Cases reported "Otosclerosis"

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1/16. 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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2/16. 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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3/16. Superior semicircular canal dehiscence simulating otosclerosis.

    This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.
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4/16. Perilymphatic gusher in stapedectomy: demonstration of a fistula of internal auditory canal.

    Gusher is a very rare phenomenon, generally associated with congenital stapes fixation or otosclerosis in adult age, that may present during stapedectomy. A sudden perilymph flow occurs following platinotomy, due to congenital malformation (abnormally wide cochlear aqueduct or internal auditory canal fistula), that causes an abnormal connection between subarachnoid and perilymphatic spaces. This report deals with a case of bilateral gusher, occurring during stapedectomy, caused by an osseous fistula between bottom of internal auditory canal and the osseous labyrinth later observed at computed tomography scan. The usefulness of a radiologic examination is stressed for a correct therapeutic approach in the even contralateral ear stapedectomy.
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5/16. An unusual complication of stapes surgery: profuse bleeding from the anteriorly located sigmoid sinus.

    Major bleeding during stapes surgery is a rare condition. The anterior course of the sigmoid sinus is comparatively common in contracted mastoid bones, but rare in good pneumatized temporal bones. We report a case of an unusual anterior course of the vertical segment of the sigmoid sinus, which led to profuse bleeding during a stapedotomy operation. The 34-year-old female patient presented with conductive-type hearing loss in the right ear. During the operation, the unintentional sliding movement of a blunt curette caused injury to the anteriorly positioned sigmoid sinus, which was separated from the posterior wall of the external auditory canal by an extremely thin shell of bone. Profuse bleeding from the sigmoid sinus was controlled by pushing the middle part of the oxidised regenerated cellulose inside the lumen, without compromising the sinus flow. After bleeding was restrained, the stapedotomy operation was completed successfully. During the 1-year follow-up, there was neither an air-bone gap nor a sensorineural hearing loss in pure tone audiogram.
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6/16. 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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7/16. The unilateral stapes gusher.

    A stapes gusher is an alarming situation occurring during stapes surgery for mixed hearing loss. It is characterized by the sudden profuse flow of cerebrospinal fluid in the middle ear and external ear canal after opening the vestibule of the inner ear. An adult patient with unilateral stapes gusher is described; he was operated on both ears with a good hearing result on both sides. The stapes on the side with the cerebrospinal fluid leak was malformed, especially in the posterior crus--a finding which may suggest the possibility of a stapes gusher. The small-hole stapedotomy is a safe technique when dealing with a profuse cerebrospinal fluid leak, and it enables the closure of the leak with additional tamponade of the oval niche and the achievement of a good hearing result.
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8/16. Mondini-like malformation mimicking otosclerosis and superior semicircular canal dehiscence.

    In 2003, it was reported that superior semicircular canal dehiscence can mimic otosclerosis because of low-frequency bone conduction hearing gain and dissipation of air-conducted acoustic energy through the dehiscence. We report the case of a 17-year-old girl with left-sided combined hearing loss thought to be due to otosclerosis. bone conduction thresholds were -10 dB at 250 and 500 Hz and she had a 40 dB air-bone gap at 250 Hz. When a tuning fork was placed at her ankle she heard it in her left ear. Acoustic reflexes and vestibular evoked myogenic potentials could be elicited bilaterally. Imaging of the temporal bones showed no otosclerosis, superior semicircular canal dehiscence or large vestibular aqueduct, but a left-sided, Mondini-like dysplasia of the cochlea with a modiolar deficiency could be seen. Mondini-like cochlear dysplasia should be added to the causes of inner-ear conductive hearing loss.
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9/16. 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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10/16. endolymphatic hydrops after fenestration: a temporal bone study with implications on the function of the utriculo-endolymphatic valve.

    A temporal bone specimen demonstrating endolymphatic hydrops 13 years after fenestration of the lateral semicircular canal is presented. Fibro-osseous tissue extending from the lateral semicircular canal and reaching the vestibule produced fixation of the membranous wall of the utricle to the bony wall. Fixation and retraction of the utricule appears to have resulted in a permanently open utriculo-endolymphatic valve leaflet. Similar findings of fibro-osseous changes arising from the area of the crus commune and an open valve were found in a Meniere's disease specimen. The implications of these findings on the function of the valve are discussed.
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