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1/117. Successful cochlear implantation in a patient with melas syndrome.

    OBJECTIVE: To describe methods of assessing cochlear implant candidacy in patients with potentially significant peripheral and central nervous system (CNS) degeneration. STUDY DESIGN: A patient with a degenerative CNS disease (melas syndrome) undergoing evaluation for cochlear implantation is described. SETTING: This study took place at a tertiary care center. PATIENT: A patient with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had cortical blindness and profound sensorineural hearing loss was evaluated and rehabilitated with cochlear implantation. INTERVENTIONS: Pure-tone audiogram, behavioral responses to promontory stimulation electrical auditory brainstem response, and electrically evoked middle-latency responses (MLRs) were used to assess eighth nerve, auditory brainstem, and cortical auditory pathways. cochlear implantation with Cochlear Corporation mini 22 implant was performed. RESULTS: Repeatable electrically evoked MLRs and behavioral responses to promontory stimulation documented the presence of auditory cortical responses. Successful implantation resulted in open set speech recognition and communication using the auditory/oral mode. CONCLUSION: This report describes successful implantation in a patient with melas syndrome and demonstrates the ability to preoperatively confirm the integrity of brainstem and cortical auditory pathways despite significant CNS degeneration.
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keywords = operative
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2/117. Case report and discussion of hearing preservation after translabyrinthine excision of small acoustic tumors.

    OBJECTIVE: Since 1991, three separate reports have shown how hearing may be salvaged after translabyrinthine excision of small acoustic tumors. The authors submit yet another report of a complete translabyrinthine excision of a 1.4-cm intracanalicular acoustic tumor with modest hearing preservation. An attempt is made to retrace the steps of the operation and recognize and discuss what particular events may have safeguarded the viability of the cochlea. With the availability of cochlear implantation, there should be added incentive to preserve the cochlear neurones if hair cells cannot be saved. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was conducted at a primary care hospital. INTERVENTION: Therapeutic and rehabilitative measures were performed. MAIN OUTCOME MEASURES: Hearing preservation was measured. CASE REPORT: A 55-year-old woman presented with a left-sided hearing loss and a 1.4-cm left acoustic tumor completely filling the internal auditory canal (speech reception threshold [SRT] 30 dB, discrimination [Pb] 28%). A successful translabyrinthine excision of the tumor was performed in November 1995. A 1-year postoperative audiogram showed a mixed hearing loss in the left ear with SRT 85 dB and Pb 0%. Average pure-tone threshold for 500 Hz, 1 kHz, and 3 kHz was 50 dB and aided SRT 40 dB with Pb 64%. Postoperative magnetic resonance imaging confirmed complete excision of the tumor. CONCLUSION: An exceptional case of hearing preservation after translabyrinthine excision of a small acoustic tumor illustrates how it may be possible to preserve cochlear hair cells and neurones simultaneously in certain selected cases. A review of the surgical events shows the value of sealing the cochlear duct with bone wax, selectively removing the vestibular nerves with the tumor by sharp dissection, and safeguarding the meatal segment of the anterior inferior cerebellar artery by a limited dural incision.
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ranking = 2
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3/117. cochlear nerve aplasia: its importance in cochlear implantation.

    OBJECTIVE: The objective of this study was to outline the possible implications and potentially valuable techniques for managing cases in which the neural integrity of the peripheral auditory system is in question. STUDY DESIGN: This study was a retrospective case review. SUBJECT AND METHOD: A 3-year-old child with a profound blilateral sensorineural hearing loss was assessed for suitability of cochlear implantation. Audiologic tests confirmed that the child met the audiologic criteria for cochlear implantation. Computed tomographic scanning and magnetic resonance imaging were undertaken. RESULTS: Computed tomographic scanning showed bilateral narrow internal auditory canals. magnetic resonance imaging showed the absence of the acousticofacial bundle on the left side and possible atrophy of the bundle on the right. After detailed discussion, the parents elected to proceed with implantation on the right ear using the Nucleus mini-22 cochlear implant. Tuning of the device resulted in myogenic facial activity with no electrically stimulated auditory sensation. Postoperative electrophysiologic testing confirmed the presence of a compound muscle action potential only. CONCLUSIONS: Seven months after implantation, the child was explanted uneventfully. The electrical auditory nerve action potential and the electrically evoked auditory brainstem response, using intracochlear stimulation, are potentially valuable measurements to assess neural integrity before the decision to proceed with implantation is made.
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4/117. Salivary gland choristoma in the middle ear: a case report.

    Salivary gland choristoma (ectopic or heterotopic salivary gland tissue) is a rare condition that occurs in various locations within the head and neck regions. We present a 13-year-old boy with a salivary gland choristoma in the middle ear associated with congenital alopecia around the auricle in addition to facial nerve and ossicular chain abnormalities. Because the lesions commonly involve the facial nerve, intraoperative facial nerve monitoring is helpful in allowing safe biopsy without facial nerve damage.
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5/117. Intraoperative mitomycin C in endolymphatic sac surgery for Meniere's disease: A pilot study.

    The long-term results of endolymphatic sac (ES) shunt surgery in Meniere's disease have been reported to be unsatisfactory compared to the short-term results, probably because of fibrosis and/or reclosure of the incised ES. To solve this problem, we tried to apply mitomycin C (MMC) intraoperatively to the incised ES as used in trabeculectomy for glaucoma. MMC has an antiproliferative effect as well as an antineoplastic effect. Thus, it is expected that MMC would have a benefit to prevent rapid fibrosis and/or reclosure of the ES. MMC did not show any ototoxicity in our animal study and we started a clinical trial after being approved by the ethics Committee for Human research at our university. Fourteen patients with Meniere's disease underwent ES mastoid shunt surgery with intraoperative application of MMC and they were followed up more than 6 months. No patients experienced vertigo, although some complained of slight dizziness. Five patients out of 14 showed remarkable hearing improvement by more than 10 dB, accompanied with decrease of tinnitus.
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ranking = 6
keywords = operative
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6/117. Reversible sensorineural hearing impairment induced by a carotid body tumor.

