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Auswahl der wissenschaftlichen Literatur zum Thema „Progressive hearing loss“
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Zeitschriftenartikel zum Thema "Progressive hearing loss"
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Hayes, Deborah, und Susan Dreith. „Catastrophic Progressive Hearing Loss in Childhood“. Journal of the American Academy of Audiology 11, Nr. 06 (Juni 2000): 300–308. http://dx.doi.org/10.1055/s-0042-1748058.
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AbstractWe present a report on a 5-year-old child with a complex medical and audiologic history who exhibited catastrophic progression in hearing loss. Hearing loss was initially attributed to bacterial meningitis at age 3 months; progression was apparently related to perilymph fistula at age 8 years. Etiologies associated with progressive hearing loss in children as well as signs of progression and monitoring protocols for children at risk for progressive hearing loss are discussed. Abbreviations: ABR = auditory brainstem response, CMV = cytomegalovirus, JCIH = Joint Committee on Infant Hearing, LVA = large vestibular aqueduct, PPHN = persistent pulmonary hypertension of the newborn, TCH = The Children's Hospital
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Fitzpatrick, Elizabeth M., Flora Nassrallah, Bénédicte Vos, JoAnne Whittingham und Jessica Fitzpatrick. „Progressive Hearing Loss in Children With Mild Bilateral Hearing Loss“. Language, Speech, and Hearing Services in Schools 51, Nr. 1 (08.01.2020): 5–16. http://dx.doi.org/10.1044/2019_lshss-ochl-19-0013.
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Purpose This study investigated progressive hearing loss in a cohort of children who were identified with permanent mild bilateral hearing loss. Method This population-based study included 207 children with permanent mild bilateral hearing loss, diagnosed and followed from 2003 to 2016 in 1 region of Canada. Clinical characteristics and initial audiologic results were collected prospectively at diagnosis, and audiologic information was updated. Changes in hearing levels between the 1st and most recent assessment were analyzed to determine progressive hearing loss. Clinical characteristics were compared between children with progressive and stable hearing loss. The association between risk indicators and progressive hearing loss was explored through logistic regression. Results A total of 47.4% (94 of 207) had progressive hearing loss in at least 1 ear, and 50% (47 of 94) of those experienced more than 20-dB average drop in thresholds. For these 94 children, a total of 147 ears were affected, and 116 (78.9%) ears experienced sufficient change in thresholds to be coded as a worse category of hearing loss. In the subset of 85 children with more than 5 years of audiologic follow-up, 56.4% (53/85) showed a decrease in hearing. Of the total sample of 207 children, 137 (66.2%) continued to have mild hearing loss in the better ear. There was no association between the risk factors examined (family history of hearing loss, admission to the neonatal intensive care unit, or presence of a syndrome) and progressive hearing loss. Conclusion This study found that almost half of children with mild bilateral hearing loss showed a decrease in hearing in at least 1 ear. One third of the children first diagnosed with mild hearing loss in the better ear now have moderate or worse hearing loss in both ears. These findings point to the importance of careful long-term monitoring of children who present with mild hearing loss.
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Roberts, Deanne M., Matthew L. Bush und Raleigh O. Jones. „Adult Progressive Sensorineural Hearing Loss“. Otology & Neurotology 35, Nr. 2 (Februar 2014): 241–45. http://dx.doi.org/10.1097/mao.0b013e3182a437b3.
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Whelan, Alison, und Anne Hing. „Genetics of Progressive Hearing Loss“. Seminars in Hearing 16, Nr. 03 (August 1995): 246–56. http://dx.doi.org/10.1055/s-0028-1083722.
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Vrabec, Jeffrey T. „Hydrocodone Use and Sensorineural Hearing Loss“. Pain Physician 3;10, Nr. 5;3 (14.05.2007): 467–72. http://dx.doi.org/10.36076/ppj.2007/10/467.
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Background: The hydrocodone/acetaminophen combination is one of the most commonly used analgesic preparations. Isolated incidences of suspected association between hydrocodone abuse and rapidly progressive hearing loss have been reported. In this study, we describe the clinical characteristics of 5 patients presenting with progressive hearing loss and a history of hydrocodone use. Methods: Patients presenting with rapidly progressive bilateral hearing loss who had a documented history of hydrocodone use were selected for the study. The presentation, audiologic findings, associated comorbidities, and treatment outcomes were reviewed Results: All patients displayed rapidly progressive sensorineural hearing loss without vestibular symptoms. Hearing loss was asymmetric in 3 patients at initial presentation, but progressed to profound loss, usually within months. Steroid treatment has no effect on the progression of the hearing loss. The admitted quantity of hydrocodone consumed ranged from 10 to 300 mg per day. Hepatitis C was the most common comorbidity, present in 60% of the patients. All patients underwent cochlear implantation with satisfactory results. Conclusions: The chronic use of hydrocodone can be associated with progressive sensorineural hearing loss. Successful auditory rehabilitation can be achieved with cochlear implantation. Genetic polymorphisms of drug metabolizing enzymes as well as associated comorbidities such as hepatitis C infection may be significant in the development of hydrocodone ototoxicity, though additional investigations are necessary. Key words: hydrocodone, sensorineural hearing loss, cochlear implant
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Levi, Haya, Lilly Tell und Moshe Feinmesser. „Progressive Hearing Loss in Hard-of-Hearing Children“. International Journal of Audiology 32, Nr. 2 (Januar 1993): 132–36. http://dx.doi.org/10.3109/00206099309071862.
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Hashimoto, Sho, und Harold F. Schuknecht. „Progressive Hearing Loss from Strial Dysplasia“. Annals of Otology, Rhinology & Laryngology 96, Nr. 2 (März 1987): 229–31. http://dx.doi.org/10.1177/000348948709600219.
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An otherwise healthy male patient had a bilateral, slowly progressive hearing loss first noticed in early childhood and possibly present at birth. Audiometric studies at the age of 32 showed a moderately severe, bilateral, mixed type hearing loss. He died of unrelated causes at the age of 34. Studies of the temporal bones showed bilateral hypoplasia and atrophy of the striae vascularis as the cause of hearing loss. The history and findings are consistent with a genetically determined hypoplasia and atrophy of this structure.
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Karjalainen, Seppo, Leena Pakarinen, Helena Kääriäinen, Markku Teräsvirta und Eero Vartiainen. „Progressive Hearing Loss in Usher's Syndrome“. Annals of Otology, Rhinology & Laryngology 98, Nr. 11 (November 1989): 863–66. http://dx.doi.org/10.1177/000348948909801106.
