Cases reported "Hearing Loss, Bilateral"

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1/7. Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report.

    Transient hearing decrease following loss of cerebrospinal fluid (CSF) has been reported in patients undergoing lumbar puncture, spinal anesthesia, myelography, and/or different neurosurgical interventions. The authors present the first well-documented case of a patient with persistent bilateral low-frequency sensorineural hearing loss after shunt placement for hydrocephalus and discuss the possible pathophysiological mechanisms including the role of the cochlear aqueduct. These findings challenge the opinion that hearing decreases after loss of CSF are always transient. The authors provide a suggestion for treatment.
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2/7. 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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keywords = aqueduct
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3/7. hearing loss as a sequel of lumbar puncture.

    Only a few case reports have been published about hearing impairment following lumbar puncture, and not all were thoroughly documented by audiograms. We present nine cases of hearing loss following myelography, lumbar puncture, and spinal anesthesia. We speculate that this rare complication arises only in persons with a wholly or partially patent cochlear aqueduct, and occurs via the release of perilymphatic fluid in the cerebrospinal space. hearing loss was seen in eight of the nine patients in the lower frequencies, and in six of the nine patients on both sides. Recovery to normal hearing was noticed in six of the nine patients. Transient hearing loss may occur more often than it is generally assumed, and the symptom can remain unnoticed. Since not all of these hearing losses proved to be fully reversible, we suggest informing patients about this complication for medicolegal reasons.
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keywords = aqueduct
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4/7. Considerations for cochlear implantation of children with sudden, fluctuating hearing loss.

    The histories of two pediatric patients who received cochlear implants with subsequent partial recovery of hearing in the nonimplanted ear are reviewed. One child had a sudden bilateral hearing loss, presumably secondary to autoimmune ear disease. The other child had a bilateral progressive hearing loss diagnosed as large vestibular aqueduct syndrome (LVAS). The rationale for the timing of the surgical implantation is discussed. Retrospectively, recovery of hearing in the nonimplanted ear suggests the possibility that the implant could have been delayed or eliminated as a treatment option, and that wearable hearing aids may have been appropriate. A number of factors, however, suggest the decision to implant was appropriate. Issues involved in the decision-making process of when to implant are presented and discussed.
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keywords = aqueduct
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5/7. Large vestibular aqueduct syndrome: a case study.

    A 23-month-old female was referred for hearing aid fitting after failing newborn hearing screening and being diagnosed with significant hearing loss through subsequent diagnostic testing. Auditory brainstem response (ABR) and behavioral testing revealed a moderate-to-severe bilateral mixed hearing loss. Prior to the hearing aid evaluation, tympanostomy tubes had been placed bilaterally with little or no apparent change in hearing sensitivity. Initial testing during the hearing aid fitting confirmed earlier findings, but abnormal middle ear results were observed, requiring referral for additional otologic management. Following medical clearance, binaural digital programmable hearing aids were fit using Desired sensation Level parameters. Behavioral testing and probe microphone measures showed significant improvements in audibility. Decrease in hearing sensitivity was observed six months following hearing aid fitting. Radiological studies, ordered due to the mixed component and decreased hearing sensitivity, revealed large vestibular aqueduct syndrome (LVAS). Based on the diagnosis of LVAS, a cochlear implant was placed on the right ear; almost immediate speech-language gains were observed.
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ranking = 5
keywords = aqueduct
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6/7. The supranuclear disturbances of gaze in kernicterus.

    Five patients with kernicterus and ocular motility disturbances are reported. All of the patients showed some defect in vertical gaze but not always upgaze. The most severely affected patient exhibited slowness of horizontal saccades in addition to a total vertical gaze palsy. In general, the severity of the ocular motility disturbance paralleled the severity of hearing loss. It is suggested that damage to the periaqueductal area accounts for disturbances in vertical gaze and the infrequent horizontal saccadic disturbances results from interruption of the descending centrifugal fibers. It is emphasized that kernicterus must be included in the differential diagnosis of any supranuclear vertical gaze disturbance.
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keywords = aqueduct
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7/7. Large vestibular aqueduct syndrome and its implication for cochlear implant surgery.

    The large vestibular aqueduct syndrome (LVAS) is commonly found when presurgical computed tomography is performed in advance of cochlear implantation. We present data on two patients with LVAS, in whom computed tomography (CT) and magnetic resonance imaging (MRI) was performed. cochlear implantation when these malformations are present may be complicated by an intraoperative gusher, but it appears to be safe and produces a favorable outcome.
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ranking = 5
keywords = aqueduct
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