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1.
J Clin Neurosci ; 124: 1-14, 2024 Jun.
Article de Anglais | MEDLINE | ID: mdl-38615371

RÉSUMÉ

BACKGROUND: Vestibular schwannomas (VS) are benign tumors arising from vestibular nerve's Schwann cells. Surgical resection via retrosigmoid (RS) or middle fossa (MF) is standard, but the optimal approach remains debated. This meta-analysis evaluated RS and MF approaches for VS management, emphasizing hearing preservation and Cranial nerve seven (CN VII) outcomes stratified by tumor size. METHODS: Systematic searches across PubMed, Cochrane, Web of Science, and Embase identified relevant studies. Hearing and CN VII outcomes were gauged using the American Academy of Otolaryngology-Head and Neck Surgery, Gardner Robertson, and House-Brackmann scores. RESULTS: Among 7228 patients, 56 % underwent RS and 44 % MF. For intracanalicular tumors, MF recorded 38 % hearing loss, compared to RS's 54 %. In small tumors (<1.5 cm), MF showed 41 % hearing loss, contrasting RS's lower 15 %. Medium-sized tumors (1.5 cm-2.9 cm) revealed 68 % hearing loss in MF and 55 % in RS. Large tumors (>3cm) were only reported in RS with a hearing loss rate of 62 %. CONCLUSION: Conclusively, while MF may be preferable for intracanalicular tumors, RS demonstrated superior hearing preservation for small to medium-sized tumors. This research underlines the significance of stratified outcomes by tumor size, guiding surgical decisions and enhancing patient outcomes.


Sujet(s)
Neurinome de l'acoustique , Procédures de neurochirurgie , Humains , Fosse crânienne moyenne/chirurgie , Nerf facial/chirurgie , Ouïe/physiologie , Perte d'audition/étiologie , Perte d'audition/prévention et contrôle , Perte d'audition/chirurgie , Neurinome de l'acoustique/chirurgie , Procédures de neurochirurgie/méthodes
2.
Eur J Pediatr ; 183(1): 61-71, 2024 Jan.
Article de Anglais | MEDLINE | ID: mdl-37870611

RÉSUMÉ

The optimal duration of antibiotic treatment for the most common bacterial meningitis etiologies in the pediatric population, namely Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis, is not well-established in the literature. Therefore, we aimed to perform an updated meta-analysis comparing shorter versus longer antibiotic treatment in children with meningitis. PubMed, EMBASE, and Cochrane databases were searched for randomized controlled trials (RCTs) that compared shorter (up to 7 days) versus longer (10 days or double the days of the equivalent short course) duration of antibiotic treatment in children with meningitis and reported the outcomes of treatment failure, death, neurologic sequelae, non-neurologic complications, hearing impairment, nosocomial infection, and relapse. Heterogeneity was examined with I2 statistics. RevMan 5.4.1 was used for statistical analysis and RoB-2 (Cochrane) for risk of bias assessment. Of 684 search results, 6 RCTs were included, with a cohort of 1333 children ages 3 weeks to 15.5 years, of whom 49.51% underwent a short antibiotic course. All RCTs included monotherapy with ceftriaxone, except one, which added vancomycin as well. No differences were found comparing the short and long duration of therapy concerning treatment failure, relapse, mortality, and neurologic complications at discharge and at follow-up.  Conclusion: Because no statistically significant differences were found between groups for the analyzed outcomes, the results of this meta-analysis support shorter therapy. However, generalizing these results to complicated meningitis and infections caused by other pathogens should be made with caution. (PROSPERO identifier: CRD42022369843). What is Known: • Current recommendations on the duration of antibiotic therapy for bacterial meningitis are mostly based on clinical practice. • Defining an optimal duration of antibiotic therapy is essential for antimicrobial stewardship achievement, improving patient outcomes, and minimizing adverse effects. What is New: • There are no differences between shorter versus longer antibiotic treatment duration in regard to treatment failure, relapse, mortality, neurologic complications, and hearing impairment at discharge and at follow-up.