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In 18 patients with Usher's syndrome, progressive hearing loss was verified audiologically in eight cases. Despite poor auditory threshold values and low speech discrimination scores, there was only one patient who could not communicate with speech. The possibility of hearing impairment being mainly progressive in Usher's syndrome is discussed.
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Lee, Jennifer W., und Manohar L. Bance. „Hearing loss“. Practical Neurology 19, Nr. 1 (05.09.2018): 28–35. http://dx.doi.org/10.1136/practneurol-2018-001926.
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Hearing loss affects one in six people in the UK and is a significant disease burden. In addition to communication problems, there is also an association with depression and dementia. Clinical assessment with targeted history and examination can identify the characteristics and cause of hearing loss, and complementary audiological testing can confirm its type and severity. Retrocochlear screening is recommended for sudden, rapidly progressive or asymmetric sensorineural hearing loss. Medical or surgical therapies may be indicated in cases of conductive hearing loss, while hearing assistive devices and hearing aids are the mainstay of rehabilitation for sensorineural hearing loss.
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de Wolf, M. J. F., J. Honings, F. B. M. Joosten, L. Hoefsloot, E. A. M. Mylanus und C. W. R. J. Cremers. „Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation“. Journal of Laryngology & Otology 124, Nr. 1 (23.06.2009): 86–89. http://dx.doi.org/10.1017/s0022215109990296.
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AbstractObjective:Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.Design:Case report.Subjects:Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.Results:These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.Conclusion:Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.
Dissertationen zum Thema "Progressive hearing loss"
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Hilton, Jennifer Maglona. „Progressive hearing loss in mouse mutants“. Thesis, University of Cambridge, 2012. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.610680.
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Light, Katrina Jane. „Reactions and Responses to the Diagnosis of a Progressive Hearing Loss in Adults“. Thesis, University of Canterbury. Communication Disorders, 2009. http://hdl.handle.net/10092/3614.
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Being given the diagnosis of a disability generally affects an individual's emotional state, however, this has not previously been investigated with respect to audiology and the diagnosis of hearing loss. The first aim of this study was to describe some of the common initial reactions to the diagnosis of hearing loss (HL). An awareness of these emotional reactions will aid audiologists in counselling their patients. Counselling occurs at the time of the diagnosis and throughout the aural rehabilitation process. However, counselling tuition is currently not provided for audiology students at New Zealand universities and there are few professional development courses for practicing audiologists. The second aim of this study was to evaluate current audiological counselling services and ascertain the impact on patients' decisions to get hearing aids (HAs). To accomplish these aims, 27 adults who had been newly-diagnosed with a HL completed an initial reaction questionnaire, partook in an interview which followed up on the questionnaire, and subsequently completed a second questionnaire at least three weeks later. There were two versions of the second questionnaire, depending on whether they had chosen to have HA(s) fitted. The results found that the common emotions reported were a sense of loss, sadness and resignation, as well as relief. Furthermore, an individual's level of optimism tended to decrease in response to the hearing test result. The ratings of the audiological counselling services were positive and seemed not to significantly influence the individual with respect to their decision to purchase HAs. The two areas of audiological counselling which could be improved related to how the audiologist explained the HL, particularly in relation to the individual's life, and also the provision of information to patients prior to the fitting of the HA. In addition to the data that was collected in relation to these aims, information was collected with respect to patients' perceptions of their HL prior to the hearing test, their interpretation of the hearing test results, and also how the patient's significant other responded to the diagnosis. The information from this study will be useful for equipping audiologists, both new graduates and those with more experience, to provide optimal audiological care for their patients.
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Wentling, Maureen. „Characterization of the disease mechanisms underlying clarin-mediated progressive hearing loss“. Electronic Thesis or Diss., Sorbonne université, 2023. https://accesdistant.sorbonne-universite.fr/login?url=https://theses-intra.sorbonne-universite.fr/2023SORUS386.pdf.
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Malgré la forte prévalence de la déficience auditive, les mécanismes sous-jacents de la perte auditive progressive restent inconnus. Notre laboratoire a étudié le(s) rôle(s) de la clarine-1, responsable du syndrome d'Usher de type III, qui entraîne une perte auditive progressive, et de la clarine-2, responsable de la surdité non syndromique, dans le système auditif. En raison de la variabilité phénotypique chez les patients atteints du syndrome d'Usher de type III, même parmi les patients présentant les mêmes mutations génétiques, nous avons émis l'hypothèse qu'il pourrait y avoir une redondance fonctionnelle entre les deux clarines. Nous avons donc généré des souris knock-out totales et conditionnelles (Bhlhb5-cre et Myo15-cre) pour la clarine-1/clarine-2. En utilisant une approche multidisciplinaire, intégrant des études omiques et électrophysiologiques avec une imagerie à haute résolution dans le temps, j'ai identifié les voies moléculaires clés dérégulées en l'absence de clarine-1 et clarine-2 dans les cellules ciliées auditives et les neurones auditifs primaires. L'analyse phénotypique des souris Clrn1-/-Clrn2-/- a révélé une surdité profonde dès l'apparition de l'audition. Les enregistrements de courant de transduction mécano-électrique (MET) étaient absents chez les souris Clrn1-/-Clrn2-/-, mais seulement réduits chez les souris Clrn1-/- et Clrn2-/-. Ces résultats démontrent un rôle fonctionnel compensatoire de la clarine-1 et de la clarine-2 au niveau de la touffe ciliaire. J'ai également observé des anomalies de l'homéostasie ionique, nécessaires au fonctionnement normal de la MET et à la transmission synaptique, qui étaient plus graves chez les souris Clrn1-/-Clrn2-/- que chez les souris Clrn1-/- et Clrn2-/-. Ces changements ioniques s'accompagnent de troubles pré- et postérieurs à l'apparition de la maladie. Pour valider l'hypothèse selon laquelle le(s) rôle(s) principal(aux) de la clarine-1 et de la clarine-2 se situe(nt) dans les cellules ciliées, j'ai étudié des souris chez lesquelles la clarine-1 et la clarine-2 ont été inactivées de manière spécifique dans les cellules