Cases reported "Otosclerosis"

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1/10. 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.
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ranking = 1
keywords = vertigo
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2/10. 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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ranking = 5
keywords = vertigo
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3/10. Posterior semicircular canal dehiscence: value of VEMP and multidetector CT.

    OBJECTIVE: To illustrate that posterior semicircular canal dehiscence can present similarly to superior semicircular canal dehiscence. CASE STUDY: The symptomatology initially presented as probable Meniere's disease evolving into a mixed conductive hearing loss with a Carhart notch-type perceptive component suggestive of otosclerosis-type stapes fixation. A small hole stapedotomy resulted in a dead ear and a horizontal semicircular canal hypofunction. Recurrent incapacitating vertigo attacks developed. Vestibular evoked myogenic potential (VEMP) testing demonstrated intact vestibulocollic reflexes. Additional evaluation with high resolution multidetector computed tomography (MDCT) of the temporal bone showed a dehiscence of the left posterior semicircular canal. CONCLUSIONS: Besides superior semicircular canal dehiscence, posterior semicircular canal dehiscence has to be included in the differential diagnosis of atypical Meniere's disease and/or low tone conductive hearing loss. The value of performing MDCT before otosclerosis-type surgery is stressed. VEMP might contribute to establishing the differential diagnosis.
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ranking = 1
keywords = vertigo
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4/10. hearing loss with delayed onset of vertigo.

    The association between the delayed onset of episodic true vertigo and previous sensorineural hearing loss has been previously described. We have reviewed our series of sixty consecutive labyrinthectomies to determine the prevalence of this disorder in this population, and found a variety of etiologic agents of sensorineural hearing loss with subsequent delayed vertigo. Relief from vertigo following surgery has confirmed that the previously deafened ear has been the cause of the vertigo. Case histories are presented and the literature is reviewed.
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ranking = 8
keywords = vertigo
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5/10. Otosclerotic inner ear syndrome.

    Twenty-seven patients with clinical otosclerosis and vertigo underwent stapedectomies. vertigo improved or completely disappeared in 23 patients, indicating that stapedectomy is probably helpful. Three case reports are also presented as well as photomicrographs of temporal bones showing the focus in contact with the pars superior.
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ranking = 1
keywords = vertigo
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6/10. otosclerosis and Meniere's syndrome: diagnosis and treatment.

    Occasionally a patient with otosclerosis and a conductive hearing loss will develop typical findings of Meniere's syndrome in the involved ear, years later. A review of clinical and pathological studies in the literature and in our laboratory and clinic indicates a likely cause-and-effect relationship for these cases. The pathology and pathogenesis of the syndrome of otosclerosis and Meniere's syndrome is discussed. A stapedectomy/sacculotomy was used to treat 17 patients, 13 of whom acquired a satisfactory result in terms of improvement of hearing and control of vertigo. This technique and findings are described and discussed.
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keywords = vertigo
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7/10. otosclerosis and endolymphatic hydrops.

    It has been postulated that otosclerosis may produce vertigo by several mechanisms. One mechanism is by causing endolymphatic hydrops. We present six temporal bones in which otosclerosis and endolymphatic hydrops coexist. We consider that there is a spectrum-like interrelationship between these two entities. At one end of the spectrum the relationship is coincidental, while at the other end of the spectrum we consider the massive amount of active otosclerosis to be a causative factor in the development of the endolymphatic hydrops.
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keywords = vertigo
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8/10. Fluctuating hearing loss and recurrent vertigo in otosclerosis. An audiologic and temporal bone study.

    This report summarizes audiologic and temporal bone findings in a 47-year-old white female with recurrent attacks of vertigo, tinnitus, and bilateral hearing loss. A series of audiograms, obtained over a 15-year period, showed a fluctuating mixed (sensorineural and conductive) hearing loss with a relatively flat configuration in the left ear and a relatively stable, mild sensorineural loss with a sloping contour in the right ear. temporal bone studies revealed the existence of otosclerotic foci bilaterally. In the left ear, the otosclerotic focus has grown close to the utricular and lateral ampullary nerves, displaced and ankylosed the stapedial footplate, invaded the endosteal zone (1,000-2,000 Hz area), and deformed the lateral cochlear wall. In the right ear, the otosclerotic invasion was limited to the inferior portion on the promontory (vestibular cecum) and round window niche.
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ranking = 5
keywords = vertigo
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9/10. endolymphatic hydrops associated with otosclerosis.

    endolymphatic hydrops (EH) associated with otosclerosis has been noted for many years. However, the causal relationship of these two entities remains controversial. Having reviewed the records of patients with otosclerosis describing fluctuant hearing loss and vertiginous symptoms, the authors found the EH may coexist with otosclerosis preoperatively; they may be two separate diseases that exist coincidentally; or EH may be caused by the otosclerotic process. Secondly, EH may occur with a fistula after surgery. Occurring after stapedectomy, EH may be caused by fistulization of the bony labyrinth, which is effectively treated by surgery to seal the fistula, which may cause EH to subside and hearing to improve. Thirdly, delayed EH may occur months or years after stapedectomy, possibly as a result of otosclerotic foci or surgical insult to the labyrinth. dexamethasone, diuretics, and a room air rebreather can be used in the treatment of delayed EH. Hearing may be maintained or may deteriorate, but there usually is no dizziness. The clinical manifestations of EH associated with otosclerosis include a conductive or mixed type of hearing loss; the presence of fullness, tinnitus, fluctuation of hearing, episodic vertigo, an elevated negative summating potential (SP), and an increased summating potential:action potential (SP:AP) ratio shown by ECoG. This report presents five cases of EH associated with otosclerosis.
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ranking = 1
keywords = vertigo
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10/10. Camurati-Engelmann disease presenting as 'juvenile otosclerosis'.

    Camurati-Engelmann disease (CED, MIM # 131300) was diagnosed in a 27-year-old woman, who had pain in her lower legs. There was no history of hearing loss (or CED) in her family. She had been suffering from progressive hearing impairment since childhood. Serial audiograms demonstrated progressive mixed hearing loss from the age of 11 years onwards, for which she underwent successful stapes surgery bilaterally at the age of 13-18 years. Clinical ('juvenile') otosclerosis was found with stapes footplate fixation. Unfortunately, the sensorineural hearing loss component proved to be progressive bilaterally after surgery. At the age of 30 years, she experienced more rapid deterioration of her hearing loss in the right ear and episodes of vertigo. Vestibular examinations demonstrated progressive vestibular impairment 'otospongiotic' lesions with encroachment on the cochlear and vestibular labyrinth were found on high resolution CT scans at the age of 40 years; the internal auditory canal was normal bilaterally. CED may have formed a chance combination with juvenile otosclerosis in our patient, or otosclerosis may be part of the CED phenotype.
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ranking = 1
keywords = vertigo
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