1/378. 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.- - - - - - - - - - ranking = 1keywords = rate, death (Clic here for more details about this article) |
2/378. Intraoperative loss of auditory function relieved by microvascular decompression of the cochlear nerve.BACKGROUND: Brainstem auditory evoked potentials (BAEP) are useful indicators of auditory function during posterior fossa surgery. Several potential mechanisms of injury may affect the cochlear nerve, and complete loss of BAEP is often associated with postoperative hearing loss. We report two cases of intraoperative auditory loss related to vascular compression upon the cochlear nerve. methods: Intra-operative BAEP were monitored in a consecutive series of over 300 microvascular decompressions (MVD) performed in a recent twelve-month period. In two patients undergoing treatment for trigeminal neuralgia, BAEP waveforms suddenly disappeared completely during closure of the dura. RESULTS: The cerebello-pontine angle was immediately re-explored and there was no evidence of hemorrhage or cerebellar swelling. The cochlear nerve and brainstem were inspected, and prominent vascular compression was identified in both patients. A cochlear nerve MVD resulted in immediate restoration of BAEP, and both patients recovered without hearing loss. CONCLUSION: These cases illustrate that vascular compression upon the cochlear nerve may disrupt function, and is reversible with MVD. awareness of this event and recognition of BAEP changes alert the neurosurgeon to a potential reversible cause of hearing loss during posterior fossa surgery.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
3/378. 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.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
4/378. 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.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
5/378. Discourse deficits following right hemisphere damage in deaf signers.Previous findings have demonstrated that hemispheric organization in deaf users of American sign language (ASL) parallels that of the hearing population, with the left hemisphere showing dominance for grammatical linguistic functions and the right hemisphere showing specialization for non-linguistic spatial functions. The present study addresses two further questions: first, do extra-grammatical discourse functions in deaf signers show the same right-hemisphere dominance observed for discourse functions in hearing subjects; and second, do discourse functions in ASL that employ spatial relations depend upon more general intact spatial cognitive abilities? We report findings from two right-hemisphere damaged deaf signers, both of whom show disruption of discourse functions in absence of any disruption of grammatical functions. The exact nature of the disruption differs for the two subjects, however. Subject AR shows difficulty in maintaining topical coherence, while SJ shows difficulty in employing spatial discourse devices. Further, the two subjects are equally impaired on non-linguistic spatial tasks, indicating that spared spatial discourse functions can occur even when more general spatial cognition is disrupted. We conclude that, as in the hearing population, discourse functions involve the right hemisphere; that distinct discourse functions can be dissociated from one another in ASL; and that brain organization for linguistic spatial devices is driven by its functional role in language processing, rather than by its surface, spatial characteristics.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
6/378. 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.- - - - - - - - - - ranking = 0.37608669232653keywords = death (Clic here for more details about this article) |
7/378. 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.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
8/378. Implantation of patients with prelingual long-term deafness.The purpose of this paper is to provide performance data on long-term congenitally and prelingually deafened children and adults who are implanted with the CLARION Multi-Strategy Cochlear Implant and who use the Continuous Interleaved Sampler (CIS) speech processing strategy. Open-set speech perception was assessed preoperatively and postoperatively with word and sentence recognition tests. Results indicate that a majority of subjects showed improvement from preoperative performance to the last available postoperative evaluation. These results represent a change from previously reported data on a similar population who used earlier speech processing strategies.- - - - - - - - - - ranking = 1.8717399230204keywords = rate (Clic here for more details about this article) |
9/378. Fractionation of visual memory: evidence from a case with multiple neurodevelopmental impairments.It is known that the adult visual memory system is fractionable into functionally independent cognitive subsystems, selectively susceptible to brain damage. However, it is unclear whether these cognitive subsystems can fractionate developmentally. The present study describes an investigation of visual memory of a patient (PE) with multiple developmental disorders. PE was congenitally deaf, had Gilles de la tourette syndrome and autism, with non-verbal ability in the normal range. The patient presented with a recognition memory impairment for unknown human faces. This contrasted with his superior recognition memory for unknown buildings, landscapes and outdoor scenes. PE's memory impairment for faces could not be explained by a general deficit in face processing. Interestingly, PE also showed a recognition memory impairment for animals. These findings indicate that different domains of the visual memory system can be fractionated developmentally. In particular, it demonstrates that topographical memory can develop independently from other aspects of visual memory.- - - - - - - - - - ranking = 0.62391330767347keywords = rate (Clic here for more details about this article) |
10/378. Inherited susceptibility to aminoglycoside ototoxicity: genetic heterogeneity and clinical implications.PURPOSE: Aminoglycoside-induced ototoxicity appears to have a genetic susceptibility in some individuals, and the A1555G mutation in the mitochondrial 12S ribosomal rna gene has been shown to be responsible for this susceptibility in all familial cases. An Italian family with 5 family members who became deaf after aminoglycoside exposure presented to us, and molecular analysis excluded the A1555G mutation. The purpose of this study is to identify the molecular basis for the aminoglycoside susceptibility in this family. patients AND methods: Two sisters and three of their children developed severe to profound high-frequency hearing loss after aminoglycoside exposure. dna was extracted from the blood of these individuals and their unaffected relatives, and analyzed for mitochondrial dna mutations. The region around nucleotide 961 was also cloned and individual clones were sequenced. RESULTS: Sequencing of the 12S ribosomal rna gene revealed a thymidine deletion at position 961, with a complex pattern of sequence around this mutation. Sequencing of individual clones around the 961 mutation demonstrated a varying number of inserted cytosines in different mitochondrial molecules. CONCLUSION: This family establishes the nucleotide 961 thymidine deletion associated with a varying number of inserted cytosines in the mitochondrial 12S ribosomal rna gene as the second pathogenic mutation that can predispose to aminoglycoside ototoxicity. It demonstrates the clinical relevance of taking a family history before administering aminoglycosides to any patient. In addition, it would be desirable for sporadic patients with aminoglycoside-induced hearing loss to be screened with molecular tests for the presence of the 1555 and 961 mutations. Such screening could significantly decrease the prevalence of aminoglycoside-induced hearing loss.- - - - - - - - - - ranking = 1.2478266153469keywords = rate (Clic here for more details about this article) |
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