ciliées (Myo15-cre). Ces souris knock-out conditionnelles reproduisaient les changements ioniques et synaptiques observés chez les souris knock-out totales pour les clarines, entraînant une dégénérescence des neurones auditifs primaires. Pour renforcer cette hypothèse, j'ai étudié le phénotype auditif chez des souris ayant subi une délétion spécifique des neurones auditifs primaires (Bhlhb5-cre) de la clarine-1 et de la clarine-2. Ces souris avaient une audition normale jusqu'à l'âge de 6 mois, sans modification de l'épithélium sensoriel cochléaire ni de la dégénérescence des neurones auditifs primaires. Afin d'approfondir les fonctions moléculaires communes et uniques de clarine-1 et clarine-2, j'ai effectué une analyse séquentielle de l'ARN sur l'organe de Corti entier de souris Clrn1-/-, Clrn2-/- et Clrn1-/-Clrn2-/-. Conformément aux observations physiologiques, j'ai constaté une dysrégulation dans 8 catégories distinctes et physiologiquement pertinentes : le flux cationique, l’organisation et la fonction synaptique, l’endocytose et l’exocytose, la fonction neuronale et différenciation, la fonction métabolique, l’organisation de l'actine et du cytosquelette, l’homéostasie lipidique et l’inflammation. Nous concluons que la clarine-1 et la clarine-2 jouent des rôles communs et compensatoires dans l'activité de transduction mécano-électrique et dans l'intégrité pré- et post-synaptique. Les clarines sont également nécessaires à l'intégrité de la touffe ciliaire auditive, à l'homéostasie ionique dans les cellules ciliées auditives et à la survie des neurones auditifs primaires. Ces résultats permettront d'élucider de nouveaux mécanismes impliqués dans la perte progressive de l'auditionDespite high prevalence of debilitating hearing loss, underlying mechanisms of progressive hearing loss remain elusive. Our lab has been investigating the role(s) of clarin-1, responsible for Usher syndrome type III, causing progressive hearing loss, and clarin-2, responsible for non-syndromic hearing impairment, in the auditory system. Due to phenotypic variability in Usher Syndrome type III patients, even among patients with the same genetic mutations, we hypothesized that there may be a functional redundancy between the two clarins. Therefore, we generated clarin-1/clarin-2 total and conditional (Bhlhb5-cre and Myo15-cre) knockout mice. Using a multidisciplinary approach, integrating omics and electrophysiological studies with high resolution imaging over time, I pinpointed key molecular pathways dysregulated in the absence of clarin-1 and clarin-2 in auditory hair cells and primary auditory neurons. Phenotypic analysis of Clrn1-/-Clrn2-/- mice revealed profound deafness from hearing onset. Mechanoelectrical transduction (MET) current recordings were absent in Clrn1-/-Clrn2-/- mice, but only reduced in Clrn1-/- and Clrn2-/- mice. These results demonstrate a compensatory functional role of clarin-1 and clarin-2 at the hair bundle. I also observed abnormalities in ionic homeostasis, required for normal MET function and synaptic transmission, that were more severe in Clrn1-/-Clrn2-/- mice, relative to Clrn1-/- and Clrn2-/- mice. These ionic changes were accompanied by pre- and post-synaptic abnormalities, resulting in abnormal cytoplasmic vesicle accumulation and synaptic function in hair cells. Furthermore, I observed a progressive degeneration of the cochlear sensory epithelium and primary auditory neurons over time. To validate the hypothesis that the primary role(s) of clarin-1 and clarin-2 are in hair cells, I studied mice with hair cell-specific (Myo15-cre) deletion of clarin-1 and clarin-2. These conditional clarin knockout mice mimicked the ionic and synaptic changes found in total clarin knockout mice, resulting in primary auditory neuron degeneration. To reinforce this hypothesis, I studied the auditory phenotype in mice with primary auditory neuron-specific (Bhlhb5-cre) deletion of clarin-1 and clarin-2. These mice had normal audition up to 6 months of age, with no cochlear sensory epithelial changes or primary auditory neuron degeneration. To dig deeper into the common and unique molecular functions of clarin-1 and clarin-2, I performed RNA-seq on whole organ of Corti from Clrn1-/-, Clrn2-/-, and Clrn1-/-Clrn2-/- mice. In accordance with physiological observations, I found dysregulation in 8 distinct and physiologically relevant categories: cationic flux, synaptic organization and function, endocytosis and exocytosis, neuronal function and differentiation, metabolic function, actin and cytoskeletal organization, lipid homeostasis, and inflammation. We conclude that clarin-1 and clarin-2 play common and compensatory roles in mechanoelectrical transduction activity and pre- and post-synaptic integrity. The clarins are also required for auditory hair bundle integrity, ion homeostasis in auditory hair cells, and primary auditory neuronal survival. These findings will help elucidate novel mechanisms implicated in progressive hearing loss
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Rönnåsen, Berit. „Aspekter på lärande vid dövblindhet : möjligheter och begränsningar för personer med Alström syndrom“. Licentiate thesis, Örebro universitet, Institutionen för hälsovetenskap och medicin, 2015. http://urn.kb.se/resolve?urn=urn:nbn:se:oru:diva-44667.
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Dahlgren, Simon. „The association between cognition and speech-in-noise perception : Investigating the link between speech-in-noise perception and fluid intelligence in people with and without hearing loss“. Thesis, Linköpings universitet, Institutionen för datavetenskap, 2020. http://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-166708.
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The link between speech-in-noise recognition and cognition has been researched extensively over the years, and the purpose of this thesis was to add to this field. Data from a sample of 394 participants from the n200 database (Rönnberg et al., 2016) was used to calculate the correlation between their performance on a speech-in-noise test and their score on a test measuring fluid intelligence. The speech-in-noise test consisted of matrix sentences with 4-talker babble as noise and fluid intelligence was represented by the score on a Raven’s Progressive Matrices test. Around half of the participants (n = 199) had documented hearing loss and were hearing aid users, while the rest were participants with normal hearing. The overall correlation between speech-in-noise recognition and fluid intelligence was -.317, which shows that a better (lower) score on the speech-in-noise test is correlated to a better score in the Raven’s test. The same type of correlation was calculated within the two different groups, and the results showed correlation of -.338 for the group without hearing loss and one of -.303 for the group with hearing loss. The results indicate that there is a weak to moderate correlation between speech-in-noise and fluid intelligence, and they support the theory that cognitive processing is related to speech perception in all people, regardless of hearing status.
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Dell\'Aringa, Alfredo Rafael. „Contribuição ao estudo da etiologia das perdas auditivas hereditárias, progressivas e de causas desconhecidas“. Universidade de São Paulo, 1999. http://www.teses.usp.br/teses/disponiveis/5/5143/tde-05092014-101309/.
