Cases reported "Deafness"

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1/730. Sudden deafness. A histopathological study.

    Histopathological studies were performed in the temporal bones from a 77-year-old woman who had bilateral sudden deafness 31 years before her death. The common findings in both cochleas were atrophy of the organ of corti and stria vascularis, and degeneration of the tectorial membrane. pathology of the tectorial membrane consisted of atrophy and droplet formation of the middle and marginal zones. In one area, an atrophied tectorial membrane was lifted up by a mass on the limbus. The saccular wall in the left temporal bone was ruptured. There was no pathology in the vascular system in the labyrinths except for moderate degeneration of sensory epithelium. The pathogenesis of sudden deafness was discussed in the light of the present findings. Alteration of the tectorial membrane seemed to be the main factor as a possible cause of sudden deafness. Histopathological findings were reviewed from the literature.
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2/730. Intravenous azithromycin-induced ototoxicity.

    Intravenous azithromycin is increasingly administered for treatment of hospitalized patients with community-acquired pneumonia. Macrolide antibiotics cause ototoxicity, which occurs most frequently when high serum concentrations are achieved. Current dosing guidelines for intravenous azithromycin can result in much higher serum concentrations than is seen with oral administration. We describe a 47-year-old woman who developed complete deafness after receiving 8 days of intravenous azithromycin.
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ranking = 0.14285714285714
keywords = deafness
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3/730. Contralateral deafness following unilateral suboccipital brain tumor surgery in a patient with large vestibular aqueduct--case report.

    A 68-year-old female developed contralateral deafness following extirpation of a left cerebellopontine angle epidermoid cyst. Computed tomography showed that large vestibular aqueduct was present. This unusual complication may have been caused by an abrupt pressure change after cerebrospinal fluid release, which was transmitted through the large vestibular aqueduct and resulted in cochlear damage.
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ranking = 0.71428571428571
keywords = deafness
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4/730. Progressive ponto-bulbar palsy with deafness. A clinico-pathological study.

    Progressive ponto-bulbar palsy with deafness is a rare disease. It seems to be an abiotrophic process with autosomal recessive inheritance in most instances. Only one autopsy case had been briefly described (Lelong et al., 1941). The clinical features and the pathological findings of a new case are reported. The structures primarily involved are the grey matter of the brain stem and the spinal cord, including to some extent the optic tracts and most of the fiber tracts in the brain stem with exception of the pyramidal tracts.
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ranking = 0.71428571428571
keywords = deafness
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5/730. Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by both renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula and intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has been included in the definition of Alport's syndrome. However, other types of nephropathy have been occasionally associated with hereditary deafness. The association of ADPKD with hereditary SNHL has not been previously documented. We report a family with ADPKD associated with bilateral sensorineural deafness in a pedigree of four affected members in four generations.
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ranking = 0.85714285714286
keywords = deafness
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6/730. Narrow and vacant internal auditory canal.

    A case of unilateral congenital deafness revealing a narrow vacant internal auditory canal and a more anterior and superior second canal where the facial and vestibulocochlear nerves are well visualised is presented. Having reviewed the scientific and embryological data, the authors consider the mechanism of this malformation.
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ranking = 0.14285714285714
keywords = deafness
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7/730. The assessment and treatment of deaf children with psychiatric disorders.

    The assessment and treatment of deaf children with psychiatric disorder is intimately related to the individual child's communication, which in turn is affected by a number of factors, medical, social, and cultural. The deafness can be aetiologically related to the psychiatric disorder or can be incidental. Treatment strategies should be adapted to meet the individual child and family's needs. Deaf professionals have a vital role in mental health services for this population. The use of an interpreter can clarify communication and cultural issues for deaf and hearing children, families, and professionals.
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ranking = 0.14285714285714
keywords = deafness
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8/730. Labyrinthine involvement in Langerhans' cell histiocytosis.

    BACKGROUND: Langerhans' cell histiocytosis, a rare condition caused by the proliferation of abnormal Langerhans' cells ('LCH cells') and an accompanying granulomatous infiltrate, can affect several organs including the ear. External and middle ear involvement are common with a reported incidence as high as 61%. The bony labyrinth is resistant to erosion by the granulation tissue, thereby protecting the cochlea and vestibular structures. Probably for this reason, involvement of the inner ear is rare, with few case reports in the literature. patients: We report two girls, one with bilateral and the other with unilateral mastoid involvement, in whom there was invasion of the labyrinth. The first girl had 'single system' LCH affecting only bone and developed an acute hearing loss due to invasion of the cochlea, while the second had both bone and skin involvement and labyrinthine involvement was diagnosed on imaging prior to the onset of labyrinthine symptoms. CONCLUSION: Inner ear involvement can lead to permanent deafness, which may be prevented by early institution of treatment. Threatened inner ear involvement requires urgent systemic medical therapy with steroids, possibly combined with chemotherapy.
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ranking = 0.14285714285714
keywords = deafness
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9/730. Hereditary nephritis, deafness and abnormal thrombopoiesis. Study of a new kindred.

    A fourth kindred displaying the triad of hereditary nephritis, deafness and thrombocytopenia with giant platelets is described. Renal involvement, a common cause of death amongst afflicted subjects, appears to have a better prognosis in the affected members of this family. Although the electron microscopic appearance of the megakaryocytes in the present case appears similar to that in previously reported cases, we suggest that the "giant" platelets may result from a degenerative process of megakaryocytes leading to nuclear regression and cytoplasmic fragmentation, rather than the usual blebbing process.
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ranking = 0.71428571428571
keywords = deafness
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10/730. Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study.

    We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. magnetic resonance imaging showed a diffuse brain atrophy. A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients.
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ranking = 0.57142857142857
keywords = deafness
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