Cases reported "Cranial Nerve Diseases"

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1/17. Malignant external otitis with multiple cranial nerve involvement.

    A case of bilateral malignment external otitis with multiple cranial nerve deficits is presented. Thirty-five similar cases reported in the world literature are reviewed. All cranial nerves have been involved with the exception of the first and fourth. The resultant pseudomonas ostemyelitis may be spread extensively in these elderly diabetic patients to involve the entire base of the skull as well as other structures. The preferred treatment is long term systemic antibiotics followed by surgical intervention for plateau or further progression of disease. The overall mortality is 61 percent (22/36), a lower figure than previously reported.
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keywords = otitis
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2/17. Multiple cranial nerve palsies complicating tympanomastoiditis: case report.

    otitis media either acute or chronic, is not uncommon in childhood. Multiple cranial nerve palsies occuring as a complication of either form of otitis media is unusual. A case of a nine year old boy with chronic suppurative otitis media with associated mastoiditis complicated with ipsilateral multiple cranial nerve palsies is presented. A skull X-ray and MRI scan showed sclerotic mastoids. The outcome on antibiotic treatment was good.
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ranking = 0.4
keywords = otitis
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3/17. Central skull base osteomyelitis in patients without otitis externa: imaging findings.

    BACKGROUND AND PURPOSE: skull base osteomyelitis typically arises as a complication of ear infection in older diabetic patients, involves the temporal bone, and has pseudomonas aeruginosa as the usual pathogen. Atypical skull base osteomyelitis arising from the sphenoid or occipital bones without associated external otitis occurs much less frequently and initially may have headache as the only symptom. The purpose of this study was to review the clinical and MR imaging features of central skull base osteomyelitis. methods: We retrospectively reviewed MR images obtained in six patients with central skull base osteomyelitis. No patient had predisposing external otitis or osteomyelitis of the temporal bone. RESULTS: All of our patients presented with headache, no external ear pain, and cranial nerve deficits. Five of six patients had a predisposition to infection, and the erythrocyte sedimentation rate was elevated in the five patients in whom it was checked. In each case, the diagnosis was delayed until MR imaging demonstrated central skull base abnormality, and the diagnosis was then confirmed with tissue sampling. The most consistent imaging findings were clival bone marrow T1 hypointensity and preclival soft tissue infiltration. Five of six patients were cured with no recurrence of skull base infection over a 2-4-year follow-up period. CONCLUSION: In the setting of headache, cranial neuropathy, elevated erythrocyte sedimentation rate, and abnormal clival imaging findings, central skull base osteomyelitis should be considered as the likely diagnosis. Early tissue sampling and appropriate treatment may prevent or limit further complications such as intracranial extension, empyema, or death.
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ranking = 1.2
keywords = otitis
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4/17. Hyper-IgM syndrome with CHARGE association.

    A girl with coloboma of the iris, sensorineural deafness, growth delay, distinctive face, and cranial nerve dysfunction was diagnosed of CHARGE association in the first year of life. She presented with repeated otitis. At 3 yr of age, the patient suffered a septicemia (streptococcus pneumoniae, corynebacterium sp.). The immunoglobulin g (IgG) and IgA serum levels were decreased, IgM increased and cellular immunity parameters were normal, supporting the diagnosis of hyper-IgM (HIM) syndrome. The sequence of cd40 ligand and cytidine deaminase genes were normal. From then on, she was receiving immunoglobulin intravenously with an excellent outcome. Here, we report the first case of CHARGE association and HIM syndrome in the same patient. Although the cause could not be identified, a non-random link is likely.
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ranking = 0.2
keywords = otitis
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5/17. aspergillosis of the petrous apex.

    We present the case of an immunocompetent man who presented with multiple and progressive cranial nerve palsies. The cause was found to be aspergillosis of the petrous bone, arising as a result of chronic fungal otitis media. Despite treatment with local excision and systemic anti-fungal therapy, he died after suffering a subarachnoid haemorrhage. diagnosis rests upon prompt radiological imaging and the acquisition of tissue for histological examination and culture but cannot be made without awareness of the condition.
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ranking = 0.2
keywords = otitis
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6/17. Gradenigo syndrome: a case report and review of a rare complication of otitis media.

    otitis media is a commonly seen condition in the Emergency Department. The complications of otitis media that were seen frequently in the preantibiotic era are now rare today. We report a case of a diabetic man who presented with otorrhea, retro-orbital pain, and diplopia secondary to a sixth cranial nerve palsy--Gradenigo syndrome. This syndrome occurs as infection from the middle ear spreads medially to the petrous portion of the temporal bone. The emergency physician should consider this condition in patients with chronic ear drainage or pain not responsive to conventional treatment or in any patient with a cranial nerve palsy in the setting of acute or chronic otitis. work-up should include a CT scan of the temporal bones. otolaryngology consultation and admission for i.v. antibiotics is recommended.
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ranking = 1.2
keywords = otitis
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7/17. Jugular foramen syndrome caused by choleastatoma.

