1/75. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/75. suggestion audiometry for non-organic hearing loss (pseudohypoacusis) in children.Pertaining to non-organic hearing loss in children, three goals should be attained: detection of this disease, determination of true hearing levels and information about the possible cause. Recently, objective tests have been used principally for children with non-organic hearing loss; however, these lack the simplicity and convenience of traditional audiometry. A new method, which is referred to as suggestion audiometry, since it is suggested to the patient that hearing will be improved as a result of the test procedure, was developed for the purpose of simultaneously achieving the above-stated three goals. The subjects were 20 patients aged 8-16 years suspected of demonstrating non-organic hearing loss and whose apparent hearing loss had been identified by school hearing examinations. suggestion pure tone audiometry was useful for the detection of non-organic hearing loss and suggestion speech audiometry was valuable for the determination of true hearing levels. The subjects were classified into four groups according to the test results. We discuss causes of the disease based on the classification of the subjects obtained from this test procedure.- - - - - - - - - - ranking = 0.049502741707688keywords = organ (Clic here for more details about this article) |
3/75. 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.- - - - - - - - - - ranking = 6keywords = nerve (Clic here for more details about this article) |
4/75. 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.- - - - - - - - - - ranking = 2.0123756854269keywords = nerve, organ (Clic here for more details about this article) |
5/75. 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.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
6/75. Isolation of the spirochetes in the perilymph despite prior antisyphilitic therapy: a case report.For the congenital syphilitic patient to develop sensorineural deafness is uncommon. For the congenital syphilitic patient to have persistence of treponemes after treatment is rare. To demonstrate viable organisms in the inner ear perilymph of the congenital syphilitic patient, despite treatment with penicillin, has not been recorded to date. This is a case report of such a patient.- - - - - - - - - - ranking = 0.006187842713461keywords = organ (Clic here for more details about this article) |
7/75. 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.- - - - - - - - - - ranking = 10keywords = nerve (Clic here for more details about this article) |
8/75. 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.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
9/75. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/75. 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.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
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