predictor of abnormal CT outcome, while the severity of hearing loss was not. The CT scans offered valuable information regarding the aetiological diagnosis in all cases and, in addition, prompted the appropriate vestibular rehabilitation in three cases, further investigations in four (with dilated vestibular aqueduct) and hearing preservation counselling in two (bilateral DVA) (seven out of 35 = 20%). CONCLUSION: All children with unilateral sensorineural hearing loss should have a CT scan of the petrous pyramids/IAMs performed at some stage, as not only aetiology but also prognosis and management of these cases may be significantly influenced by the CT outcome.
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ranking = 31.384847082473
keywords = sensorineural hearing loss, hearing loss, sensorineural, sensorineural hearing
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5/104. Cavernous angioma of the internal acoustic meatus--case report.

    A 39-year-old female presented with an intrameatal cavernous angioma manifesting as hearing loss and tinnitus in the right ear which progressed over 8 months. Magnetic resonance (MR) images revealed an intrameatal lesion as ultra-high intensity, nearly as bright as cerebrospinal fluid, on the T2-weighted images, and isointensity on the T1-weighted images. Computed tomography (CT) showed the mass accompanied by stippled patterns of calcification. The patient underwent surgery under a diagnosis of calcified acoustic neurinoma. Histological studies were compatible with cavernous angioma. Intrameatal cavernous angioma is a rare disease which requires differential diagnosis from the more common neurinoma or meningioma in this location. Intrameatal lesion with ultra-high intensity on T2-weighted MR imaging and stippled patterns of calcification on CT is more likely to be cavernous angioma than acoustic neurinoma. These neuroimaging features are important information in deciding the treatment strategy.
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ranking = 1
keywords = hearing loss
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6/104. Neuro-otologic manifestations of sarcoidosis.

    sarcoidosis is an idiopathic disease which presents in anatomic areas of concern to otorhinolaryngologists. It can cause dysfunction of both auditory and vestibular systems. In patients known previously to have sarcoidosis, this disease should be seriously considered. In patients presenting with otologic disorders and associated facial nerve paralysis or other neuropathies, uveitis, granulomatous meningitis or diabetes insipidus, sarcoidosis should be suspected. An examination of the eyes as well as a chest X-ray is imperative. Sudden and fluctuating neurosensory hearing loss has been reported. There is a great need for the study of temporal bones from sarcoidosis patients.
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keywords = hearing loss
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7/104. Lesions of the internal auditory canal and cerebellopontine angle in an only hearing ear: is surgery ever advisable?

    OBJECTIVE: To define the indications for surgery in lesions of the internal auditory canal (IAC) and cerebellopontine angle (CPA) in an only hearing ear. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. patients: Seven patients with lesions of the IAC and CPA who were deaf on the side opposite the lesion. Five patients had vestibular schwannoma (VS), and one each had meningioma and progressive osseous stenosis of the IAC, respectively. The opposite ear was deaf from three different causes: VS (neurofibromatosis type 2 [NF2]), sudden sensorineural hearing loss, idiopathic IAC stenosis. INTERVENTION(S): Middle fossa removal of VS in five, retrosigmoid resection of meningioma in one, and middle fossa IAC osseous decompression in one. MAIN OUTCOME MEASURE: Hearing as measured on pure-tone and speech audiometry. RESULTS: Preoperative hearing was class A in four patients, class B in two, and class C in one. Postoperative hearing was class A in three patients, class B in one, class C in two, and class D in one. CONCLUSIONS: Although the vast majority of neurotologic lesions in an only hearing ear are best managed nonoperatively, in highly selected cases surgical intervention is warranted. Surgical intervention should be considered when one or more of the following circumstances is present: (1) predicted natural history of the disease is relatively rapid loss of the remaining hearing, (2) substantial brainstem compression has evolved (e.g., large acoustic neuroma), and/or (3) operative intervention may result in improvement of hearing or carries relatively low risk of hearing loss (e.g., CPA meningioma).
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ranking = 5.1978352974961
keywords = sensorineural hearing loss, hearing loss, sensorineural, sensorineural hearing
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8/104. syndrome of recurrent meninigitis due to congenital perilymph fistula with two different clinical presentations.

    Recurrent meningitis secondary to a congenital labyrinthine anomaly is a rare clinical entity, diagnosis of which is dependent upon certain clinical, radiological and intraoperative features. In the following report we describe two children with congenital labyrinthine fistula and recurrent meningitis whose clinical presentation, radiological features and intraoperative findings were dissimilar and thus, illustrative of two different ways of presentation of this rare disorder. While one had a classical Mondini defect and unilateral hearing loss, the other had normal audiometric and radiographic findings. The fistulae were successfully closed via a tympanotomy approach in both the patients.
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ranking = 1
keywords = hearing loss
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9/104. Clinical features of sudden hearing loss associated with a high signal in the labyrinth on unenhanced T1-weighted magnetic resonance imaging.

    We report on five patients with high signals in the labyrinth on unenhanced magnetic resonance imaging who developed sudden hearing loss and vertigo. Weissman et al. (1992) suggested the possibility that such high signals were caused by hemorrhage. We assessed these patients using audiograms, caloric tests, and auditory brainstem responses to investigate the possibility of inner ear hemorrhage. Most of the patients were found to have severe and irreversible impairment of both cochlear and vestibular function. These findings were consistent with the hypothesis that their symptoms were caused by inner ear hemorrhage.
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ranking = 5
keywords = hearing loss
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10/104. 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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ranking = 12.197835297496
keywords = sensorineural hearing loss, hearing loss, sensorineural, sensorineural hearing
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