Sujet(s)
Perte d'audition , Méningite bactérienne , Enfant , Humains , Antibactériens/effets indésirables , Méningite bactérienne/traitement médicamenteux , Méningite bactérienne/complications , Ceftriaxone/usage thérapeutique , Perte d'audition/étiologie , Perte d'audition/induit chimiquement , Récidive
3.
Braz J Otorhinolaryngol ; 90(1): 101342, 2024.
Article de Anglais | MEDLINE | ID: mdl-37879254

RÉSUMÉ

OBJECTIVES: To identify and understand the evidence regarding hearing changes related to acquired Dengue, Chikungunya, and Zika virus infection in adult individuals. METHODS: A scoping review was performed according to the recommendations of The Joanna Briggs Institute and guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews in the Embase, PubMed/Medline, ScienceDirect, Scopus, and Web of Science databases without restriction on language and year of publication. Case studies, observational studies, and clinical trials reporting hearing loss in adult subjects (>18-60 years of age) of both sexes with DENV, CHIKV, or ZIKV diagnosed by positive molecular/serological examination by RT-PCR or IgM/IgG by ELISA method were included. RESULTS: Thirteen studies met the inclusion criteria and were selected for review. The occurrence of auditory symptoms caused by arboviroses and the presence of permanent or transient sensorineural hearing loss was variable in adults. CONCLUSIONS: Dengue, Chikungunya, and Zika infections in adults are associated with a variety of auditory symptoms. The frequency of permanent or transient sensorineural hearing loss is low but not negligible.


Sujet(s)
Fièvre chikungunya , Virus du chikungunya , Co-infection , Virus de la dengue , Dengue , Surdité neurosensorielle , Perte d'audition , Infection par le virus Zika , Virus Zika , Adulte , Mâle , Femelle , Humains , Infection par le virus Zika/complications , Infection par le virus Zika/diagnostic , Fièvre chikungunya/complications , Fièvre chikungunya/diagnostic , Fièvre chikungunya/épidémiologie , Dengue/complications , Dengue/diagnostic , Dengue/épidémiologie , Co-infection/épidémiologie , Perte d'audition/étiologie , Surdité neurosensorielle/étiologie , Ouïe
4.
Distúrb. comun ; 35(3): 63637, 25/10/2023.
Article de Anglais, Portugais | LILACS | ID: biblio-1526043

RÉSUMÉ

Introdução: A COVID-19 pode afetar o sistema auditivo, justificando a avaliação da audição de indivíduos infectados. Objetivo: analisar a via auditiva até o tronco encefálico de indivíduos acometidos por COVID-19 comparados ao grupo controle. Método: Estudo observacional transversal analítico realizado em uma amostra não probabilística de adultos que tiveram COVID-19, que foram comparados com um grupo controle, sem queixa auditiva. A avaliação consistiu em: medidas de imitância acústica, audiometria tonal liminar (ATL), emissões otoacústicas evocadas por estímulo transiente (EOET) e potencial evocado auditivo de tronco encefálico (PEATE). Resultados: Foram avaliados 77 indivíduos, sendo, 41 participantes do grupo COVID-19 (idade média de 26,3) e 36 do grupo controle (idade média de 25,8). Os limiares auditivos estavam dentro dos padrões da normalidade para todos os indivíduos do grupo COVID-19, sendo significativamente maiores para as frequências de 1000, 2000 e 3000 Hz à direita. A amplitude das EOET foi significativamente menor na banda de frequência de 1500 à direita. Houve correlação significativa e negativa para as frequências de 1000 Hz e 3000 Hz à direita e para as frequências de 1000, 2000 e 3000 Hz à esquerda, entre EOET e ATL. Foi verificado aumento da latência absoluta da onda I, do PEATE, na orelha esquerda. Conclusão: a COVID-19 afetou locais específicos do sistema auditivo. Houve diminuição da acuidade auditiva e do funcionamento das células ciliadas externas da cóclea, bem como aumento do tempo de condução neural do som na porção distal do VII par craniano à esquerda. (AU)


Introduction: COVID-19 can affect the auditory system, justifying the evaluation of the hearing of infected individuals. Objective: to analyze the auditory pathway to the brainstem of individuals affected by COVID-19 compared to the control group. Method: Analytical cross-sectional observational study carried out in a non-probabilistic sample of adults who had COVID-19, who were compared with a control group, without hearing complaints. The evaluation consisted of: acoustic immittance measurements, pure tone audiometry (PTA), transient stimulus-evoked otoacoustic emissions (TEOAE) and brainstem auditory evoked potential (BAEP). Results: 77 individuals were evaluated, 41 participants in the COVID-19 group (average age of 26.3) and 36 in the control group (average age of 25.8). Hearing thresholds were within normal limits for all individuals in the COVID-19 group, being significantly higher for the frequencies of 1000, 2000 and 3000 Hz on the right. TEOAE amplitude was significantly lower in the 1500 frequency band on the right. There was a significant and negative correlation for the frequencies of 1000 Hz and 3000 Hz on the right and for the frequencies of 1000, 2000 and 3000 Hz on the left, between TEOAE and PTA. An increase in the absolute latency of wave I, of the BAEP, was observed in the left ear. Conclusion: COVID-19 affected specific locations in the auditory system. There was a decrease in auditory acuity and the functioning of the outer hair cells of the cochlea, as well as an increase in the neural conduction time of sound in the distal portion of the VII cranial nerve on the left. (AU)


