Cases reported "Otosclerosis"

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1/20. malleus ankylosis: a clinical, audiometric, histologic, and surgical study of 123 cases.

    OBJECTIVE: malleus ankylosis is a cause of conductive hearing loss that can be difficult to assess, particularly in association with otosclerotic stapes fixation. The aim of this study is to seek the clinical and audiometrical features unique to this pathology. STUDY DESIGN: The study design was a review of malleus ankylosis cases and an analysis of functional results after ossiculoplasty. SETTING: The study was performed at Jean Causse Clinic in france. patients: The authors reviewed a series of 123 ears in 112 patients who underwent surgery for incudo-mallear ankylosis from January 1991 to September 1997. Intervention: The surgical technique depends on the type of pathology encountered. In case of associated stapedial fixation, a stapedotomy with vein graft interposition and reconstruction with a total prosthesis will be performed during a same step. MAIN OUTCOME MEASURES: Clinical evaluation, preoperative and postoperative audiometrical evaluation, operative findings, histologic examination, and postoperative functional results. RESULTS: In our series, a postoperative air-bone gap smaller than 10 dB was obtained in 77% of cases. These results confirm the possibility of managing both pathologies in a single surgical step. CONCLUSION: Incudo-mallear ankylosis remains an unusual pathology but should be systematically assessed during surgery and preferably after separation of the incudo-stapedial joint. A preoperative diagnosis is difficult to ascertain; some audiometrical features allow a suspicion. In this series, it is shown that otosclerosis can be responsible for ankylosis as seems to be confirmed by the two cases presented in this study.
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2/20. Lesions of the internal auditory canal and cerebellopontine angle in an only hearing ear: is surgery ever advisable?

    OBJECTIVE: To define the indications for surgery in lesions of the internal auditory canal (IAC) and cerebellopontine angle (CPA) in an only hearing ear. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. patients: Seven patients with lesions of the IAC and CPA who were deaf on the side opposite the lesion. Five patients had vestibular schwannoma (VS), and one each had meningioma and progressive osseous stenosis of the IAC, respectively. The opposite ear was deaf from three different causes: VS (neurofibromatosis type 2 [NF2]), sudden sensorineural hearing loss, idiopathic IAC stenosis. INTERVENTION(S): Middle fossa removal of VS in five, retrosigmoid resection of meningioma in one, and middle fossa IAC osseous decompression in one. MAIN OUTCOME MEASURE: Hearing as measured on pure-tone and speech audiometry. RESULTS: Preoperative hearing was class A in four patients, class B in two, and class C in one. Postoperative hearing was class A in three patients, class B in one, class C in two, and class D in one. CONCLUSIONS: Although the vast majority of neurotologic lesions in an only hearing ear are best managed nonoperatively, in highly selected cases surgical intervention is warranted. Surgical intervention should be considered when one or more of the following circumstances is present: (1) predicted natural history of the disease is relatively rapid loss of the remaining hearing, (2) substantial brainstem compression has evolved (e.g., large acoustic neuroma), and/or (3) operative intervention may result in improvement of hearing or carries relatively low risk of hearing loss (e.g., CPA meningioma).
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3/20. Audit of stapedectomy in the north west of england for 1996 and an analysis of the criteria used to describe success.

    A questionnaire was used to collect information related to stapedectomy from all the NHS Trusts in the North West region. The data collected included the identity and rank of the surgeon, the age and sex of the patients, the side of the operation, the audiometric values of the air conduction audiogram preoperatively and postoperatively, and an account of any complications. The results were analysed and compared to national and international standards. The criteria for analysis of success in surgery for conductive hearing loss were reviewed.
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4/20. Histopathology of residual and recurrent conductive hearing loss after stapedectomy.

    HYPOTHESIS: Histopathologic examination of temporal bones from patients who had undergone stapedectomy may provide information concerning the causes of both residual and recurrent conductive hearing loss (CHL). BACKGROUND: Although closure of the air-bone gap to within 10 dB occurs in approximately 90% of primary stapedectomies, a residual CHL occurs in approximately 10% and recurrent CHL may occur in up to 35% of cases. Putative causes of failure of surgery as determined during revision include erosion of the incus, bony regrowth at the oval window, and displacement of the prosthesis. Most reports on the histopathologic findings of temporal bones from such patients have focused on complications of surgery, with little attempt to correlate postoperative air-bone gap with the observed histopathology. methods: A retrospective review of the author's collection of temporal bones ascertained 22 cases with postoperative CHL of 10 dB or greater (air-bone gap averaged at 500, 1,000, 2,000, 3,000, and 4,000 Hz, using postoperative air- and bone-conduction levels) after stapedectomy. These temporal bones were prepared by standard methodology for light microscopy. RESULTS: Of the 22 cases with postoperative CHL equal to or greater than 10 dB, there were 19 with residual CHL, 2 with recurrent CHL, and 1 with both residual and recurrent CHL. The most common histopathologic correlates of residual and recurrent hearing loss included resorptive osteitis of the incus (64%); obliteration of the round window by otosclerosis (23%); the prosthesis lying on a residual footplate fragment (23%); the prosthesis abutting the bony margin of the oval window (18%); adhesions in the middle ear (14%); and new bone formation in the oval window (14%). CONCLUSIONS: Histopathologic examination of temporal bones from patients who in life had undergone stapedectomy provides useful information concerning causes of both residual and recurrent CHL. These data provide a basis for improving both surgical technique and prosthesis design.
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5/20. Reparative granuloma seen in cases of gold piston implantation after stapes surgery for otosclerosis.