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O autor estudou famílias com surdez hereditária, congênita e de causas desconhecidas, na região da cidade de Marília, Estado de São Paulo (Brasil). Foram analisadas 33 famílias, com 100 pessoas afetadas. A história familiar de cada uma, abrangeu no mínimo três gerações. Todo paciente afetado, e seus familiares, que foram avaliados, submeteram-se a exame otorrinolaringológico, audiológico, laboratorial e radiológico. Heredogramas foram analisados para se avaliar o tipo de transmissão em cada família ( dominante, recessivo, ligado ao cromossomo X, genético desconhecido, não genético e de causa desconhecida). Verificou-se ainda a faixa etária predominante, a lateralidade, o grau de surdez, a evolução da perda auditiva, o tipo da curva audiométrica e o tipo da perda auditiva. O autor criou o Registro e Centro de Apoio das Perdas Auditivas Hereditárias ( RCAPAH), baseado no HHIRR(Hereditary Hearing Impairment Registry Resource ), existente no Boystown Hearing Loss Research Registry, da University of Nebraska, Omaha, U.S.A. Esse Registro fornece informações sobre avanços genéticos, aconselhamento e informes para as famílias, profissionais da saúde, sobre descobertas e avanços na genética da surdez, através de um informativo. Nesse estudo, o autor encontrou nas famílias analisadas, uma distribuição de transmissão de: 61% recessivas, 12% dominantes, 18% desconhecidas ou não genéticas, e 9% não genéticas. Quanto à faixa etária obtivemos 83%. Quanto à orelha comprometida 100% bilateral. Quanto ao grau de severidade, obtivemos surdez profunda em 65% dos casos, 25% severas, 9% moderadas, e 1% leve. Quanto à evolução encontramos 83% não progressivas e 17 % progressivas. Em relação ao tipo de curva audiométrica, encontramos 92% do tipo plano, 7% com queda em freqüências agudas e 1% com queda em freqüências graves. Com relação ao tipo de perda, obtivemos 99% neurosensorial e 1% condutivaThe author studied families with Hereditary, Progressive, and Unknown Deafness in the Marilia region, São Paulo, Brazil, between October 1996 and December 1998. During that time, 33 families with 100 persons affected were found. The family history of each family included at least three generations. All affected patients, and patterns, that was available had the following examined: otolaryngology (ear, nose, throat and mouth); audiological, blood examination , and radiological. Pedigrees were analyzed for the type of transmission in each family (dominant, recessive, X-linked, unknown genetic, non-genetic or unknown). The author created a Registry and Center of Support for Hereditary Hearing Loss, based upon the Boystown Hearing Loss Research Registry. These registries provide information to aid genetic counseling and inform families and health care providers about research and advances in genetic deafness by a newsletter. The distribution of transmission in the families are as follows: 61% recessive, 12% dominant, 18% unknown or non genetic, and 9% non-genetic ,according the age founded 83% between 0 and 1 year, according the ear 100% bilateral, according the severity 65% with profound, 25% with severe, 9% moderate and 1% superficial, according the evolution 83% no progressive, 17% progressive, according the type 92% plane, 7% loss on acute and 1% on grave, according the loss, 99% neurosensorial, 1% conductive. 1 1. INTRODUÇÃO O estudo e a história da genética humana é diferente do estudo e da história da genética vegetal e animal. As semelhanças entre pais e filhos, entre as famílias, como também, a existência de doenças hereditárias, intriga os cientistas e os médicos através dos anos. Entretanto, algumas descrições sistemáticas de doenças hereditárias no homem foram descritas antes do século X, sendo que as primeiras referências de perdas auditivas aparecem no século XVI ,MOTULSKI (1959). Vários autores já faziam referência sobre o termo genética médica antes do século X. Assim, MALPERTIUS, em 1752, publicou a descrição de uma família com polidactilia em quatro gerações e demonstrou que essa característica poderia ser igualmente transmitida por pai e mãe. Demonstrou ainda, por cálculos de probabilidade, que o acaso sozinho não poderia descrevê-la. As características ligados ao sexo, como daltonismo e hemofilia, foram estabelecidas no final do século VIII e início do IX. PORTAL, em 1808, publicou a primeira edição de um tratado intitulado \"Considérations sur la Nature et traitement de quelques maladies héréditaries ou de famille\", onde faz referências a doenças hereditárias. Posteriormente, em 1814, um livro intitulado \"A Treatise on the Supposed Hereditary Properties of Disease\", publicado em Londres por JOSEPH ADAMS, descreve os princípios gerais da genética médica que são válidos até hoje, 185 anos depois. O objetivo desse livro foi o de prover um guia para o entendimento da hereditariedade humana através da transmissão de doenças genéticas. O autor descreve também uma família com otoesclerose ao longo de quatro
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Kii, Márcia Akemi. „Perfil de auto-anticorpos na surdez súbita, surdez rapidamente progressiva e doença de Ménière“. Universidade de São Paulo, 2004. http://www.teses.usp.br/teses/disponiveis/5/5143/tde-13102014-111008/.
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A surdez neurossensorial imunomediada (SNIM) é uma das formas reversíveis de surdez neurossensorial, justificando a necessidade de identificação de marcadores mais específicos que ajudem na sua abordagem. O Western blot com antígenos de tecidos bovinos detecta um anticorpo contra a proteína de 68kD (hsp-70) em SNIM. Entretanto, anticorpos antihsp- 70 são comumente encontrados em indivíduos sadios. O objetivo do estudo foi determinar a reatividade de soros de pacientes com doenças otológicas freqüentemente relacionadas à etiologia auto-imune contra antígeno celular de linhagem humana (HeLa) através do Western blot, comparando com outros marcadores sorológicos de auto-imunidade. Soros de 81 pacientes com surdez neurossensorial (25 surdez súbita, 35 rapidamente progressiva e 21 doença Ménière) foram testados por Western blot utilizando extrato total de célula HeLa como antígeno alvo. Os pacientes com surdez foram comparados com indivíduos com audição normal e sem queixas otológicas ou história de doença sistêmica auto-imune (n=48). Observou-se reatividade contra célula HeLa principalmente nas regiões de 42, 48 and 62kD no grupo com surdez. O padrão de reatividade foi diferente entre os diferentes subgrupos de surdez. A reatividade contra as bandas de 48 and 62kD foi observada em surdez súbita e surdez rapidamente progressiva. A proteína de 48kD proveniente da surdez rapidamente progressiva parece ser diferente daquela encontrada em surdez súbita devido a sua resistência à tripsina. Este estudo demonstrou a existência de autorreatividade contra células HeLa na surdez súbita, surdez rapidamente progressiva e doença de Ménière, sugerindo a presença de novos autoanticorpos. O seu papel é ainda desconhecido. Estudos detalhados são necessários para avaliar a real relevância clínica desta autorreatividade na patologia de orelha interna ou como marcador prognóstico ou diagnósticoImmune-mediated sensorineural hearing loss (SNHL) is one of few forms of reversible SNHL, justifying the need to define more specific markers to help clinical approach. Western blot with bovine tissues detect an autoantibody against the 68kD protein (hsp70) in immune-mediated SNHL. However, antihsp70 antibodies are also common in healthy individuals. The objective of this study was to determinate the reactivity of serum from patients with otologic entities often related to autoimmune etiology against human cell line antigen (HeLa) by Western blot, comparing to other serological markers. Sera of 81 patients with SNHL (25 sudden SNHL, 35 rapidly progressive SNHL and 21 Ménière\'s disease patients) were tested by Western blot using HeLa cell total extract as target. Western blot outcome was compared with detection of other current autoimmune markers. Experimental group data were compared to normal-hearing subjects (n=48) without otologic or systemic autoimmune disease. Reactivity to HeLa cells was observed mostly at 42, 48 and 62kD region which pattern was different among different groups. Binding to 48 and 62kD HeLa cell antigen was observed in sudden SNHL and rapidly progressive SNHL. The 48kD protein from rapidly progressive SNHL seems to be different from sudden SNHL\'s according to resistance to trypsin. This study detected autoreactivity to HeLa cells in sudden SNHL, rapidly progressive SNHL and Ménière\'s disease, suggesting the presence of new autoantibodies. Their role is still unknown and further studies should evaluate their relevance on pathology in the inner ear or as diagnostic or prognostic marker
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Baganha, Sara Barbosa. „Asymmetric Sensorineural Hearing Loss in Children: progression and involvement of the contralateral ear“. Master's thesis, 2021. http://hdl.handle.net/10316/98312.