Introducción: COVID-19 puede afectar el sistema auditivo, justificando la evaluación de la audición de individuos infectados. Objetivo: analizar la vía auditiva hacia el tronco encefálico de individuos afectados por COVID-19 en comparación con el grupo control. Método: Estudio observacional analítico transversal realizado en una muestra no probabilística de adultos que padecieron COVID-19, quienes fueron comparados con un grupo control, sin escuchar quejas. La evaluación consistió en: mediciones de inmitancia acústica, audiometría de tonos puros (ATP), otoemisiones acústicas provocadas por estímulos transitorios (OAET) y potenciales evocados auditivos del tronco encefálico (PEATE). Resultados: Se evaluaron 77 individuos, 41 participantes en el grupo COVID-19 (edad promedio de 26,3 años) y 36 en el grupo control (edad promedio de 25,8 años). Los umbrales de audición estaban dentro de los límites normales para todos los individuos del grupo de COVID-19, siendo significativamente más altos para las frecuencias de 1000, 2000 y 3000 Hz de la derecha. La amplitud de OAET fue significativamente menor en la banda de frecuencia de 1500 de la derecha. Hubo correlación significativa y negativa para las frecuencias de 1000 Hz y 3000 Hz a la derecha y para las frecuencias de 1000, 2000 y 3000 Hz a la izquierda, entre OAET y ATP. Se observó un aumento de la latencia absoluta de la onda I, del PEATE, en el oído izquierdo. Conclusión: COVID-19 afectó ubicaciones específicas del sistema auditivo. Hubo una disminución de la agudeza auditiva y del funcionamiento de las células ciliadas externas de la cóclea, así como un aumento del tiempo de conducción neural del sonido en la porción distal del VII par craneal izquierdo. (AU)


Sujet(s)
Humains , Mâle , Femelle , Adolescent , Adulte , Adulte d'âge moyen , Jeune adulte , COVID-19/complications , Ouïe/physiologie , Études cas-témoins , Études transversales , Perte d'audition/diagnostic , Perte d'audition/étiologie , Tests auditifs
5.
Distúrb. comun ; 35(3): 60822, 25/10/2023.
Article de Anglais, Portugais | LILACS | ID: biblio-1526063

RÉSUMÉ

Introdução: Com base na necessidade do diagnóstico audiológico e da intervenção precoce na vida de uma criança com perda auditiva, faz-se necessário a elaboração de protocolos de avaliação auditiva que forneçam o maior número de informações. Objetivo: Analisar um programa de saúde auditiva infantil com relação à adesão à triagem auditiva e procedimentos de diagnóstico. Metodologia: Pesquisa de caráter transversal com análise quantitativa. Realizado em três etapas: 1ª etapa: triagem auditiva de neonatos de alojamento conjunto; 2ª etapa: reteste das falhas; 3ª etapa: diagnóstico audiológico dos lactentes que falharam nas etapas anteriores com a utilização do Potencial Evocado Auditivo de Estado Estável (PEAEE) em conjunto com o Potencial Evocado Auditivo de Tronco Encefálico (PEATE). Resultados: Em 2019, 1.898 neonatos foram triados e destes, 287 (15.2%) falharam na primeira testagem em pelo menos uma orelha. Um total de 197 (10.3%) foram retestados e 14 (0,73%) falharam em pelo menos uma orelha. Dez (0,52%) neonatos retornaram para diagnóstico compondo uma amostra homogênea de neonatos nascidos a termo. Um neonato apresentou perda auditiva unilateral. O tempo necessário para coleta de dados no PEAEE foi de 20 minutos. Conclusão: O PEAEE pode ser considerado uma alternativa a ser utilizado na bateria de testes na avaliação audiológica infantil, juntamente com outros procedimentos, utilizando-se do princípio de verificação cruzada e adicionando uma informação valiosa, especialmente com relação às baixas frequências. (AU)


