Cases reported "Cholesteatoma"

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1/13. Cochleo-vestibular manifestations of jugular foramen pathologies.

    patients presenting pathologies of the jugular foramen are presented, for whom the main symptoms are of cochleo-vestibular nature. A classification of the various pathologies is attempted, the anatomy reviewed and hypotheses capable of producing the symptom elaborated.
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2/13. Acute mastoiditis and cholesteatoma.

    Acute coalescent mastoiditis is an uncommon sequela of acute otitis media. It occurs principally in the well-pneumatized temporal bone. The findings of fever, pain, postauricular swelling, and otorrhea are classic. cholesteatoma, on the other hand, being associated with chronic infection, usually occurs in the sclerotic temporal bone. The signs and symptoms are isidious in nature and consist of chronic discharge and hearing loss which result from its mass, bone erosion, and secondary infection. Of 17 consecutive cases of acute mastoiditis over a six-year period, four were atypical because they were complications of chronic otitis media and cholesteatoma, yet they had the physical findings of acute mastoiditis-subperiosteal abscess and purulent otorrhea, plus radiographic evidence of mastoid coalescence.
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3/13. Middle ear adenoma is an amphicrine tumor: why call it adenoma?

    Middle ear adenoma (MEA) is a rare tumor postulated to take origin from the lining epithelium of the middle ear cavity. The authors report on a case of MEA arising in a 53-year old woman suffering from a sensation of fullness in her left ear, otalgia, and light left-sided hearing loss. Histopathologically, the lesion was composed of cuboidal and polygonal cells displaying a trabecular, tubulo-glandular, and solid pattern of growth. Immunohistochemically, neoplastic cells diffusely stained with anti-vimentin antibodies and were focally positive for chromogranin a, neuron-specific enolase, lysozyme, and cytokeratins AE1/AE3. The majority of tumor cells showed weak and diffuse staining with both anti-PP and anti-ACTH antibodies and intense positivity with anti-glucagon and anti Leu-7 antibodies. Ultrastructural investigation revealed both mucinous-glandular and neuroendocrine differentiation. The authors suggest that the appropriate terminology would be adeno-carcinoid or amphicrine tumor of the middle ear rather than "adenoma," a term that does not reflect its dual nature.
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4/13. Supralabyrinthine approach to petrosal cholesteatoma.

    Petrosal cholesteatomas are rare lesions, which may be congenital or acquired in nature. We report an exceptional case occurring in a seven-year old girl who presented with a unilateral conductive hearing loss, despite normal tympanic membrane appearance. early diagnosis was facilitated by computed tomography (CT) scanning. Although this case satisfied the criteria for congenital cholesteatoma, it is likely that the petrous apex was secondarily involved. Complete cholesteatoma removal was accomplished using a transtemporal supralabyrinthine approach, which allowed for hearing preservation, while avoiding the morbidity associated with a craniotomy. The present case constitutes the youngest case of petrosal cholesteatoma reported. We suggest that a lower threshold for the use of CT scanning in unilateral conductive hearing loss may allow for the earlier detection of more cases of petrosal cholesteatomas, as well as facilitating their removal using more limited approaches associated with less morbidity.
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5/13. Foreign body granuloma of the external auditory canal.

    External auditory canal polyps are most commonly inflammatory in nature but may also manifest more severe disease. Prolonged conservative therapy may delay the correct diagnosis and appropriate intervention. A case is presented of a child with chronic otorrhea treated for 4 months with topical drops and antibiotics. On referral, a large external auditory canal polyp was confirmed to represent a foreign body granuloma covering a large electrical cap, with erosion approaching the facial nerve. External auditory canal polyps that fail to respond promptly to conservative medical therapy warrant a computed tomography scan and surgical exploration with biopsy.
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6/13. Giant cholesterol granuloma producing brainstem compression.

    cholesterol granuloma of the petrous apex is an unusual clinical entity that has received much attention in the recent literature. Not considered to be a life-threatening lesion, cholesterol granuloma usually presents with a variety of cranial nerve findings. A case of brainstem compression caused by a giant cholesterol granuloma is presented. This lesion prompted emergency surgical intervention with dramatic relief of neurologic symptoms. The unusual nature of this case is compared with the more common presentation of cholesterol granuloma.
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7/13. Giant invasive cholesteatoma. Report of a case with cerebellar invasion.

    We present a case of epidermoid cholesteatoma that killed the patient by relentless invasion, despite radical surgery. An autopsy disclosed a focus of dural and cerebellar invasion, which dramatized the aggressive nature of this disease. In fact, the epithelial cells of this disease are not thought to possess the ability to invade. Instead, inflammation is believed to both stimulate the squamous epithelial cells to proliferate and destroy adjacent tissues, thereby providing a pathway for epithelial cell migration. Epidermoid cholesteatoma, then, is a disease in which the processes of inflammation and hyperplasia combine to mimic a neoplasm.
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8/13. Surgery in lesions of the petrous apex.

    It is fortunate that lesions of the petrous apex are rarely encountered. The occult nature of the local disease and the poorly localizing clinical features favor a delayed diagnosis. Its site deep to the labyrinth and facial nerve and its proximity to the internal carotid artery and brain stem potentially make surgery a risky prospect. The reconciling of preservation of neurologic function and adequate surgical exposure requires an extensive knowledge of temporal bone anatomy and surgical approaches.
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9/13. Bilateral congenital middle ear cholesteatomas.

    Congenital middle ear cholesteatomas remain of interest because of their relative rarity and unknown origin. A 4-month-old child presenting with bilateral congenital middle ear cholesteatomas forms the basis for this report. The nature of the epithelial debris found suggests an external origin. An extended transcanal tympanotomy approach for removal, based upon an operative experience of 11 similar ears, will be discussed.
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10/13. Surgery of the skull base.

    Tumors involving the base of the skull are often occult and may become quite large before detection is possible. Symptomology varies depending upon the nature of the tumor and its placement. These neoplasms have frequently been considered inoperable simply because of their location, but in recent years microsurgical technique and high speed air drills have allowed the modern temporal bone surgeon to gain improved access to the skull base. A wide variety of surgical approaches to tumors in this area have been described in the past 20 years: translabyrinthine, middle fossa, transcochlear, retrolabyrinthine, retrosigmoid, transpalatal-transclival, and through the jugular bulb. The purpose of this paper is to review the indications, complications, and results of each of these procedures in relation to specific tumors involving the skull base. case reports illustrate the diagnosis and surgical management of a variety of unusual neoplasms, including an extradural meningioma of the temporal bone and clivus, a low grade squamous cell carcinoma on the tegmen in a radical cavity, a large primary cholesteatoma, an osteoblastoma of the temporal and occipital bones, an XIth nerve neuroma in the jugular bulb area, and an osseous hemangioma involving the facial nerve at the geniculate ganglion.
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