Cases reported "Vestibular Diseases"

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1/18. 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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2/18. Isolated large vestibular aqueduct syndrome in a family.

    This paper presents the second case in the literature of large vestibular aqueduct syndrome without associated cochlear anomalies in 2 members of the same family. The syndrome is frequently associated with sensorineural hearing loss presenting in childhood. The onset is commonly sudden, following an event causing increased intracranial pressure. On the basis of an emerging pattern of inheritance, we recommend screening siblings of an affected child. We also discuss the importance of characterizing the extent of disease of the inner ear.
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3/18. Vascular occlusion in the endolymphatic sac in Meniere's disease.

    In 2 patients with severe Meniere's disease (MD), there was histologic evidence of occlusion of the vein of the vestibular aqueduct (VVA). This finding coincided with total or partial occlusion of numerous small vessels around the endolymphatic sac (ES), flattening of epithelium, extensive perisaccular fibrosis, and signs of new bone formation. Ultrastructural analysis of the occluding material showed foci with dense connective tissue, calcification, lipid deposits, and layers of basement membrane, sometimes concentrically arranged. The exact nature of the occluding material was unknown. In another 2 MD patients, the VVA was not visualized, and the ES vessels showed no signs of occlusion. Seven controls with acoustic schwannoma or meningioma had normal vasculature. The presence of vascular impairment in the ES in MD patients indicated that altered hemodynamics may contribute to the pathogenesis of endolymphatic hydrops and MD.
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4/18. Familial isolated unilateral large vestibular aqueduct syndrome.

    Large vestibular aqueduct syndrome (LVAS) is one of the most frequent inner ear malformations. It is associated with varying degrees of hearing loss, that may be progressive, sudden or fluctuant; nevertheless normal hearing in cases with LVAS has been described. Although unilateral LVAS has been described, all patients show clinical and radiological findings in both ears, in contrast to the family presented here, where 2 siblings are affected by unilateral sensorineural hearing loss associated with ipsilateral LVAS, with an autosomal recessive pattern. In order to search for the origin of this inner ear malformation, mutations in genes GJB2 (connexin-26 gene), PDS (Pendred's syndrome gene) and mitochondrial 12sRNA were studied, without positive results. In this paper we hypothesize the probable embryological origin for this malformation.
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5/18. Isolated vestibular ossification after meningitis associated with sensorineural hearing loss.

    OBJECTIVE: Sensorineural hearing loss after bacterial meningitis is common. It is thought to be secondary to cochlear inflammation from direct bacterial seeding through the cochlear aqueduct. Although cochlear ossification with or without vestibular involvement is common, isolated vestibular ossification is rare. We review our recent experience with isolated vestibular ossification associated with profound sensorineural hearing loss and the implication for cochlear implantation in this population. STUDY DESIGN: Retrospective case review. SETTING: Academic tertiary referral center. patients: patients referred with profound sensorineural hearing loss after bacterial meningitis with evidence of ossified vestibular labyrinth but normal cochleae on computed tomographic scanning were included in the study.INTERVENTIONS We review the history, imaging findings, operative intervention, and operative findings in this series of patients.MAIN OUTCOME MEASURES Clinical history, imaging findings, operative intervention, and operative findings. RESULTS: Three patients with isolated vestibular ossification without computed tomographic evidence of cochlear osteoneogenesis were identified. Two of these patients subsequently underwent cochlear implantation with the Clarion device with complete insertion of the electrode array. Intraoperatively, one patient had isolated scala tympani ossification and the implant electrode array was placed within the scala vestibuli. CONCLUSION: A pattern of sensorineural hearing loss and isolated ossification of the vestibular system without radiographic evidence of cochlear involvement may be seen after bacterial meningitis. In these patients, magnetic resonance imaging scanning should be performed, because computed tomographic scanning may not always detect cochlear ossification. cochlear implantation is feasible in these patients.
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6/18. magnetic resonance imaging of the large vestibular aqueduct.

    The large vestibular aqueduct syndrome describes an abnormally large endolymphatic duct and sac with associated sensorineural hearing loss. This entity was originally reported in 1978 and has since been identified as a finding in children with progressive hearing loss. The original description of the large vestibular aqueduct employed hypocycloidal polytomography of temporal bone. Subsequent reports studied patients identified with this syndrome using computed tomographic scans. We report magnetic resonance imaging of two patients diagnosed with the large vestibular aqueduct syndrome. The magnetic resonance imaging and computed tomographic scans are compared and the significant findings on magnetic resonance imaging are reviewed. This should assist the otolaryngologist and radiologist with establishing the appropriate diagnosis.
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7/18. Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct.