    Unilateral paresis of 9th-11th cranial nerves together is defined as jugular foramen (Vernet's) syndrome. A cholesteatoma case that penetrated into intracranial area after eroding temporal bone and led to jugular foramen syndrome is presented here, since such a case has not been reported in the literature hitherto. A 46-year-old male patient was evaluated for hoarseness. It was learnt from his anamnesis that he had been operated for otitis media nearly 20 years ago. uvula deviated towards right. Loss of gag reflex was remarkable on the left. paresis was found on the left vocal cord. There was weakness in rotation of the head to the right. Computerized tomography (CT) scan revealed that the temporal bone lost its integrity and that there was an extra-axial hypodense mass neighboring pontocerebellum. Post-contrast magnetic resonance imaging showed that the mass, which showed thin, regular circular contrasting and which was hypointense in T1-weighted MR and hyperintense in T2-weighted MR, extended to the left jugular foramen. The mass was totally removed with left sub-occipital approach in the treatment. After the treatment, hoarseness, weakness in the rotation movement of the head and loss of gag reflex recovered totally, while deviation in the uvula was permanent. Cholesteatomas can extend to posterior fossa and cause jugular foramen syndrome. Early surgery is important to completely reverse the lost nerve functions in treatment.
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ranking = 0.2
keywords = otitis
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8/17. Unusual presentations of craniovertebral junction tuberculosis: a report of 2 cases and literature review.

    BACKGROUND: CVJ tuberculosis is a described entity requiring challenging ways of management. Severe neck pain, causing restricted neck movements and torticollis, is a characteristic presentation of neurologically asymptomatic suboccipital Pott's disease. CASE DESCRIPTION: Two patients with unusual CVJ tuberculosis form the basis for the present communication. The first patient presented with tubercular otitis media, causing progressive erosion of the petrous part of temporal bone, and destruction of the occipital condyle, along with the lateral mass of atlas, leading to CVJ instability. This is a first report of such a presentation, according to our knowledge. Detailed bony architectural destruction demonstrable on CT scan has been described. The second patient, with CVJ tuberculosis, presented with skull base syndrome and with multiple cranial nerve palsies. Both patients were managed without surgical intervention and showed clinical and radiological recovery. CONCLUSION: In such patients with unusual clinical presentations, histopathologic examination is necessary to arrive at a correct diagnosis. The management of patients with tubercular involvement of CVJ remains controversial. In the present communication, both the patients were managed successfully with full dose of antitubercular drugs and immobilization.
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ranking = 0.2
keywords = otitis
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9/17. Gradenigo's syndrome: a rare complication of otitis media.

    A 58-year-old woman developed Gradenigo's syndrome. The clinical signs and symptoms of the otitis were rather atypical and there was a considerable delay before the correct diagnosis was made. The pathophysiology, therapy and differential diagnosis of this syndrome, which has become rare since the introduction of antibiotics, are discussed.
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ranking = 1
keywords = otitis
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10/17. Malignant external otitis: long-term (months) antimicrobial therapy.

    Since Chandler's initial report in 1968 on "malignant external otitis" (MEO), this entity has been recognized in its earlier stages with increasing frequency. As a result of this, the availability of new antimicrobial agents, and the judicious use of surgery, there has been an improvement in the results of therapy. There remains, however, a subgroup of patients who continue to experience a significant mortality from this infection. A review and selected discussion of our experience with MEO from 1976 to 1979 is presented. Six patients have been diagnosed and successfully treated for MEO. They presented with problems ranging from severe otalgia to multiple cranial neuropathy. For the most part, therapy consisted of the now standard aminoglycoside and carbenicillin combination. Two of the patients were in the high mortality risk group. One of these patients developed an osteomyelitis which extended across the skull base resulting in bilateral cranial neuropathies. His therapy included surgery as well as long-term (months) outpatient treatment with tobramycin and carbenicillin with an excellent result. The second patient was treated similarly. The efficacy of this approach is discussed as well as the usefulness of radionuclide bone scanning in assessing the course and therapy of patients with MEO.
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ranking = 1
keywords = otitis
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