Cases reported "Otosclerosis"

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1/15. Delayed facial paralysis after stapedotomy using KTP laser.

    OBJECTIVE: Delayed facial paralysis after stapes surgery is uncommon and has been reported after traditional, nonlaser techniques for stapedotomy. The purpose of this paper is to inform the reader of the potential risk of delayed facial nerve paralysis associated with the use of the potassium titanyl phosphate (KTP) laser for stapedotomy. Etiologic mechanisms are discussed. STUDY DESIGN: The study was a descriptive study-case report. SETTING: The study was conducted at a university-based otologic practice. patients: Two patients with otosclerosis and delayed onset facial palsy 5 to 7 days after uncomplicated stapedotomy using the KTP laser were included in the study. INTERVENTION: potassium titanyl phosphate laser stapedotomy was performed. patients received treatment of facial palsy with a tapering course of oral steroids. MAIN OUTCOME MEASURE: House-Brackmann facial nerve grade scores were used. RESULTS: Improvement of House-Brackmann facial nerve scores from Grade VI to Grade I-II in one patient, and improvement from Grade IV to Grade I-II in the other was seen. CONCLUSION: The probable etiology of delayed facial palsy is viral neuritis from reactivation of dormant virus within the facial nerve, initiated by thermal stress of the KTP laser. Presentation and resolution of the facial palsy is similar to other types of delayed facial palsy resulting from nonlaser techniques of stapes surgery and other types of middle ear and neurotologic surgeries previously reported.
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2/15. Ultrastructural investigations on the degenerative and regenerative processes in chorda tympani in bell's paralysis.

    Our ultrastructural findings in investigated cases of Bell's palsy have demonstrated that the degenerative changes did not proceed at the same speed from the onset of the paralysis--in other words the regeneration changed in accordance with the topography of the nerve. Although the endoneural sheath preserved its integrity, swellings in the schwann cells have attracted much attention. Furthermore the perineural tissue presented changes which were closely correlated with the degenerative and regenerative processes due to paralysis. In addition to these findings, although the interval between the onset of paralysis and the operation was the same in all our cases, the observation of a more severe degenerative picture of the nerves in the female patients may seem to be an interesting finding, but to the lack of an adequate number of cases which would enable a statistical evaluation, we are strongly, of the opinion that this subject deserves further study.
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3/15. facial nerve schwannoma in revision stapedotomy surgery.

    We describe a male patient who presented a progressive conductive unilateral hearing loss 20 years after otosclerosis surgery. Computed tomography (CT) scan and magnetic resonance imaging (MRI) findings suggested a facial schwannoma in its tympanic segment. At the time of revision surgery, a facial schwannoma was found to originate at the tympanic segment, pushing the prosthesis out of the oval window fenestration. The Teflon-piston was repositioned with difficulties in the central platinotomy, and the facial schwannoma was left intact.
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4/15. cochlear implantation in 53 patients with otosclerosis: demographics, computed tomographic scanning, surgery, and complications.

    OBJECTIVES: To collect data from a large number of cochlear implant recipients with otosclerosis and to make an assessment of these patients' clinical characteristics, computed tomographic scans, surgical findings, and complications, and to quantify the occurrence of postoperative facial nerve stimulation. STUDY DESIGN: Retrospective multicenter study. patients: Fifty-three patients with otosclerosis from four cochlear implant centers in the United Kingdom and The netherlands were reviewed. Sixty surgical procedures were performed in these patients: 57 devices were placed in 56 ears. RESULTS: The computed tomographic imaging demonstrated retrofenestral (cochlear) otosclerotic lesions in the majority of patients. Although not statistically significant, the extent of otosclerotic lesions on the computed tomographic scan as categorized in three types tends to be greater in patients with rapidly progressive hearing loss, in patients in whom there is surgically problematic insertion of the electrode array, and in patients with facial nerve stimulation. In four patients, revision surgery had to be performed. Twenty of 53 (38%) patients experienced facial nerve stimulation at various periods postoperatively. CONCLUSION: Cochlear implant surgery in patients with otosclerosis can be challenging, with a relatively high number of partial insertions and misplacements of the electrode array demanding revision surgery. A very high proportion of patients experienced facial nerve stimulation mainly caused by the distal electrodes. This must be discussed with patients preoperatively.
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5/15. temporal bone histopathology of a patient with a nucleus 22-channel cochlear implant.