Introduction: Based on the need for audiological diagnosis and intervention as soon as possible in the life of a child with hearing loss, it is necessary to elaborate of hearing evaluation protocols with high efficiency, which provide the greatest amount of information. Aim: To analyze a pediatric hearing health program regarding their adherence to hearing screening, failure rates, and diagnostic procedures. Method: This is a cross-sectional, descriptive, quantitative study, and consisted of tree stages: Performed in three steps: 1st step: hearing screening of rooming-in neonates; 2nd stage: retest of failures; 3rd stage: audiological diagnosis of infants who failed in the previous stages using the Steady State Response (ASSR) together with the Brainstem Evoked Response Audiometry (BERA). Results: In 2019, 1,898 infants were submitted to the program, of whom 287 (15.2%) failed the screening in at least one of the ears. A total of 197 (10.3%) infants attended the retest and 14 (0.73%) failed the TOAE in at least one of the ears. Ten (0.52%) infants returned for diagnosis. The sample was homogeneously full-term children. One child showed unilateral HL. The average amount of time required to collect information in the ASSR was 20 minutes. Conclusion: For diagnosis, ASSR can be an alternative to be used in the battery of examinations in pediatric hearing assessment along with the other procedures, using the cross-check principle and adding valuable information, especially regarding the low frequencies. (AU)


Introducción: En base a la necesidad de diagnóstico audiológico e intervención lo antes posibles en la vida de un niño con pérdida auditiva, es necesario elaborar protocolos de evaluación auditiva de alta eficiencia, que proporcionan la mayor cantidad de información. Objetivo: Analizar un programa de salud auditiva infantil en cuanto a la adherencia al tamizaje auditivo, tasa de fracaso y procedimientos diagnósticos. Metodología: Investigación transversal con análisis cuantitativo, Realizado en tres pasos: 1er paso: tamizaje auditivo de los neonatos en alojamiento conjunto; 2ª etapa: retest de fallas; 3ª etapa: diagnóstico audiológico de los lactantes que fracasaron en las etapas anteriores utilizando el Potencial Evocado Auditivo de Estado Estacionario junto con el Potencial Evocado Auditivo de Tallo Cerebral. Resultados: Em 2019, se cribaron 1,898 neonatos y de estos, 287 (15,2%) no pasaron la primera prueba en al menos un oído. Un total de 197 (10,3) fueron reevaluados y 14 (0,73%) fallaron en al menos un oído. Diez (0,52%) neonatos regresaron para diagnóstico, conformando una muestra homogénea de neonatos a término, con una edad gestacional media de 39 semanas y dos días. Un neonato tuvo pérdida auditiva unilateral. El tiempo de recogida de los resultados en el ASSR fue de 20 min. Conclusión: Para el diagnóstico, la ASSR puede considerarse una alternativa para ser utilizada en la batería de pruebas en la evaluación audiológica infantil, junto con otros procedimientos, utilizando el principio de verificación cruzada y agregando información valiosa, especialmente en lo que se refiere a las bajas frecuencia. (AU)


Sujet(s)
Humains , Mâle , Femelle , Nouveau-né , Potentiels évoqués auditifs du tronc cérébral/physiologie , Dépistage néonatal/méthodes , Études transversales , Diagnostic précoce , Perte d'audition/diagnostic , Perte d'audition/étiologie
6.
Braz J Otorhinolaryngol ; 89(5): 101314, 2023.
Article de Anglais | MEDLINE | ID: mdl-37688911

RÉSUMÉ

OBJECTIVES: The degree of endolymphatic hydrops in Meniere's disease may be related to hearing loss. However, the results of prior studies have been inconsistent. We aimed to investigate the relationship between endolymphatic hydrops and hearing loss characteristics in Meniere's disease. METHODS: This study included 54 patients (62 ears) with Meniere's disease. Patients underwent three-dimensional real inversion recovery sequences for magnetic resonance imaging and pure-tone audiometry. Endolymphatic hydrops were assessed according to Gurkov's criteria (2011). Correlations between different degrees of endolymphatic hydrops and pure-tone audiometry, as well as staging, were analysed. RESULTS: Pure tone audiometry and staging were higher for vestibular endolymphatic hydrops complicated by cochlear ones than isolated cochlear or vestibular hydrops (both p < 0.05). There was no significant correlation between vestibular endolymphatic hydrops and pure-tonal audiometry or staging (all p > 0.05). The degree of hydrops in the middle turn was correlated with the mid-frequency hearing threshold (p < 0.05). The degree of cochlear hydrops was correlated with the audiometry, low-frequency hearing thresholds, mid-frequency hearing thresholds and staging (p < 0.01). CONCLUSION: The types and sites of endolymphatic hydrops based on a 3D-real inversion recovery sequence can be used to indicate the degree of hearing loss in patients with Meniere's disease. LEVEL OF EVIDENCE: Level III.


