Cases reported "Hearing Disorders"

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1/62. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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2/62. facial nerve schwannoma in the cerebellopontine cistern. Findings on high resolution CT and MR cisternography.

    We report a case of facial nerve schwannoma originating in the cerebellopontine cistern, diagnosed by cochlear sensorineural hearing loss, and involvement in the facial nerve canal and the anterior epitympanic recess. Precise analyses of neuro-otological and neuroradiological studies are crucial to obtain an accurate preoperative diagnosis.
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3/62. The role of radiation in delayed hearing loss in nasopharyngeal carcinoma.

    Although radiation damage to the inner ear can be induced experimentally in animals, its incidence in humans as a complication of therapy for nasopharyngeal carcinoma (NPC) remains a matter of conjecture due to inadequate histological validation. A case of advanced NPC with a mixed conductive and neurosensory hearing loss is presented that at necropsy showed tumour invasion of the VIIIth cranial nerve in the internal auditory meatus with associated infection. The architecture of the organ of corti was well-preserved despite heavy doses of radiation but degeneration, the cause of which could not be substantiated, was present in the auditory nerve pathway. A diagnosis of radiation damage to the end-organ of hearing in the absence of supporting histological evidence should be made with caution.
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4/62. Useful residual hearing despite radiological findings suggestive of anacusis.

    A severe malformation of the inner ear, often referred to as severe labyrinthine dysplasia or common cavity deformity, consists of an absent or dilated cochlear basal coil, wide communication with the vestibule and a tapered internal acoustic meatus and can be associated with absent hearing. We discuss two children with severe labyrinthine dysplasia as shown by computed tomography (CT) scans and, in the first case, an absent VIIIth nerve bilaterally shown by magnetic resonance imaging (MRI). In 1995, both cases were precluded from cochlear implantation, on the basis of the absent VIIIth nerve (first case) and increased risk of CSF leak during operation (second case). However, audiometric results and vocalization patterns of both children suggested the presence of some residual hearing function, while recently reported specific surgical techniques have been found to be safe and effective in the cochlear implantation of the common cavity deformity. The management of such cases should be decided on the grounds of a full audiological assessment in conjunction with the radiological features, in the light of current surgical trends shown to be safe and effective.
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5/62. Prediction of the nerves of origin of vestibular schwannomas with vestibular evoked myogenic potentials.

    OBJECTIVE: To determine whether the nerves of origin of vestibular schwannomas can be predicted using vestibular evoked myogenic potentials (VEMPs). STUDY DESIGN: The study was a retrospective analysis. SETTING: The ear, nose, and throat department of tokyo Medical and Dental University. patients: Twenty-eight patients undergoing removal of vestibular schwannomas were included in the study. INTERVENTIONS: patients underwent pure tone audiometry, VEMP testing, caloric testing, and magnetic resonance imaging preoperatively. Hearing level, caloric weakness, maximum tumor size, and the nerves of origin of tumors were compared with VEMP testing. MAIN OUTCOME MEASURE: Results of VEMP testing. RESULTS: Comparisons between VEMPs and results of the other three examinations revealed no correlations. Complete disappearance of VEMPs was observed only in patients with tumors arising from inferior vestibular nerves. patients in whom hearing was preserved tended to have preserved VEMPs. Some patients showed damaged hearing and normal VEMP results, although with inferior vestibular schwannomas. A patient with a tumor arising from a cochlear nerve exhibited preservation of VEMP, preserved caloric response, and moderate hearing loss. CONCLUSIONS: Inferior vestibular nerve function and hearing level were reflected in VEMP results. Prediction of the nerve of origin of a tumor was possible only in certain restricted cases.
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6/62. Extended middle fossa surgery for meningiomas within or at the internal auditory canal.

    OBJECTIVE: To show the clinical outcome in patients with meningioma within or at the internal auditory canal (IAC) operated on by the extended middle cranial fossa approach. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. patients: Twelve patients, 9 women and 3 men, whose ages ranged from 37 to 70 years (mean 57 years). One tumor was entirely intracanalicular, 5 had an intra-extracanalicular growth, 3 were centered at the posterior porus lip, and 3 tumors were localized in the midpetrosal region with spread into the IAC. INTERVENTION: Extended middle cranial fossa approach. MAIN OUTCOME MEASURES: magnetic resonance imaging and computed tomography were used for follow-up and reevaluation of the operative sites. facial nerve function and hearing were examined. RESULTS: Complete resection was achieved in 10 (83%) of 12 patients. The retrosigmoid approach was necessary to complete tumor resection in one patient, and in another, partial resection was done because of the en plaque type of tumor growth. There was one unexpected recurrence. All patients retained normal or near-normal facial nerve function postoperatively. Three patients were deaf before surgery, and preoperative hearing level was preserved in 42%. CONCLUSIONS: Attempted hearing preservation surgery is justified in patients with small tumors and preserved hearing, because tumor exposure and safety of resection are comparable with that of hearing-destructive procedures. Lateral extension of the tumor to the fundus does not prevent complete resection with preservation of function, and elective bone resections beyond the visible tumor margins seem not to substantially influence the tumor recurrence rates.
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7/62. Giant congenital intracranial epidermoid tumor: a case report.

