Cases reported "Labyrinth Diseases"

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1/45. cholesteatoma extending into the internal auditory meatus.

    We report our experiences in managing a patient with cholesteatoma complicated by meningitis, labyrinthitis and facial nerve palsy. The antero-inferior half of the tympanum was aerated but the postero-superior portion of the tympanic membrane was tightly adherent to the promontry mucosa. An attic perforation was present at the back of the malleolar head. High-resolution computed tomography also uncovered a fistula in the lateral semicircular canal. Surgical exploration of the middle ear cavity demonstrated that both the vestibule and cochlea were filled with cholesteatoma, and the cholesteatoma extended into the internal auditory meatus through the lateral semi-circular canal fistula. The cholesteatoma was removed by opening the vestibule and cochlea with a preservation of the facial nerve. Post-operatively, an incomplete facial palsy remained, but has improved slowly. There is no sign of recurrence to date after a 3-year period of observation.
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keywords = operative
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2/45. meningioma of the internal auditory canal with extension into the vestibule.

    Meningiomas account for approximately 18 to 19 per cent of all brain tumours. Although they can arise in numerous locations, meningiomas of the internal auditory canal (IAC) are rare. Most tumours that originate in the IAC are schwannomas of the VIIIth cranial nerve (acoustic neuromas). We report a case of a meningioma which appears to originate from the IAC and extends into the vestibule. The clinical findings and the radiographical features of meningiomas of the IAC are similar to those of acoustic neuromas. Pre-operative differentiation between acoustic neuromas and meningiomas of the IAC may be difficult.
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ranking = 1
keywords = operative
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3/45. 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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ranking = 4
keywords = operative
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4/45. syndrome of recurrent meninigitis due to congenital perilymph fistula with two different clinical presentations.

    Recurrent meningitis secondary to a congenital labyrinthine anomaly is a rare clinical entity, diagnosis of which is dependent upon certain clinical, radiological and intraoperative features. In the following report we describe two children with congenital labyrinthine fistula and recurrent meningitis whose clinical presentation, radiological features and intraoperative findings were dissimilar and thus, illustrative of two different ways of presentation of this rare disorder. While one had a classical Mondini defect and unilateral hearing loss, the other had normal audiometric and radiographic findings. The fistulae were successfully closed via a tympanotomy approach in both the patients.
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ranking = 2
keywords = operative
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5/45. Traumatic perilymphatic fistulas in children: etiology, diagnosis and management.

    Post-traumatic perilymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. However, indications for exploratory surgery in cases of trauma without temporal bone fracture are vague and not well described. We describe three children who presented with symptoms suggestive of perilymphatic fistula (PLF) without an associated temporal bone fracture: two with penetrating tympanic membrane injuries and one with blunt temporal bone trauma. All had symptoms of hearing loss and vestibular disturbance. Two of the children cooperated with ear-specific audiologic assessment, which demonstrated sensorineural hearing loss (SNHL) on the traumatized side. The third child showed audiometric evidence of a SNHL on the injured side, but due to his age, the degree of severity of the SNHL was unable to be appropriately addressed prior to the patient being surgically managed. All three children underwent exploratory surgery and were found to have bony defects in the region of the oval window. All were repaired with fascial grafts to the oval and round windows with complete resolution of vestibular symptoms. However, two of the three patients with documented post-operative audiograms suffered from persistent SNHL on the injured side. We conclude that exploratory middle ear surgery is indicated in patients suffering from blunt or penetrating temporal bone or middle ear trauma who demonstrate persistent vestibular symptoms, sensorineural hearing loss or radiographic evidence of oval window pathology. As this is a limited number of patients, a larger series may be warranted to study the actual incidence of post-traumatic PLF in the child with persistent hearing loss and vertigo after head or ear trauma.
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ranking = 1
keywords = operative
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6/45. Labyrinthine fistula detection: the predictive value of vestibular symptoms and computerized tomography.

    A retrospective case record study of 20 patients in Oslo operated on for chronic otitis media with labyrinthine fistula between 1986 and 1999 was performed in order to estimate the incidence of, and identify predictors for, labyrinthine fistulas. The incidence of fistula was 0.3 per 100 000, with a median age at diagnosis of 37 years. The median duration of chronic otitis media prior to labyrinthine fistula detection was significantly correlated with age at surgery. Subjective hearing loss (90%), otorrhoea (65%) and dizziness (50%) were presenting symptoms. Modified canal-wall-down mastoidectomy was performed in all patients. Preoperative hearing levels could not predict postoperative hearing outcome. Positive signs of fistula were found in only 4 patients (20%). Correspondingly, computerized tomography (CT) diagnosed the fistula in 11 patients (55%). The seven patients presenting without dizziness and with a negative CT scan and fistula test were characterized by lower age, absence of previous middle ear surgery, lower preoperative pure-tone thresholds for bone conduction and better hearing outcome after surgery. In conclusion, the identification of a younger group of patients presenting with fewer symptoms indicates that fistulas should be suspected in all patients undergoing surgery for chronic middle ear and mastoid disease.
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ranking = 3
keywords = operative
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7/45. Hamartomas of the internal auditory canal: report of two cases.