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Trabalho Final do Mestrado Integrado em Medicina apresentado à Faculdade de MedicinaObjective: Sensorineural hearing loss (SNHL) has a profound negative impact on children’s lives. This study aims to examine the progression of asymmetric sensorineural hearing loss in children, according to the baseline hearing thresholds in the worst ear and to the technological level of the hearing aid fitted. Methods: Eighteen children with asymmetric SNHL fitted with a nonlinear hearing aid for more than 2 years were selected for this retrospective study. The participants were interviewed and submitted to a pure tone audiogram at the age of 5 years (T0) and again at the age of 10 years (T1), performed as part of the usual medical follow-up. Children were divided into 3 groups, according to the technological level of the hearing aid fitted: low, middle, advanced. Results: There were 3 cases of unilateral SNHL and 15 cases of bilateral asymmetric SNHL. A positive Pearson correlation was established between the hearing thresholds in the worst ear at T0 and in the best ear at T1: weak at 1kHz, moderate at 0.25kHz and at 2kHz, and strong at 4kHz. A Wilcoxon test for paired samples showed that in the worst ear there is no significant difference between the hearing thresholds at T0 and T1 regardless of the technological level of the hearing aid fitted. In the best ear, a Wilcoxon test showed a statistically significant progression of the SNHL in children fitted with low technological level hearing aids, and a not statistically significant improvement of the hearing thresholds in children fitted with technologically advanced hearing aids. Conclusions: This study shows that the baseline hearing thresholds in the worst ear influence the progression of the SNHL in the best ear over time, especially at high frequencies. Even though the results were not statistically significant, technologically advanced hearing aids led to a slower progression of the SNHL and to an improvement of the hearing thresholds over time in controlled laboratory conditions.
Objetivo: A surdez neurossensorial (SNS) tem um profundo impacto negativo na vida das crianças. Este estudo tem como objetivo avaliar a progressão da SNS assimétrica na criança, de acordo com os limiares auditivos basais do pior ouvido e com a gama tecnológica do aparelho auditivo equipado. Métodos: Dezoito crianças com SNS assimétrica reabilitadas com um aparelho auditivo não linear há pelo menos 2 anos foram selecionadas para este estudo retrospetivo. Os participantes foram entrevistados e submetidos a um Audiograma Tonal Simples aos 5 anos de idade (T0) e novamente aos 10 anos de idade (T1), realizados no âmbito do seguimento médico habitual. As crianças foram divididas em 3 grupos, de acordo com a gama tecnológica do aparelho auditivo utilizado: baixa, intermédia, avançada. Resultados: Foram detetados 3 casos de SNS unilateral e 15 casos de SNS bilateral assimétrica. Foi estabelecida uma correlação de Pearson positiva entre os limiares auditivos do pior ouvido em T0 e do melhor ouvido em T1: fraca em 1kHz, moderada em 0.25kHz e em 2kHz, e forte em 4kHz. Um teste de Wilcoxon para amostras emparelhadas demonstrou que, no pior ouvido, não há diferença estatisticamente significativa entre os limiares auditivos em T0 e T1 independentemente da gama tecnológica do aparelho auditivo utilizado. No melhor ouvido, o teste de Wilcoxon revelou uma progressão estatisticamente significativa da SNS nas crianças reabilitadas com um aparelho auditivo de gama baixa, e uma melhoria não estatisticamente significativa dos limiares auditivos nas crianças reabilitadas com um aparelho auditivo de gama avançada. Conclusão: Este estudo mostra que os limiares auditivos basais do pior ouvido influenciam a progressão da SNS no melhor ouvido ao longo do tempo, sobretudo em frequências elevadas. Apesar dos resultados não terem sido estatisticamente significativos, os aparelhos auditivos de gama avançada associaram-se a uma progressão mais lenta da SNS e a uma melhoria dos limiares auditivos em condições controladas.
Bücher zum Thema "Progressive hearing loss"
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Heidet, Laurence, Bertrand Knebelmann und Marie Claire Gubler. Alport syndrome. Herausgegeben von Neil Turner. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0321.
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Alport syndrome is an inherited renal disorder characterized by early haematuria, progressing to proteinuria, sensorineural hearing loss, and progressive renal failure typically in the third or fourth decade but with wide variation. It is responsible for about 1% of end-stage renal failure. Over 80% of cases are X-linked and young men are most affected, but heterozygous carriers of the abnormal gene are also at significantly increased risk of end-stage renal failure in their lifetime. Those affected by the autosomal recessive variant are phenotypically very similar. It is caused by mutations in tissue-specific isoforms of basement membrane (type IV) collagen encoded by COL4A5 (X chromosome), COL4A3, and COL4A4 (chromosome 2).
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Heidet, Laurence, Bertrand Knebelmann und Marie Claire Gubler. Alport syndrome. Herausgegeben von Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0322_update_001.