Sujet(s)
Surdité , Hydrops endolymphatique , Perte d'audition , Maladie de Ménière , Humains , Maladie de Ménière/complications , Maladie de Ménière/imagerie diagnostique , Hydrops endolymphatique/complications , Hydrops endolymphatique/imagerie diagnostique , Perte d'audition/étiologie , Audiométrie tonale , Oedème/complications , Imagerie par résonance magnétique/effets indésirables , Imagerie par résonance magnétique/méthodes
7.
Int J Pediatr Otorhinolaryngol ; 172: 111689, 2023 Sep.
Article de Anglais | MEDLINE | ID: mdl-37562319

RÉSUMÉ

OBJECTIVE: To verify the frequency of risk factors for hearing loss in newborns and their possible associations with universal neonatal hearing screening results before and during the COVID-19 pandemic. METHODS: Historical cohort study with data analysis of newborns attended in a reference hearing health service of the Unified Health System (SUS) between January 2017 and December 2021. RESULTS: Those born in 2020 and 2021 were 91% less likely to fail the screening than those born in 2017, 2018, and 2019; therefore, they had a lower percentage of referrals for a retest. There was a decrease in congenital syphilis (1.00%), decrease in HIV (0.95%), and an increase in toxoplasmosis (0.58%) and increase in rubella cases in 2021 in relation to 2017. Syphilis had lower frequency rates during the COVID-19 pandemic (2020-2021). CONCLUSION: Newborns born in the pandemic year compared to those born pre-pandemic showed a reduction in the presence of two risk indicators for hearing loss and, consequently, a lower chance of failing the UNHS and a lower percentage of referral for retest.


Sujet(s)
COVID-19 , Surdité , Perte d'audition , Nouveau-né , Humains , Enfant d'âge préscolaire , Pandémies , Études de cohortes , Dépistage néonatal/méthodes , COVID-19/complications , Perte d'audition/diagnostic , Perte d'audition/épidémiologie , Perte d'audition/étiologie , Surdité/complications , Tests auditifs/méthodes , Ouïe
8.
Braz J Otorhinolaryngol ; 89(5): 101300, 2023.
Article de Anglais | MEDLINE | ID: mdl-37579571

RÉSUMÉ

OBJECTIVES: To determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery. METHODS: A systematic review of the literature was performed without language or publication year restrictions in the MEDLINE/PubMed, EMBASE, Web of Science, LILACS/VHL and Cochrane Library databases. Studies that met the following criteria were included: 1) population: adults of both sexes who underwent radiosurgery for vestibular schwannoma treatment; 2) exposure: cochlear radiation; 3) outcome: hearing loss; 4) type of study: cohort. Two independent reviewers conducted the entire review process. The registration number in PROSPERO was CRD42020206128. RESULTS: From the 333 articles identified in the searches, seven were included after applying the eligibility criteria. There was no standardization as to how to measure exposure or outcome in the included studies, and most studies did not present sufficient data to enable meta-analysis. CONCLUSION: It was not possible to determine a cut-off point for high cochlear dose that could be considered a risk factor for hearing loss.


Sujet(s)
Surdité , Perte d'audition , Neurinome de l'acoustique , Radiochirurgie , Adulte , Femelle , Humains , Mâle , Surdité/chirurgie , Perte d'audition/étiologie , Perte d'audition/chirurgie , Neurinome de l'acoustique/radiothérapie , Neurinome de l'acoustique/chirurgie , Neurinome de l'acoustique/complications , Dose de rayonnement , Radiochirurgie/effets indésirables , Études rétrospectives , Résultat thérapeutique
9.
Distúrb. comun ; 35(2): 59932, 02/08/2023.
Article de Anglais, Portugais | LILACS | ID: biblio-1444680