    OBJECTIVE: to determine the occurrence of the unusual side effect of a reparative granuloma after the implantation of a pure gold piston in cases of otosclerosis. STUDY DESIGN: a retrospective case review study of 475 stapes operations with a pure gold piston. SETTING: Department of Otorhinolaryngology of the University of Amsterdam, The netherlands and the HNO clinic in Luenen (Brambauer) in germany. patients: four hundred and seventy five patients (328 women, 147 men, average age: 45.2 years), who clinically and per-operatively had otosclerosis, underwent a stapedotomy using a pure gold piston prosthesis. Therapeutic intervention: in cases of suspicion of a reparative granulomas or those cases that did not have improvement of the hearing after the stapedotomy, a re-operation by transcanal approach was performed. RESULTS: in seven cases a reparative granuloma was diagnosed by this revision surgery. The postoperative incidence of these granulomas following stapedotomy using the pure gold piston turned out to be 1.5%. CONCLUSION: reparative granulomas can occur after stapedotomy with a pure gold stapes piston although the incidence is low. The role of grafting material to seal the oval window niche and the treatment of these reparative granulomas following stapes surgery are discussed.
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6/20. Evaluation of cochlear function in a patient with "far-advanced" otosclerosis.

    A patient with "far-advanced" otosclerosis with a profound bilateral hearing loss was evaluated for cochlear reserve using transtympanic electrocochleography (ECoG) and promontory stimulation. Results indicated that both ECoG and promontory responses were present, indicating that cochlear function was present bilaterally. A stapedectomy resulted in closure of the air-bone gap. Postoperative hearing yielded excellent aided responses compared to no measurable preoperative hearing aid benefit. This case serves to demonstrate the value of evaluating cochlear function using these electrophysiologic techniques.
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7/20. Far advanced otosclerosis and intractable benign paroxysmal positional vertigo treated with combined cochlear implantation and posterior semicircular canal occlusion.

    This paper presents a combined procedure for the management of intractable benign paroxysmal positional vertigo (BPPV) and profound hearing loss in a patient with far advanced otosclerosis. The procedure comprised of a posterior semicircular canal occlusion and cochlear implantation as one combined procedure in the same ear. The combined approach added little to the operative morbidity and proved effective in this patient's management. A search of the literature reveals this to be a unique case.
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8/20. 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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9/20. 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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10/20. A new tool for testing ossicular mobility during middle ear surgery: preliminary report of four cases.

    OBJECTIVE: We developed an ossicular vibration tester for the objective and quantitative assessment of ossicular mobility, which is one of the most critical factors affecting postoperative hearing after tympanoplasty. methods: Our device consists of three components: a probe shaft with a curved tip to be attached to the target ossicle, a vibration exciter to activate the probe, and a piezoelectric sensor to detect vibrations of the probe. These components are encased in a stainless steel holder, allowing easy hand manipulation during ear surgery. The probe is activated with an electric signal at around 1,600 Hz. The system is controlled with a laptop computer, and the results are presented as the ratio of the ossicular resistance (ROR) to a reference value as a percentage. One measurement takes 10 ms. The device was applied in four selected patients during ear surgery. RESULTS: Several measurements in two of the cochlear implantees showed a greater difference in the RORs of the stapes (15-20% in Case 1 and 35-45% in Case 2), whereas the RORs of the malleus and incus were within the same range. This was thought to correspond to the partial cochlear calcification noted in Case 2. In Case 3, who underwent surgery because of otosclerosis, the ROR of the stapes was high, ranging from 70 to 80%. When measured for the malleus-incus fixation anomaly (Case 4), the ROR of the malleus and incus was in the range of 60 to 70%. Owing to the limited surgical view, the ROR of the stapes could not be measured. No problems related to the measurements with this device were noted. CONCLUSION: The design, principles, measuring procedures, and preliminary results of our new tool for testing ossicular mobility are reported. Measuring the ossicular mobility during surgery may provide important information for deciding the surgical procedures.
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