Deficient Hearing
Mostrando 1-12 de 15 artigos, teses e dissertações.
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1. Cochleovestibular Manifestations in Fabry Disease
Abstract Fabry disease is a rare, X-linked lysosomal storage disorder resulting from deficient α-galactosidase A activity and globotriaosylceramide accumulation throughout the body. This accumulation leads to various clinical disorders, including inner ear lesions, with sensorineural hearing loss and dizziness. Although hearing loss is recognized in these p
J. inborn errors metab. screen.. Publicado em: 30/05/2019
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2. Otoneurological Abnormalities in Patients with Friedreich's Ataxia
Abstract Introduction Friedreich's ataxia is a neurodegenerative disease and progressive by nature. It has autosomal recessive inheritance and early onset inmost cases. Nystagmus and hearing loss (in some cases) make up some of the common symptoms seen in this disorder. Objective The objective of this study is to examine vestibular disorders in patientsw
Int. Arch. Otorhinolaryngol.. Publicado em: 2017-03
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3. Mobilizando-se para resgatar a vida através do implante coclear: a experiência da família da criança com deficiência auditiva / Mobilizing to rescue the life through the cochlear implant: the experience of the cochlear implant user auditive deficient childs family
Este trabalho objetivou compreender a experiência da família da criança com deficiência auditiva usuária de implante coclear, mais especificamente entender seu papel, identificar as mudanças e dificuldades na vida diária, descrever as estratégias de enfrentamento e os elementos facilitadores, conhecer os sentimentos que permeiam conviver com a defici
Publicado em: 2011
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4. Formation of the speech-language and hearing pathologist: challenges and perspectives for inclusive education. / A formação do fonoaudiólogo: desafios e perspectivas para a educação inclusiva
Following the line of research of University, Teaching and Formation of Teachers, the objective of this study is to promote reflection about the formation of the speech-language and hearing pathologist for working together with Inclusive Education of deficient children. Data was collected by means of documental analysis and semi-structured interviews with th
Publicado em: 2007
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5. A COMUNICAÇÃO VIA INTERNET NA INCLUSÃO E INTEGRAÇÃO DE DEFICIENTES AUDITIVOS: um estudo sobre a região do ABC / SP
O objetivo deste trabalho é compreender como a comunicação, através da Internet, é utilizada e construída, consideradas as questões da inclusão e integração de pessoas com deficiência auditiva. Analisam-se os conceitos sobre comunicação, inclusão e integração; a deficiência auditiva e seu significado para os indivíduos; a questão dos sites
Publicado em: 2007
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6. Hearing loss and retarded cochlear development in mice lacking type 2 iodothyronine deiodinase
The later stages of cochlear differentiation and the developmental onset of hearing require thyroid hormone. Although thyroid hormone receptors (TRs) are a prerequisite for this process, it is likely that other factors modify TR activity during cochlear development. The mouse cochlea expresses type 2 deiodinase (D2), an enzyme that converts thyroxine, the ma
National Academy of Sciences.
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7. A role for pneumolysin but not neuraminidase in the hearing loss and cochlear damage induced by experimental pneumococcal meningitis in guinea pigs.
We investigated the roles of pneumolysin and neuraminidase in the pathogenesis of deafness and cochlear damage during experimental pneumococcal meningitis. Anesthetized guinea pigs were inoculated intracranially with 7.5 log10 CFU of either (i) wild-type Streptococcus pneumoniae D39 (n = 8), (ii) PLN-A, a defined isogenic derivative of D39 deficient in pneum
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8. Thyroid hormone receptor β-dependent expression of a potassium conductance in inner hair cells at the onset of hearing
To elucidate the role of thyroid hormone receptors (TRs) α1 and β in the development of hearing, cochlear functions have been investigated in mice lacking TRα1 or TRβ. TRs are ligand-dependent transcription factors expressed in the developing organ of Corti, and loss of TRβ is known to impair hearing in mice and in humans. Here, TRα1-deficient (TRα1�
The National Academy of Sciences.
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9. Urocortin-Deficient Mice Display Normal Stress-Induced Anxiety Behavior and Autonomic Control but an Impaired Acoustic Startle Response
Corticotropin-releasing hormone (Crh) plays an important role in modulating physiological and behavioral responses to stress. Its actions are mediated through two receptors, Crhr1 and Crhr2. Urocortin (Ucn), a Crh-related neuropeptide and the postulated endogenous ligand for Crhr2, is a potential mediator of stress responses. We generated Ucn-deficient mice
American Society for Microbiology.
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10. Six4, a Putative myogenin Gene Regulator, Is Not Essential for Mouse Embryonal Development
Six4 is a member of the Six family genes, homologues of Drosophila melanogaster sine oculis. The gene is thought to be involved in neurogenesis, myogenesis, and development of other organs, based on its specific expression in certain neuronal cells of the developing embryo and in adult skeletal muscles. To elucidate the biological roles of Six4, we generated
American Society for Microbiology.
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11. Deafness and Cochlear Fibrocyte Alterations in Mice Deficient for the Inner Ear Protein Otospiralin
In the cochlea, the mammalian auditory organ, fibrocytes of the mesenchymal nonsensory regions play important roles in cochlear physiology, including the maintenance of ionic and hydric components in the endolymph. Occurrence of human deafness in fibrocyte alterations underlines their critical roles in auditory function. We recently described a novel gene, O
American Society for Microbiology.
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12. Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding α-N-acetylglucosaminidase
The Sanfilippo syndrome type B is an autosomal recessive disorder caused by mutation in the gene (NAGLU) encoding α-N-acetylglucosaminidase, a lysosomal enzyme required for the stepwise degradation of heparan sulfate. The most serious manifestations are profound mental retardation, intractable behavior problems, and death in the second decade. To generate a
The National Academy of Sciences.