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This chapter describes the clinical features of Alport syndrome. The characteristic features of this familial condition are haematuria with progressive nephropathy and sensorineural hearing loss. Most cases are X-linked so this is typically seen in boys and young men, but female heterozygous (‘carriers’) of X-linked Alport syndrome are also at significant risk of renal disease in their lifetime. The average age of end-stage renal failure is in the third or fourth decade. Those with autosomal recessive disease (approximately 15%) show a similar phenotype. Hearing loss characteristically develops during teenage years or as a young adult, usually as proteinuria becomes prominent and renal function begins to be lost. Angiotensin-converting enzyme inhibitors may modify this classic description. Ocular abnormalities are less consistent and tend to occur later, often after end-stage renal failure. Retinal changes do not affect sight. Lenticonus can be treated by lens replacement. Other ocular abnormalities occur rarely. Aortic disease has been reported in occasional families.
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Lucas, Joshua, Dawn Fishback und Steven Giannotta. Skull Base Schwannoma. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190696696.003.0013.
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This chapter presents a case example of a patient with progressive unilateral hearing loss who was ultimately diagnosed with a skull base schwannoma. The workup and differential diagnosis are presented and options for treatment are reviewed based on published evidence. Treatment options include observation, stereotactic radiosurgery, and surgical resection. The objective status of a patient’s hearing as well as the patient’s age influence treatment recommendations and the surgical approach. Intraoperative neuromonitoring provides real-time assessment of facial nerve irritation as well as cochlear nerve function and is an important component of surgery. Complication avoidance and management are also discussed in this chapter.
Buchteile zum Thema "Progressive hearing loss"
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Naz, Sadaf. „Genetics of Nonsyndromic Recessively Inherited Moderate to Severe and Progressive Deafness in Humans“. In Hearing Loss. InTech, 2012. http://dx.doi.org/10.5772/31808.
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„9 Unilateral Slowly Progressive Hearing Loss“. In Differential Diagnosis in Otolaryngology-Head and Neck Surgery, herausgegeben von Michael G. Stewart und Samuel H. Selesnick. Stuttgart: Georg Thieme Verlag, 2011. http://dx.doi.org/10.1055/b-0034-81089.
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„Idiopathic Progressive Bilateral Sensorineural Hearing Loss (IPBSNHL)“. In Encyclopedia of Otolaryngology, Head and Neck Surgery, 1225. Berlin, Heidelberg: Springer Berlin Heidelberg, 2013. http://dx.doi.org/10.1007/978-3-642-23499-6_100476.
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Brice, Alejandro. „Noise Induced Hearing Loss: A Case Study from a Speech-Language Pathologist’s Perspective“. In Hearing Loss - From Multidisciplinary Teamwork to Public Health. IntechOpen, 2021. http://dx.doi.org/10.5772/intechopen.96332.
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Hearing loss is very common in the United States and the most widespread disability in the U.S. Hearing loss is the third most chronic health condition in the U.S. Noise induced hearing loss (NIHL) results from damaging external noise. This injury leads to temporarily or permanently affecting sensitive inner ear structures (e.g., cochlea, organ of Corti, and hair cells). NIHL can result from a single high-level noise exposure or repeated exposures to excessively loud noises [i.e., typically 85 dBA or greater, (A weighted decibel)]. Damage to the inner ear can also result from aging (i.e., presbycusis). This case study documents the hearing loss of an otherwise healthy 21-year-old, male individual and his progressive moderate-to-severe sensorineural hearing loss over a period of 41 years. His history will be reported along with his perspective as a speech-language pathologist and speech scientist. The individual with hearing loss has adapted to wearing hearing aids over the last five years. Issues that have occurred affecting comprehension along with compensatory strategies that assisted listening and comprehension will be discussed.
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Toriello, Helga V. „Genetic Hearing Loss Associated with Neurologic and Neuromuscular Disorders“. In Hereditary Hearing Loss And Its Syndromes, 290–358. Oxford University PressNew York, NY, 2004. http://dx.doi.org/10.1093/oso/9780195138498.003.0010.
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Abstract Many progressive neurodegenerative diseases are associated with sensorineural hearing loss, but the classification of these disorders remains controversial. This chapter combines reports of families with similar disorders and different ages of onset, but separates similar families if the pattern of inheritance was different. It is apparent that some neurological manifestations of these diseases tend to cluster. For example, ataxia is frequently associated with spastic paraplegia (“spinocerebellar ataxia”) and with motor and sensory neuropathy. Pigmentary retinopathies (which include retinitis pigmentosa) are often associated with optic atrophy. Some are considered in this chapter, but others were assigned to Chapter 7. In many older reports, modern neurodiagnostic tests often were not available, making clinical distinctions more difficult. Because of the clinical similarity, it is possible that some disorders described in this section represent unrecognized mitochondrial encephalomyopathies.
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Glindzicz, Maria Bitner, Karen E. Heath, und Angel Campos-Barros. „Genetic Hearing Loss Associated with Renal Disorders“. In Hereditary Hearing Loss And Its Syndromes, 267–89. Oxford University PressNew York, NY, 2004. http://dx.doi.org/10.1093/oso/9780195138498.003.0009.
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Abstract In this chapter, we have attempted to place the conditions considered here into groups such as specific nephritides, nephroses, and renal acidoses. Molecular genetic studies are rapidly clarifying the molecular basis of various syndromes, confirming genetic heterogeneity in some while identifying allelic disorders in others. Alport syndrome (nephritis and sensorineural hearing loss) The syndrome of hereditary progressive glomerulonephritis with intermittent or gross hematuria and sensorineural hearing loss was first described by Alport (I) in 1927. Earlier reports of the same family were published by Guthrie (45) and Kendall and Hurst (67). Classic Alport syndrome represents a defect in type IV collagen. What has been called Alport syndrome is now known to represent a genetically heterogeneous group of at least six different disorders (Table 9-1) that exhibit unique ultrastructural and antigenic abnormalities in basement membranes due to defective type IV collagen (7,59,60,62,64,106). The gene frequency is about 1/5000 (30,33). The syndrome is seen in at least I% of those with congenital hearing loss and in 0.6% of those seeking renal replacement (30).
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Mara, Rissatto-Lago. „Hearing Damage Caused by Sickle Cell Disease“. In Sickle Cell Disease [Working Title]. IntechOpen, 2022. http://dx.doi.org/10.5772/intechopen.104705.