    The temporal bone histopathology of a patient with profound sensorineural hearing loss attributable to cochlear otosclerosis is presented. The patient had a Nucleus 22-channel cochlear prosthesis implanted in the right ear prior to his demise. Unintentional scala media insertion of the electrode array resulted from labyrinthitis ossificans obliteration of the scala tympani. Inadvertent facial nerve stimulation necessitated program exclusion of electrodes 11 to 16 located in proximity to the labyrinthine and geniculate segments of the facial nerve. The patient received significant benefit from use of the device. The histopathologic findings are correlated with psychophysical and speech perception results.
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6/15. Revision cochlear implantation for facial nerve stimulation in otosclerosis.

    OBJECTIVE: To find if patients experiencing postsurgical facial nerve stimulation caused by underlying disease process (ie, otosclerosis) can improve their hearing performance with their cochlear implant by reimplantation and by an optimal programming strategy. DESIGN: Retrospective analysis. SETTING: Academic tertiary referral center. patients: Two cochlear otosclerosis patients with resistant facial nerve stimulation (FNS). Both patients were initially implanted with Nucleus 22 devices (Cochlear Corporation, Englewood, Colo) and they developed FNS after a period of use. Owing to the decreasing number of active electrodes, concurrent decreases in speech understanding occurred. INTERVENTIONS: Various programming approaches were used to address the FNS. Both subjects ultimately received Nucleus 24 devices. One was reimplanted in the same ear, and the other was implanted in the opposite ear. Both have been followed up for 8 months following the reimplantation. MAIN OUTCOME MEASURES: Cochlear implant programming levels, cochlear implant performance, and facial nerve stimulation. RESULTS: The FNS was managed for more than 3 years through optimized programming. However, the FNS progressed until performance dropped below acceptable levels. Reimplantation was believed to be the only option for improvement. After reimplantation and programming, both subjects showed immediate improvement in speech discrimination. One user increased his consonant-nucleus-consonant word score from 12% preoperatively to 42%, and the other's performance increased from 0% to 86%. CONCLUSIONS: Our results suggest that having more programming options with newer devices is critical in otosclerotic or ossified users who experience FNS. Also, reimplantation may be a useful tool to improve performance.
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7/15. Trembling chin--a report of this inheritable dominant character in a four-generation Canadian family.

    Trembling chin is an unusual, benign, dominant trait. We describe the condition in a four-generation Canadian family. Investigations, which included E.E.G., electromyograph and facial nerve conductance studies on affected and unaffected individuals, were normal. Premature hearing loss, present in all affected members of this family over age 50, strongly suggests an association of trembling chin with otosclerosis. The merits of early diagnosis and preventive treatment for otosclerosis in persons affected by trembling chin are discussed.
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8/15. osteogenesis imperfecta tarda: appearance of the temporal bones at CT.

    The computed tomographic findings in the temporal bone of two patients with osteogenesis imperfecta tarda are described. One of the patients had bilateral facial nerve paresis, and both patients presented with hearing loss.
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9/15. temporal bone showing polyarteritis nodosa, otosclerosis, and occult neuroma.

    The clinical and general histopathologic manifestations of systemic vasculitis of the polyarteritis nodosa type are well known. Although hearing loss associated with polyarteritis nodosa has been reported, only two temporal bone studies are available. The findings in a 60-year-old man with well-documented hearing loss who had rheumatoid arthritis, polyarteritis nodosa, and otosclerosis are presented. polyarteritis nodosa extensively involved the subarcuate arteries and arteries of the facial canal. There were decreased nerve fibers to and sensory cells in the crista of the semicircular canals and macula of the utricle and saccule. Focal and diffuse atrophy of the stria vascularis and decreased cellularity in the spiral prominence and ligament were present. There was a loss of outer hair cells. otosclerosis involved the left and right oval window niches (bilateral stapedectomy had been performed). There was a small Antoni type A neuroma of the superior division of the vestibular nerve on the left.
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10/15. otosclerosis and sensorineural hearing loss: a histopathologic study.

    The precise role and mechanism whereby otosclerosis is associated with sensorineural hearing loss remains unclear. Previous histopathologic reports are inconsistent with regard to the location of the otosclerotic focus, invasion of the otic capsule, and the number of remaining peripheral sensorineural elements. From the combined temporal bone collections of the University of chicago and the Mayo Clinic, we identified a group of 125 ears from 80 patients, all with confirmed otosclerosis. Six of these ears were associated clinically with sensorineural hearing loss without stapes fixation. The histopathology of the otosclerotic focus was reviewed in terms of its location and depth of invasion. The cochlea and spiral ganglion were reconstructed, and the state of the organ of corti and the presence or absence of peripheral cochlear nerve fibers were noted. Correlations with ganglion cell counts were made. The present study showed that the pattern of degeneration of peripheral sensory and neural elements in the cases presented is very similar to that found in cases of age-related processes such as presbycusis.
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