RÉSUMÉ

Introdução: As infecções congênitas durante a gravidez são indicadores de risco para a deficiência auditiva. Objetivo: Verificar a frequência da deficiência auditiva nas crianças atendidas num serviço público com indicadores de risco de infecções congênitas. Métodos: Trata-se de um estudo transversal retrospectivo. A população do estudo foi de crianças de 0 a 3 anos atendidas no período de 2011 a 2019. Foi realizada consulta e análise no banco de dados da Instituição extraindo informações das crianças quanto à presença de infecção congênita relatada (citomegalovírus, herpes, rubéola, sífilis, toxoplasmose, HIV e Zika vírus) e o diagnóstico audiológico completo. A amostra deste estudo foi constituída por 558 crianças e foram analisadas a presença de coocorrência entre as infecções ou de outros indicadores de risco para a deficiência auditiva. Realizou-se análise descritiva para estabelecer a frequência da deficiência auditiva em relação a cada infecção congênita isolada ou associada a outros indicadores de risco. Resultados: 14,40% das crianças apresentavam o relato de infecção congênita isolada ou em combinação com outro IRDA. A frequência da deficiência auditiva foi de 1,25%, com a presença da perda auditiva sensorioneural em seis crianças (85,71%) e uma perda auditiva do tipo condutiva (14,29%), das quais seis foram bilaterais (85,71%) e uma unilateral (14,29%). Esta frequência de deficiência auditiva foi relacionada ao histórico de citomegalovírus (57,14%), seguido de toxoplasmose (28,57%) e rubéola com Zika vírus (14,29%). Conclusão: A frequência do diagnóstico de deficiência auditiva foi de 1,25% nas crianças com relato de infecções congênitas. (AU)


Introduction: Congenital infections during pregnancy are risk indicators for hearing loss. Purpose: To verify the frequency of hearing loss in children attended at the public service with risk indicators for congenital infections. Methods: This is a retrospective cross-sectional study. The population consisted of children aged 0 to 3 years attended in the period from 2011 to 2019. Consultation and analysis were carried out in the Institution's database, extracting information from the children regarding the presence of reported congenital infection (cytomegalovirus, herpes, rubella, syphilis, toxoplasmosis, HIV and Zika virus) and the complete audiological diagnosis. The sample of this study consisted of 558 children and the presence of co-occurrence between infections or other risk indicators for hearing loss was analyzed. Descriptive analysis was performed to establish the frequency of hearing loss in relation to each congenital infection isolated or associated with other risk indicators. Results: 14.40% of the children had a report of isolated congenital infection or in combination with another risk indicator. The frequency of hearing loss was 1.25%, with sensorineural hearing loss in six children (85.71%) and a conductive hearing loss (14.29%), of which six were bilateral (85, 71%) and one unilateral (14.29%). This frequency of hearing loss was related to the history of cytomegalovirus (57.14%), followed by toxoplasmosis (28.57%) and rubella with zika virus (14.29%). Conclusion: The frequency of diagnosis of hearing loss was 1.25% in children with reports of congenital infections. (AU)


Introducción: Las infecciones congénitas durante el embarazo son indicadores de riesgo de hipoacusia. Propósito: Verificar la frecuencia de hipoacusia en niños atendidos en el servicio público con indicadores de riesgo de infecciones congénitas. Métodos: Se trata de un estudio transversal retrospectivo. La población de estudio estuvo constituida por los niños de 0 a 3 años atendidos en el periodo de 2011 a 2019. Se realizó consulta y análisis en la base de datos de la Institución, extrayéndose información de los niños en cuanto a la presencia de infección congénita reportada (citomegalovirus, herpes, rubéola, sífilis, toxoplasmosis, VIH y virus Zika) y el diagnóstico audiológico completo. La muestra de este estudio estuvo constituida por 558 niños y se analizó la presencia de coocurrencia entre infecciones u otros indicadores de riesgo de hipoacusia. Se realizó un análisis descriptivo para establecer la frecuencia de hipoacusia con relación a cada infección congénita aislada o asociada a otros indicadores de riesgo. Resultados: El 14,40% de los niños tenían reporte de infección congénita aislada o en combinación con otro indicador de riesgo. La frecuencia de hipoacusia fue del 1,25%, con hipoacusia neurosensorial en seis niños (85,71%) y hipoacusia conductiva (14,29%), de los cuales seis fueron bilaterales (85,71%) y uno unilateral (14,29%). Esta frecuencia de hipoacusia se relacionó con el antecedente de citomegalovirus (57,14%), seguido de toxoplasmosis (28,57%) y rubéola con virus zika (14,29%). Conclusión: La frecuencia de diagnóstico de hipoacusia fue de 1,25% en niños con reporte de infecciones congénitas. (AU)