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Sickle cell disease (SCD) is a multisystem disease associated with episodes of acute illness and progressive organ damage, leading to impairment of several organs. It is characterized by vaso-occlusive processes resulting from local hypoxia, increased number of sickled erythrocytes, and dissemination of occlusion to adjacent tissues. SCD has a chronic inflammatory mechanism that affects several organs and systems, including the auditory system. Hearing loss resulting from SCD includes conductive hearing loss, sensorineural hearing loss, in the central auditory system, in addition to otoneurological symptoms. These findings occur in both the adult and pediatric populations. At the end of this chapter, it is expected that the reader will be able to identify the main damages in the auditory system resulting from sickle cell disease, understand the pathophysiology of the damage generated in hearing, as well as understand the main care needed to monitor the hearing health of this population.
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Willott, James F. „Central auditory plasticity in mouse models of progressive sensorineural hearing loss“. In Reprogramming the Cerebral Cortex, 181–92. Oxford University Press, 2006. http://dx.doi.org/10.1093/acprof:oso/9780198528999.003.0009.
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Stevens, Michael. „New Insights into Beethoven’s Deafness“. In Pharynx – the Incredible Rendezvous Sites of Gas, Liquid and Solid [Working Title]. IntechOpen, 2022. http://dx.doi.org/10.5772/intechopen.101889.
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There have been many theories proposed to explain the deafness of Ludwig van Beethoven, because his history is complex. Evidence of otosclerosis is lacking, because close gross examination of Beethoven’s middle ear at autopsy did not find any otosclerotic foci. His slowly progressive hearing loss over a period of years differs from the reported cases of autoimmune hearing loss, which is rapidly progressive over a period of months. The absence of mercury in Beethoven’s hair and bone samples leads us to conclude that his deafness was not due to syphilis, because in that era, syphilis was treated with mercury. Microscopic examination of bone samples and examination of the middle ear have found no evidence of Paget’s Disease. High levels of lead found deep in the bone suggest repeated exposure over a long period of time. The finding of shrunken cochlear nerves at his autopsy is consistent with axonal degeneration due to heavy metals such as lead. Chronic low-level exposure, like Beethoven’s, causes sensory and autonomic findings rather than the classic wrist drop due to motor neuropathy. Beethoven’s physicians thought that he had alcohol dependence. He particularly liked wine to which lead had been added to improve the flavor. A live patient reported in 2021 from Italy with a slowly progressive hearing loss and other symptoms like Beethoven had, was found to have lead poisoning. Therefore, the most likely cause of Beethoven’s deafness was his consumption of wine tainted with lead.
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Geisler, C. Daniel. „Damage to the Ear and Hearing Impairment“. In From Sound To Synapse, 275–98. Oxford University PressNew York, NY, 1998. http://dx.doi.org/10.1093/oso/9780195100259.003.0016.
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Abstract The ear does an exquisite job of transforming acoustic signals, varying enor mously in amplitude and waveform, into a regimented neural code. The hearing that normally results is one of our greatest gifts. With it we monitor our environment in all directions, communicate with one other, and listen to the music of instruments and babbling children. The loss of this treasure can bring severe behavioral deficits (Gravel and Ruben, 1996) as well as untold personal agony. A poignant example is provided in a moving letter from the 31-year-old Beethoven to his brothers, in which he attempts to describe his misery due to a progressive hearing loss. Not only was he unable to appreciate performances of his music, he found it difficult to communicate with other people and became a virtual recluse. He even contemplated suicide (van Beethoven, 1802).
Konferenzberichte zum Thema "Progressive hearing loss"
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Baumgartner, Wolf-Dieter, Faris Brkic und Alexandra Jappel. „Progressive Sensorineural Hearing Loss in Vibrant Soundbridge Users Requiring Cochlear Implantation“. In 94. Jahresversammlung Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e.V., Bonn. Georg Thieme Verlag, 2023. http://dx.doi.org/10.1055/s-0043-1766698.
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Baumgartner, Wolf-Dieter, Faris Brkic und Alexandra Jappel. „Progressive Sensorineural Hearing Loss in Vibrant Soundbridge Users Requiring Cochlear Implantation“. In 94th Annual Meeting German Society of Oto-Rhino-Laryngology, Head and Neck Surgery e.V., Bonn. Georg Thieme Verlag, 2023. http://dx.doi.org/10.1055/s-0043-1767311.
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Momper, Theresa, Robert Mlynski und Stefanie Rettschlag. „Rare differential diagnosis of a patient with bilateral sensorineural hearing loss and progressive Tinnitus“. In 95th Annual Meeting German Society of Oto-Rhino-Laryngology, Head and Neck Surgery e. V., Bonn. Georg Thieme Verlag KG, 2024. http://dx.doi.org/10.1055/s-0044-1784626.
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Babirsoy, Dadash, Tina Brzoska, Friedrich Ihler und Oliver Dziemba. „Unexpected inadequate speech intelligibility with cochlear implant (CI) in an 81-year-old female patient with progressive hearing loss.“ In 94th Annual Meeting German Society of Oto-Rhino-Laryngology, Head and Neck Surgery e.V., Bonn. Georg Thieme Verlag, 2023. http://dx.doi.org/10.1055/s-0043-1767326.
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Martinelli, Jose, Jessica Ivanovs und Marcos Martinelli. „GERIATRIC EVALUATION IN 27 CASES OF MUSICAL HALLUCINATION“. In XIII Meeting of Researchers on Alzheimer's Disease and Related Disorders. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1980-5764.rpda073.
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Background: Musical hallucination (AM) is a type of complex auditory hallucination described as hearing musical tones, rhythms, harmonies, and melodies without the corresponding external auditory stimulus. This type of hallucination is relatively rare and is seen less often than other types of hallucination. Such hallucinations can be continuous or intermittent and are usually accompanied by a clear and critical awareness on the part of the patient. AM are found mainly in elderly women with progressive hearing loss, usually due to ear diseases or lesions. They also occur in neurological disorders, neuropsychological disorders (eg dementia) and psychiatric disorders, especially depression. Objective: To evaluate clinical and neuropsychological issues of the elderly with Musical Hallucinations Methods: Twenty-seven outpatient patients clinic of Geriatrics and Gerontology at FMJ from January 2010 to October 2019 were selected Results: Of the 27 patients, 20 were women. The average age was 83.47 years. The most prevalent diseases were systemic arterial hypertension, osteoporosis, diabetes mellitus, hypothyroidism, osteoporosis, chronic obstructive pulmonary disease and dementia syndrome. With the exception of one patient, all had hearing loss. The songs were the most varied from Gregorian chant to lullaby. Many had this picture for months and continuously (day and night). 40% of them had no insight into AM. We emphasize that all these patients sought medical care with the main complaint of musical hallucination. Conclusion: In general, AM has an uninterrupted, fragmentary and repetitive character. They are involuntary, intrusive and have an apparent exteriority. They differ from the simple mental image of auditory sensation in that they appear to come from outside the individual as if they actually hear an external device playing music. Currently, it is estimated that about 2% of elderly people with hearing loss also have AM. The neuropsychological basis of AM is not fully established. The phenomenological study, especially the perception of complex sequences and consistency with previous auditory experience strongly suggest the involvement of central auditory processing mechanisms. Normal musical auditory processing involves several interrelated brain levels and subsystems. While the recognition of elementary sounds is done in the primary auditory cortex, the recognition of musical characteristics such as notes, melody and metric rhythm occur in a secondary and tertiary association center, which in turn, are greatly influenced by regions linked to memory and emotion.
