Cases reported "Ear Diseases"

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1/92. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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keywords = bone
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2/92. A simple reconstructive procedure for radiation-induced necrosis of the external auditory canal.

    Localized necrosis of the bone, cartilage, and soft tissue of the external auditory canal is an uncommon side effect of radiotherapy to the parotid region. Five patients developed late onset skin necrosis of a quadrant of the ear canal secondary to an underlying osteoradionecrosis of the tympanic ring. We report a one-stage procedure to excise the necrotic tissue and replace it with a local rotational flap derived from the post-auricular skin. Otological side effects of radiotherapy are discussed.
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3/92. An unusual case of otogenic pneumocephalus.

    Otogenic pneumocephalus is a rare entity usually caused by temporal bone trauma. This paper describes a case of otogenic pneumocephalus of traumatic origin, in which the type of the fracture (a bony spicula was detached from the mastoid) and the location (Trautmann's triangle) were uncommon.
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4/92. Intraosseous ganglion of the temporomandibular joint presenting with otorrhea.

    A ganglion cyst of the temporomandibular joint is a rare entity that commonly presents as a minimally tender, preauricular mass. This benign cystic lesion, which is lined by synovium and can be found in association with other joints, occasionally erodes adjacent bone to form an intraosseous ganglion. We discuss an unusual case of an intraosseous temporomandibular ganglion cyst that presented with bloody otorrhea. Examination revealed an external auditory canal mass with radiographic evidence of temporal bone erosion. The cyst was excised using a combined approach to the glenoid fossa and mastoid. The management principals of this case and a pertinent review of the literature are included.
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keywords = bone
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5/92. External cholesteaoma and fibrous dysplasia of temporal bone.

    cholesteatoma is a disease that involves almost exclusively the middle ear structures and the mastoid bone. In rare cases it involves the external auditory canal. The author would like to present case report of a patient affected by external ear canal cholesteatoma associated to fibrous dysplasia of the temporal bone. The problems related to the pathogenesis and the diagnosis of the disease are presented and discussed.
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ranking = 3
keywords = bone
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6/92. Glial lesion of the infratemporal fossa presenting as a soft tissue middle ear mass - rudimentary encephalocele or neural crest remnant?

    We report about ectopic glial tissue of the skull base and the parapharyngeal space presenting as a soft tissue mass in the middle ear. An 11-year-old boy presented with bilateral conductive hearing loss since early childhood. The history included previously removed lesions consistent with ectopic neuroglial tissue of the tongue and the parapharyngeal space soon after birth, as well as surgery for cleft palate. High resolution computed tomography of the petrous bone and magnetic resonance imaging were useful in identifying the skull base defect and in characterizing the lesion's relation to the brain. There was no clinical, radiological or surgical evidence of any associated dural defect. The lesion was removed via a modified infratemporal approach. histology revealed neuroglial tissue with calcifications without any signs of mesodermal or entodermal origin. On the basis of this case the pathogenesis and diagnosis of ectopic brain tissue and its relation to the more commonly encountered meningoencephalic herniations are reviewed. Furthermore therapeutical implications are discussed.
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7/92. rupture of an internal carotid artery aneurysm in the petrous temporal bone.

    A case of otorrhagia following the rupture of an aneurysm of the intrapetrous portion of the internal carotid artery is presented. The aneurysm was successfully treated by balloon occlusion of the parent artery. The problems associated with diagnosis and clinical management of these lesions is discussed and possible solutions suggested.
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ranking = 2
keywords = bone
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8/92. 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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keywords = bone
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9/92. Eighth nerve aplasia and hypoplasia in cochlear implant candidates: the clinical perspective.

    OBJECTIVE: To identify the clinical and radiologic characteristics of aplasia and hypoplasia of the eighth nerve. STUDY DESIGN: Retrospective case-note review. SETTING: Cochlear implant program. patients: All children at the authors' institution in whom the cochlear implant assessment failed because of absence or hypoplasia of the eighth nerve. INTERVENTION: Computed tomography of petrous bones and magnetic resonance imaging of the brain. MAIN OUTCOME MEASURES: Presence or absence of eighth nerve and other radiologic factors contraindicating implantation. RESULTS: Of 143 cochlear implant candidates, 237 were judged ineligible for cochlear implantation. The preimplant assessment failed in 10 candidates of 143 because of bilateral aplasia or hypoplasia of the eighth nerve (7 cases) or unilateral aplasia or hypoplasia of the eighth nerve and a contraindication to operation on the other side (3 cases). The aplasia or hypoplasia of the eighth nerve was confirmed by magnetic resonance imaging in seven cases (5%): six were syndromic (3 CHARGE, 1 VATER-RAPADILLINO, 1 Mobius, 1 Okihiro), and one was nonsyndromic autosomal-recessive. All seven children had delayed motor milestones and absence of auditory brainstem responses. CONCLUSION: Aplasia and hypoplasia of the eighth nerve are not uncommon in pediatric cochlear implant candidates, particularly in the presence of a syndrome such as CHARGE. magnetic resonance imaging of the brain is mandatory before implantation because it can identify the presence or absence of the eighth nerve. parents of children with profound hearing loss, delayed motor milestones, absence of auditory brainstem responses, and a syndromic diagnosis, should be made aware of this possible abnormality.
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keywords = bone
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10/92. Spontaneous external auditory canal cholesteatoma complicated by rheumatoid arthritis--case report and review of the literature.

    A 63-year-old woman with rheumatoid arthritis sought medical assistance for dull and chronic pain in her left ear two and half years after her initial diagnostic examination. Otoscopic examination revealed that the posteroinferior wall of the bony external ear canal was eroded and that the small cavity was filled with squamous debris. The condition was diagnosed as external auditory canal cholesteatoma (EACC). The existence of EACC might suggest complications of bone disease, aging cerumen gland, or a low migratory rate of the epithelium.
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