Cases reported "Otosclerosis"

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1/114. Does otosclerosis occur only in the temporal bone?

    HYPOTHESIS: otosclerosis does not occur outside the temporal bone. BACKGROUND: The widely accepted assumption that otosclerosis is confined to the temporal bone has never been tested. It is important to investigate this issue, particularly because of evidence that otosclerosis may be a systemic (genetic) disease that could affect other bones. methods: Biopsies from 9 to 11 skeletal sites were obtained from 2 patients with clinical otosclerosis. Two hundred forty-one nontemporal bone sections were examined by light microscopy. RESULTS: No nontemporal skeletal bone section showed histologic evidence of otosclerosis. The data indicate, with 95% confidence, that the true prevalence of otosclerosis in the extratemporal skeleton of the 2 patients examined was < 3%. CONCLUSIONS: These findings suggest that otosclerosis is unlikely to occur outside the temporal bone. Factors unique to the otic capsule that may predispose it to otosclerosis are lack of bone remodeling and the presence of globuli interossei. ( info)

2/114. 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. ( info)

3/114. 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. ( info)

4/114. Pure sensorineural hearing loss and otosclerosis. An imaging case report.

    Pure sensorineural hearing loss is not a rare finding in otological practice. Numerous aetiologies could be at the origin of such a deficit. However, otosclerosis is very rarely cited as a cause of pure sensorineural hearing loss. We present one such case of pure sensorineural hearing loss linked to otosclerosis in a 30-year old caucasian male and underline the high contribution of computed tomography to confirm such a diagnosis. Pure sensorineural hearing loss due to otosclerosis is a rare event and can be misdiagnosed. The clinical diagnosis of such a disease may be difficult. In these cases, CT-Scan is the exam of choice to confirm the diagnosis. ( info)

5/114. 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). ( info)

6/114. Fenestration surgery for otosclerosis: CT findings of an old surgical procedure.

    Numerous attempts to deal surgically with otosclerosis were made before the current method of stapedectomy with stapes prosthesis was established. We report a case with unique CT findings of a patient who underwent fenestration surgery for otosclerosis in the early 1940s. Recognition of this old surgical procedure on the imaging scans may avoid misdiagnosis of labyrinthine fistulae or middle and inner ear malformations. ( info)

7/114. MRI-visible pericochlear lesions in osteogenesis imperfecta type I.

    osteogenesis imperfecta (OI) is an inherited generalized disorder of type-I collagen synthesis often associated with hearing loss. We present a case of OI type I in which hearing loss led to examination of the temporal bone with MRI. In the osseous otic capsule MRI demonstrated pericochlear lesions with soft tissue signal intensity and contrast enhancement. Changes similar to otosclerosis have been described in the temporal bone of OI patients when applying CT, but reports on MRI findings do not yet exist. ( info)

8/114. 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. ( info)

9/114. meningitis following stapedotomy: a rare and early complication.

    Controversy exists concerning stapedotomy for patients with small unilateral air-bone gaps. Surgical treatment of otosclerosis involves an opening to the labyrinth and accordingly, a risk of complications, usually vertigo and sensorineural hearing loss and infrequently anacusis. In this paper we present a 33-year-old woman with a small unilateral air-bone gap, who developed bacterial labyrinthitis with meningitis and anacusis three days after stapes surgery. The patient had a stapedotomy with the small fenestra piston prosthesis technique. Due to the potential for serious complications, patients with unilateral otosclerosis and mild hearing loss should be given the possibility to choose between a hearing aid and surgery. Although stapedotomy in the vast majority of interventions is a highly successful procedure and the best method of treatment for otosclerosis if successful, there is a high price to pay in the event of failure. ( info)

10/114. 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. ( info)
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