    A case of a large epidermoid tumor in a middle-aged woman with limited symptomatology is reported. Intracranial epidermoid tumors are slow growing and benign lesions. Although the treatment of choice consists in complete resection, partial removal may be preferred in some very extended cases presenting with minimal symptomatology because of the lower morbidity involved. This case illustrates a key-hole approach via a retrolabyrinthine route with preservation of all labyrinthine structures, including the endolymphatic sac and duct. The epidermoid was partially removed by extensive intracapsular debulking under endoscopic control with the aim of decreasing cerebral compression. All cranial nerve functions were preserved and the 6 month postoperative imaging has remained unchanged over a 2-year long follow-up period. The literature regarding this rare pathology is also reviewed.
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keywords = nerve
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8/62. choristoma of the middle ear: a component of a new syndrome?

    CASE REPORT: Salivary choristoma of the middle ear is a rare entity. The authors report the 26th known case, which is unique in several respects: the patient had abnormalities of the first and second branchial arches, as well as the otic capsule and facial nerve in ways not yet reported. Our patient presented with bilateral preauricular pits, conchal bands, an ipsilateral facial palsy, and bilateral Mondini-type deformities. A review of the literature revealed salivary choristomas of the middle ear to be frequently associated with branchial arch abnormalities, most commonly the second, as well as abnormalities of the facial nerve. review OF THE literature: All 25 cases were reviewed and the results reported with respect to clinical presentation, associated abnormalities, operative findings, and hearing results. It has been proposed that choristoma of the middle ear may represent a component of a syndrome along with unilateral hearing loss, abnormalities of the incus and/or stapes, and anomalies of the facial nerve. CONCLUSION: Eighty-six percent of the reported patients with choristoma have three or four of the four criteria listed to designate middle ear salivary choristoma as part of a syndrome. In the remaining four patients, all of the structures were not assessed.
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ranking = 3
keywords = nerve
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9/62. Neurinomas of the facial nerve. Report of three cases.

    Three cases of neurinomas of the facial nerve are reported. Two of them originated from the labyrinthine portion of the nerve and the other from the vertical portion. Neurinomas of the first part of the facial nerve can be suspected preoperatively since they seem to give rise to specific clinical and radiological manifestations that can be distinguished from tumors of other portions of the nerve, the petrous bone area, or the cerebellopontine angle. The reported cases of neurinomas of the facial nerve are reviewed and analyzed.
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ranking = 9
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10/62. facial nerve neuromas: report of 10 cases and review of the literature.

    OBJECTIVE: This study reviewed the management and outcomes of facial neuromas during the past decade at our institution. The goal was to analyze differences in presentation on the basis of location of the facial neuroma, review facial nerve function and hearing preservation postoperatively, and understand the characteristics of patients with tumors limited to the cerebellopontine angle or internal auditory canal. We also report an unusual case of a facial neuroma limited to the nervus intermedius. methods: Nine patients with facial neuromas and one with Jacobson's nerve neuroma underwent surgery, and total resection was accomplished in nine patients. A chart review for pre- and postoperative data was performed, after which all patients were evaluated on an outpatient basis. RESULTS: The mean age of the patients was 47 years; mean follow-up time was 33.1 months. The most common presenting symptoms were hearing loss (six patients) and facial paresis (five patients). A total of five patients had progressive (four patients) or recurrent (one patient) facial paresis. No patient experienced worsened hearing as a result of surgery, and one experienced improvement in a conductive hearing deficit. Five patients required cable graft repair of the facial nerve; four improved to House-Brackmann Grade 3 facial paresis. Four of five patients with preserved anatomic continuity of the facial nerve regained normal facial function. There were no surgical complications. No tumors have recurred during follow-up. We report the second nerve sheath tumor limited to the nervus intermedius. CONCLUSION: This series documents that facial neuromas can be resected safely with preservation of facial nerve and hearing function. Preservation of anatomic continuity of the facial nerve should be attempted, and it does not seem to lead to frequent recurrence. Tumors limited to the cerebellopontine angle/internal auditory canal are a unique subset of facial neuromas with characteristics that vary greatly from facial neuromas in other locations, and they are indistinguishable clinically from acoustic neuromas.
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ranking = 11
keywords = nerve
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