Sujet(s)
Humains , Nourrisson , Enfant d'âge préscolaire , Perte d'audition/étiologie , Malformations , Prévalence , Études transversales , Études rétrospectives , Facteurs de risque , Perte d'audition/épidémiologie
10.
Distúrb. comun ; 35(1): e58742, 01/06/2023.
Article de Portugais | LILACS | ID: biblio-1436216

RÉSUMÉ

Introdução: A integridade do sistema auditivo é essencial para o desenvolvimento das habilidades auditivas e aquisição da linguagem da criança. Considerando a alta prevalência de perda auditiva em recém-nascidos, devido a infecções congênitas que ocorrem durante a gestação, há a necessidade de investigar os efeitos da Covid-19 na audição do RN. Objetivo: Verificar a associação entre perda auditiva em neonatos de gestantes diagnosticadas com COVID-19. Estratégia de Pesquisa: A busca de artigos científicos foi realizada nas bases de dados Medline (Pubmed), LILACS, SciELO, Scopus, Web of Science e Bireme sem restrição de idioma, período e localização. Para complementar e evitar viés de risco foi realizada uma busca por literatura cinzenta no Google Acadêmico. Critérios de Seleção: A revisão sistemática foi conduzida de acordo com as recomendações do Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) e incluiu estudos que pontuaram ≥ 6 pontos de acordo com o protocolo de pontuação qualitativa proposto por Pithon et al. (2015). Análise dos dados: Os artigos elegíveis foram analisados e quantificados seguindo os critérios propostos no presente estudo com juízes cegos nas etapas de recuperação. Resultados: foram recuperados 29 artigos com potencial de inclusão, dos quais 6 responderam à questão norteadora com potencial de elegibilidade. Quatro estudos encontrados não detectaram associação entre infecção materna por COVID-19 e perda auditiva congênita. Conclusão: A infecção por COVID-19 durante a gravidez não parece ser fator de risco para perda auditiva congênita e não foram verificadas alterações auditivas impactantes que comprometessem estes neonatos por infecção vertical. (AU)


Introduction: The integrity of the auditory system is essential for children to develop auditory skills and acquire language. Considering the high prevalence of hearing loss in newborns due to congenital infections that occur during pregnancy, there is a need to investigate the effects of COVID-19 on newborns' hearing. Objective: To verify the association between hearing loss in newborns whose mothers had been diagnosed with COVID-19 during pregnancy. Research Strategy: Scientific articles were searched in the MEDLINE (PubMed), LILACS, SciELO, Scopus, Web of Science, and BIREME databases, without restrictions on the language, time, and place of study. The grey literature was also searched in Google Scholar to complement the sample and avoid risk bias. Selection Criteria: The systematic review followed the recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and included studies that scored ≥ 6 points according to the protocol for qualitative scoring proposed by Pithon et al. (2015). Data analysis: It was performed using a specific form for systematic reviews prepared by two researchers in Excel®. Results: 29 potentially eligible articles were retrieved, six of which answered the research question. Four studies did not detect an association between maternal COVID-19 infection and congenital hearing loss. Conclusion: COVID-19 infection during pregnancy does not seem to be a risk factor for congenital hearing loss and there were no impacting hearing changes due to vertical infection that might affect these newborns. (AU)


Introducción: La integridad del sistema auditivo es fundamental para el desarrollo de las habilidades auditivas y la adquisición del lenguaje de los niños. Considerando la alta prevalencia de hipoacusia (HL) en recién nacidos (RN), debido a infecciones congénitas que ocurren durante el embarazo, surge la necesidad de investigar los efectos del Covid-19 en la audición del recién nacido. Objetivo: Verificar la asociación entre hipoacusia en neonatos de gestantes diagnosticadas con COVID-19. Estrategia de investigación: La búsqueda de artículos científicos se realizó en las bases de datos Medline (Pubmed), LILACS, SciELO, Scopus, Web of Science y Bireme, sin restricción de idioma, período y ubicación. Para complementar y evitar sesgos de riesgo, se realizó una búsqueda de literatura gris en Google Scholar. Criterios de selección: La revisión sistemática se realizó de acuerdo con las recomendaciones de los Elementos de información preferidos para revisiones sistemáticas y metanálisis (PRISMA). Los estudios que obtuvieron una puntuación ≥ 6 puntos según el protocolo de puntuación cualitativa propuesto por Pithon et al. (2015). Análisis de datos: Se realizó mediante un formulario específico para revisión sistemática elaborado por dos investigadores del Programa Excel®. Resultados: se recuperaron 29 artículos con potencial de inclusión, de los cuales 6 respondieron a la pregunta orientadora Cuatro estudios encontrados no detectaron una asociación entre la infección materna por COVID-19 y la pérdida auditiva congénita. Conclusión: La infección por COVID-19 durante el embarazo no parece ser un factor de riesgo para la pérdida auditiva congénita y no hubo cambios auditivos impactantes que pudieran comprometer a estos recién nacidos debido a la infección vertical. (AU)