    An enlarged vestibular aqueduct (LVA) is a common congenital inner ear anomaly responsible for some unusual vestibular and audiological symptoms. Most of the cases show bilateral early onset and progressive hearing loss in children. The gross appearance on CT scan of the inner ear is generally normal. However, precise measurements of the inner ear components reveal abnormal dimensions, which may account for the accompanying auditory and vestibular dysfunction. Despite extensive studies on hearing and the vestibular apparatus, saccular function is not studied. To our knowledge this is the first report of saccular malfunction in three patients with LVA by means of vestibular evoked myogenic potentials. Conventional audiograms revealed bilateral severe sensorineural hearing loss in two patients and mixed type hearing loss in one patient. Two of the patients complained about vertigo and dizziness but vestibular assessments of the patients showed normal results. The diagnosis had been made by high-resolution CT scans and MR images of the skull that showed LVA in the absence of other anomalies. The VEMP threshold measured from the ear with LVA in two patients with unilateral enlargement of the vestibular aqueduct was 75-80 dB nHL whereas the threshold from normal ears was 95 dB nHL. The third patient with mixed type hearing loss and bilateral LVA had VEMP responses despite a big air-bone gap in the low frequency range. The VEMP in this patient was greater in amplitude and lower in threshold in the operated ear (the patient had a tympanoplasty which did not improve her hearing). These findings and results of other patients with Tullio phenomenon and superior semicircular canal dehiscence, who also showed lower VEMP threshold, confirmed the theory of a 'third window' that allows volume and pressure displacements, and thus larger deflection of the vestibular sensors, which would cause the vestibular organ to be more responsive to sound and pressure changes.
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8/18. Sudden hearing loss due to large vestibular aqueduct syndrome in a child: should exploratory tympanotomy be performed?

    A 16-year-old girl applied to our ENT clinic with a 3-day history of right hearing loss, tinnitus, and pressure in the right ear. She had had surgery for right perilymph fistula two times, one at the age of 7 and the second at the age of 9. She had recovered after both of these surgeries. This time she had exploratory tympanotomy and perylymh fistula was detected. Computerized tomography investigation obtained after 5 days postoperatively showed bilateral large vestibular aqueducts and otherwise normal inner ear structures. thyroid function tests and neck palpation were normal. It was an unusual case with both large vestibular aqueduct syndrome (LVAS) and simultaneous spontaneous perilymph fistula.
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9/18. Presentation of large vestibular aqueduct syndrome to a dizziness unit.

    An abnormally enlarged vestibular aqueduct has been associated with sensorineural hearing loss in children. Vestibular complaints, in this patient population, have not been characterized. Several patients have presented to the dizziness unit at Sunnybrook with vestibular related complaints. These patients all had sensorineural hearing loss noted in childhood. All provided recollections of periods of imbalance and vertigo. High resolution CT scan documented the presence of bilateral enlarged vestibular aqueducts. The Large vestibular aqueduct syndrome (LVAS) will be discussed with reference to the pathophysiology of the vestibular complaints.
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10/18. Obstructing lesions of the endolymphatic sac and duct mimicking Meniere's disease.

    In this retrospective case series and literature review, we demonstrate that temporal bone lesions that obstruct the endolymphatic sac or duct can cause symptoms of Meniere's disease. This finding is likely attributable to endolymphatic hydrops; initially, such cases typically masquerade as Meniere's disease. Between July 1995 and April 2002, a total of 379 patients were treated for an initial diagnosis of Meniere's disease at our institution. Among this group, 3 patients were found to have an obstructing lesion of the endolymphatic sac or duct that we felt was causally related to their Meniere's-like symptomatology. We reviewed these cases and noted the similarities in each patient's presentation, including a common pathophysiology. On imaging studies, each patient had a different pathologic lesion that involved the endolymphatic sac or duct: patient 1 had a jugular megabulb, and she was ultimately treated with vestibular nerve section; patient 2 had a cholesterol granuloma, which was treated with surgical excision; patient 3 had an endolymphatic sac tumor that was treated with surgical excision. As has been suggested in previous reports, not all cases of Meniere's disease are idiopathic. We conclude that obstruction of the endolymphatic sac or vestibular aqueduct by a mass lesion or vascular anomaly can lead to vestibulocochlear pathology that mimics Meniere's disease.
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