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Soares, Izadora Fonseca Zaiden, João Nicoli Ferreira dos Santos und Lis Gomes Silva. „Dramatic cognitive improvement with acetylcholinesterase inhibitor in cerebral amyloid angiopathyrelated inflammation“. In XIII Congresso Paulista de Neurologia. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1516-3180.578.
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Context: Cerebral amyloid angiopathy (CAA) is characterized by progressive deposition of amyloid-ß fibrils in the walls of small arterioles and capillaries of the leptomeninges and cerebral cortex. A rare subtype of CAA is CAA-related inflammation (CAA-RI), which exhibits marked perivascular or transmural inflammatory infiltration in brain tissue. The major clinical features of CAA-RI are rapidly progressive dementia, behavioral changes, headache, seizures, or stroke-like signs. Conclusive diagnosis requires histopathological confirmation, but validated clinicoradiological criteria for the diagnosis of probable CAA-RI have good sensitivity (82%) and specificity (97%). Treatment with high dose corticosteroids with or without other immunosuppressive therapy is recommended. We report a case of probable CAA-RI that did not respond to corticosteroid therapy but had a surprising improvement with acetylcholinesterase inhibitor. Case report: A 77-year-old illiterate woman presented with a history of subacute onset of seizures and behavioral changes. Her medical history was positive for a hearing loss due to a toxic exposure in childhood, and a cured breast cancer. The neurological examination showed attention impairment, disorientation, and incoherent speech. CSF showed a mildly elevated protein count. Brain MRI met criteria for probable CAA-RI. She had a poor response with high doses of corticosteroids, but after a trial with Donepezil she showed important cognitive and functional improvement. Conclusion: This result attracts attention to the importance of the cholinergic pathway in the etiology and pathological mechanisms of CAA. Randomized Controlled Trials would be required to confirm our hypothesis and to find new therapeutic options for CAA.
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Moya, Manoel Vilela, Mariana Laranjeira Pierotti und Alyosha Fabiana Rodrigues. „Relationship between hearing loss and cognitive memory decline in an elderly population“. In XIII Congresso Paulista de Neurologia. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1516-3180.666.
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Background: The major complaints of the elderly are hearing and memory loss, which have a devastating impact on the communication process. Previous studies have shown that hearing loss is associated with the acceleration of this cognitive decline. Objectives: To analyze the relationship between moderate to moderatelysevere hearing loss and memory deficit in elderly individuals. Design and setting: This is an observational, cross-sectional study realized in seniors of Hearing Care Program at the Taubaté University Hospital, Taubaté - SP/ Brazil. Methods: Data was collected using audiometry, anamnesis, and the cognition test Mini Mental State Examination (MMSE) in 60-75 years old individuals, without knowledge of previous cognitive memory deficit and without the use of hearing aids. Results: Between the 61 seniors interviewed, 68% had moderate degree of deafness and 32% moderately severe degree. Among the normal results in the MMSE, 24% had moderately severe deafness; of those with cognitive impairment without indication of investigation of dementia, 35.7% had moderately severe deafness, and of those with indication of investigation of dementia, 50% had moderately severe deafness. In addition, 23% of the total sample had results in the normal cutoff range, showing the tendency for cognitive decline in this population. Conclusions: these data indicate a progression in the proportion of individuals with a higher degree of hearing loss, the higher the cognitive deficit.
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Silva, Bruno Custódio, Tatiane Andressa Gasparetto, Fábio Biguelini Duarte, Paulo Ricardo Gazzola Zen und Rafael Fabiano Machado Rosa. „Clinical and neurological findings of a patient with type 2 neurofibromatosis“. In XIII Congresso Paulista de Neurologia. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1516-3180.069.
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Context: Neurofibromatosis type 2 (NF2) is an autosomal dominant genetic disease that predisposes to tumors development, especially schwannomas involving vestibular nerves. Case report: A 13-year-old girl, a couple’s daughter with no cases of genetic diseases in the family, had photophobia and recurrent left eye paralysis since she was 5 years old. At 11, she had dizziness at rest and on moving. Physical exam showed a lack of balance, weakness in the legs and bilateral papilledema. Cranial computed tomography revealed a bilateral vestibular nerve schwannoma. The increase in tumor volume led to obstructive hydrocephalus and hypertensive signs. She evolved with vision loss and magnetic resonance imaging showed ventricular dilation, thus she underwent endoscopic third ventriculostomy. The patient reported a gradual worsening of balance when walking and episodes of sporadic headache, progressing to seizures treated with valproic acid. Ophthalmological evaluation revealed vision loss and small bilateral scotoma in campimetry. She reported occasional ear pain and audiometry showed mild bilateral hearing loss. A surgery plan was performed for tumor resection. Conclusions: The clinical findings and complementary exams were compatible with the diagnosis of NF2, which required ventricular shunt and indication for tumor removal, according to disease progression. Thus, these patients must be monitored, due to symptoms progression as well as oriented to family recurrence possibility.
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Trier, Barbara, Daniel Hirth, Timo Stöver und Sabine Kramer. „Progression, severity and management of hearing loss in preschool children with congenital CMV infection“. In 94th Annual Meeting German Society of Oto-Rhino-Laryngology, Head and Neck Surgery e.V., Bonn. Georg Thieme Verlag, 2023. http://dx.doi.org/10.1055/s-0043-1767529.
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Krech, Lisa, Anja Pähler vor der Holte, Merle Bock, Yasmin Loga, Martin Seidel, Meike Ricke und Hans-Jürgen Welkoborsky. „Health care research project on hearing ability and Dementia in residential care facilities in the greater Hanover area – Progression of hearing loss and Dementia“. In 95th Annual Meeting German Society of Oto-Rhino-Laryngology, Head and Neck Surgery e. V., Bonn. Georg Thieme Verlag KG, 2024. http://dx.doi.org/10.1055/s-0044-1785160.
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