    A case of a 62-year-old Austrian man having a 25-year history of a right-sided carotid body tumor (CBT) is presented. Three months before being transferred to the University of Vienna for tumor resection the patient developed symptoms of tinnitus, progressive ipsilateral hearing loss and dysphagia. Pure-tone audiometry demonstrated a 50 dB right sensorineural hearing loss. A 6 x 6 x 4 cm firm, pulsatile mass was found in the right carotid triangle and extending towards the base of the skull. One week after radical tumor resection all preoperative symptoms disappeared and hearing of the right ear recovered. review of the available literature showed that hearing loss and tinnitus are unusual symptoms of a CBT. Our findings suggest that routine audiometric evaluations in such cases of CBT patients should be obtained in order to determine the real incidence of audiological disorders.
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keywords = operative
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7/117. cochlear implantation for auditory rehabilitation in Camurati-Engelmann disease.

    Camurati-Engelmann disease (progressive hereditary diaphyseal dysplasia) is a rare sclerotic bone disease involving the diaphyses of the long bones, skull base, and clavicles. Progressive sclerosis of cranial nerve foramina has been implicated in cranial nerve deficits. including facial nerve palsy, vestibular disturbances, and hearing loss. Two patients with Camurati-Engelmann disease and concomitant sensorineural hearing loss are presented. Both patients were evaluated for cochlear implantation. One patient was successfully implanted after preoperative imaging revealed no involvement of the internal auditory canals. The porous nature of the affected bone, however. necessitated the inactivation of 1 electrode to prevent facial nerve stimulation. A second patient was rejected as a potential implant recipient due, in part, to narrow internal auditory canals and rapidly progressive disease. The otologic manifestations of Camurati-Engelmann disease are reviewed, and issues related to cochlear implantation in this rare disease are discussed.
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ranking = 1
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8/117. Conservative facial nerve management in jugular foramen schwannomas.

    OBJECTIVE: Although transposition of the facial nerve is crucial in infiltrative vascular lesions involving the jugular foramen, the objective was to show that a conservative approach to management of the facial nerve is sufficient with jugular foramen neuromas because of their noninfiltrative, less vascular nature and medial location in the jugular foramen. STUDY DESIGN: Retrospective case review. SETTING: Tertiary, private, multiphysician, otologic practice. patients: Sixteen patients with jugular foramen schwannoma (18 procedures) treated between January 1975 and October 1995. The 8 male and 8 female patients ranged in age from 13 to 66 years (mean age 47.7 years). INTERVENTION: One-stage, total jugular foramen neuroma removal without transposition of the facial nerve, using a variety of surgical approaches. MAIN OUTCOME MEASURES: facial nerve transposition (yes or no), House-Brackmann facial nerve grade, lower cranial nerve status, complications. RESULTS: One-stage total tumor removal was accomplished in all the cases. In 13 (72%) of the neuromas, removal was accomplished without facial nerve transposition. Transposition was performed in 2 revision cases in which scar tissue from a previous operation prevented complete control of the carotid artery and safe removal, 2 cases with large tumor extension anteriorly to the petrous apex, and 1 case with extensive involvement of the middle ear. A House-Brackmann facial nerve Grade I or II was obtained in 16 of the 18 procedures, with 1 Grade III and 1 case that remained Grade V, as it was preoperatively. CONCLUSIONS: One-stage, total tumor removal can be achieved with excellent control of the important vascular structures and without transposition of the facial nerve in a majority of jugular foramen schwannomas.
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ranking = 1
keywords = operative
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9/117. Reversible sensorineural hearing loss after non-otological surgery under general anaesthetic.

    Acute sensorineural hearing loss can occur after both otolaryngological and non-otolaryngological procedures. The mechanism of such hearing loss remains unproved; but nitrous oxide has been implicated and where used, attendants should be aware of its potential damage to hearing. It is essential that patients with sudden hearing loss are identified as soon as possible as the recovery rate had been shown to be higher in those who presented early; and as our case demonstrates, complete recovery is possible. Anaesthetists, non-otolaryngological surgeons, and ward nurses must be aware of this early postoperative complication if appropriate treatment is to be instituted.
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ranking = 1
keywords = operative
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10/117. hearing loss after cardiopulmonary bypass surgery.

    hearing loss (HL) is a rare complication following cardiac surgery with extracorporal circulation (CSWEC) or other non-otologic surgery under general anesthesia, as is HL caused by loss of cerebrospinal fluid during neurosurgery or spinal anesthesia. The incidence of HL after CSWEC is not known since preoperative hearing testing is not commonly done and a perioperative HL may occur unnoticed. We present four cases of profound sensorineural hearing loss following CSWEC for coronary artery bypass or cardiac valve surgery. The hearing loss was noticed immediately on waking from anesthesia in three of the patients, whereas the fourth patient noticed decreased hearing during the first postoperative week. In all patients audiological investigation suggested a cochlear etiology of the HL. The possible mechanisms for HL are discussed. HL after CSWEC is most likely caused by a microembolism generated by atheromatous material.
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ranking = 3
keywords = operative
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