    OBJECTIVE AND IMPORTANCE: To highlight the clinical, radiological, and surgical findings and therapeutic options for this rare entity, which may mimic a purely intrameatal vestibular schwannoma, and to define the particular aspects of preoperative differential diagnosis and surgical management. CLINICAL PRESENTATION: Two patients presented with clinical findings typical of vestibular schwannomas, i.e., tinnitus, hearing loss of 30 dB, and an intrameatal contrast-enhancing lesion on magnetic resonance imaging studies. TECHNIQUE: The lesions were exposed via a suboccipital transmeatal approach, and tumor infiltration of the cochlear and/or facial cranial nerves was identified. In view of the unclear intraoperative histology, surgical management was based on criteria of cranial nerve function. In Patient 1, after nerve decompression by subtotal tumor removal, preserved auditory brainstem responses and facial nerve electromyography indicated functional nerve preservation and facilitated the decision for partial resection. In Patient 2, minimal tumor dissection resulted in complete loss of auditory brainstem response without reversibility. Therefore, a radical tumor removal was performed that sacrificed the cochlear but preserved the facial nerve. CONCLUSION: Symptoms and signs of internal auditory canal hamartomas are congruent with other typical pathological lesions of the internal auditory canal and cerebellopontine angle. Accurate preoperative diagnosis by radiological means is not possible, but careful evaluation of the different signal intensities on magnetic resonance imaging studies may indicate this rare pathological condition. Intraoperative surgical findings of tumor infiltration of the faciocochlear cranial nerve complex may support simple observation. In view of the nonneoplastic characteristic of these lesions, a more conservative approach is justified. The decision should be based on the functional status of the cranial nerves, for which reliable electrophysiological monitoring is indispensable.
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ranking = 4
keywords = operative
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8/45. Inner ear extension of vestibular schwannomas.

    OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, italy, in this field. STUDY DESIGN: Case report and literature review. methods: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.
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ranking = 2
keywords = operative
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9/45. Traumatic perilymphatic fistula: how long can symptoms persist? A follow-up report.

    In the past 18 years 68 ears (average 3.8 per year) were explored for perilymphatic fistula (PLF). A total of nine (13%) ears had a fistula identified at operation. patients with a previous history of otologic surgery were excluded from this review. The most common etiology for PLF was head trauma (4 of 9). Most patients had persistent symptoms lasting months (average 6.7). Eighty-three percent of all patients had sudden or fluctuating hearing loss, 77 percent had vertigo or dysequilibrium, and 61 percent had tinnitus. vertigo was the most commonly improved symptom postoperatively, and only 25 percent of patients had improved hearing. There were no major complications. The authors discuss indications for operation, criteria for diagnosis of PLF, and audiometric and electronystagmographic findings. This report agrees with other recent data indicating that exploration for fistula is an uncommon procedure performed by otologists.
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ranking = 1
keywords = operative
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10/45. Inflammatory pseudotumor of the temporal bone.

    OBJECTIVE: To characterize the clinical presentation, imaging characteristics, intraoperative findings, and key histopathologic features of inflammatory pseudotumors of the temporal bone. Findings from an index case are presented, and the literature is reviewed for comparison. STUDY DESIGN: Retrospective case review. SETTING: University tertiary referral center. patients: Cases were identified by review of surgical specimens from the temporal bone and lateral skull base with histopathologic confirmation. A single case was identified at our institution. Nine additional cases were identified in the literature; clinical features were reviewed. INTERVENTION: Of reported cases, treatment consisted of complete surgical excision in eight cases and subtotal excision in one. The index patient underwent surgical excision with postoperative corticosteroid therapy for adjacent meningeal involvement, after histopathologic interpretation. Corticosteroids were administered to one patient with residual microscopic tumor, and external beam radiotherapy was used for residual/recurrent disease in one case. RESULTS: The lesions were typically locally aggressive with extensive bony erosion. Three cases (33%) demonstrated labyrinthine and otic capsule involvement. Four cases (44%) involved the facial nerve. Characteristic histopathologic features included fibroblastic proliferation and a mixed inflammatory cell infiltrate in all cases. Mitotic figures, nuclear pleomorphism, and necrosis were rare or nonexistent. CONCLUSIONS: Inflammatory pseudotumors of the temporal bone are rare but aggressive lesions. Therapy should consist of surgical excision with steroids reserved for residual or intracranial disease or in patients in whom surgery is not an option. These lesions must be differentiated from other infectious, granulomatous, and neoplastic lesions on the basis of histopathologic and immunohistochemical findings.
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ranking = 2
keywords = operative
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