Sujet(s)
Humains , Nouveau-né , Transmission verticale de maladie infectieuse , COVID-19 , Perte d'audition/congénital , Complications infectieuses de la grossesse , Facteurs de risque , Perte d'audition/étiologie
12.
Rev Med Chil ; 151(9): 1125-1142, 2023 Sep.
Article de Espagnol | MEDLINE | ID: mdl-39093150

RÉSUMÉ

INTRODUCTION: Various studies have linked lifestyle and metabolic diseases to hearing loss. Research on metabolic syndrome has reported a higher prevalence of hearing loss in individuals with it than those without it. OBJECTIVES: To conduct a systematic review and meta-analysis summarizing the risk of hearing loss in people diagnosed with metabolic syndrome or diseases versus those without metabolic syndrome. METHODS: Following the PRISMA recommendations for systematic review and meta-analysis, searches were conducted in PubMed, Web of Science, and SciELO. Only cross-sectional studies associate hearing loss with obesity, hypertension, Diabetes, Dyslipidemia, Cholesterol, metabolic syndrome, and Hyperglycemia. The odds ratios (OR) with their 95% confidence intervals (CI) of the studies were pooled in a random effects model using the Mantel-Haenszel method. Using Rev Manager 5.1, OR heterogeneity was assessed using the I2 and Q statistics. RESULTS: Twenty-eight observational studies were included in the quantitative analysis. The OR for prevalent hearing loss was 1.27 (95% CI 1.07, 1.51) in obesity, 1.97 (95% CI 1.51, 2.57) in diabetes, 1.53 (95% CI 1.31, 1.79) in hypertension, and 4.22 (95% CI 1.74, 10.20) for metabolic syndrome. CONCLUSIONS: The findings suggest that both metabolic syndrome and some diseases, such as obesity, diabetes, and hypertension, could be associated with the risk of hearing loss, and control of these diseases could reduce this risk.


Sujet(s)
Perte d'audition , Syndrome métabolique X , Humains , Syndrome métabolique X/épidémiologie , Syndrome métabolique X/complications , Perte d'audition/épidémiologie , Perte d'audition/étiologie , Facteurs de risque , Obésité/complications , Obésité/épidémiologie , Prévalence , Hypertension artérielle/épidémiologie , Hypertension artérielle/complications
13.
Int J Pediatr Otorhinolaryngol ; 162: 111329, 2022 Nov.
Article de Anglais | MEDLINE | ID: mdl-36198231

RÉSUMÉ

OBJECTIVE: To identify the prevalence of hearing impairment and associated risk factors in children living with human immunodeficiency virus (HIV) in Haiti. METHODS: A validated smartphone-based platform with pure-tone audiometry was used to screen 341 HIV-infected children for hearing impairment in Port-au-Prince, Haiti from March 2019 to September 2020. If screening was failed, a more comprehensive pure-tone audiometric evaluation was administered. Demographic, otologic, and HIV-related data were obtained through caregiver surveys and medical charts. Statistical analysis included univariate and multivariate logistic regression. RESULTS: Sixty (18%) of 341 HIV-infected children (ages 7-18 years) had hearing impairment. Of those failing their hearing assessment, 17 (28%) had moderate and 5 (8%) had severe or profound hearing loss. Hearing impairment was associated with frequent ear infections (OR 3.37; 95% CI 1.76-6.46; p < 0.001) and family history of hearing loss (OR 5.12; 95% CI 2.14-12.23; p = 0.001) but not viral load (OR 1.00; 95% CI 0.73-1.02; p = 0.28) or antiretroviral therapy duration (OR 0.96; 95% CI 0.79-1.17; p = 0.66). Only 35% of caregivers correctly perceived their child's hearing loss. CONCLUSIONS: Hearing impairment occurs at a higher prevalence in HIV-infected children in Haiti than what is expected for those living without HIV. Frequent ear infections were significantly associated with hearing loss while antiretroviral therapy duration was not. Despite their potential ototoxicity, antiretroviral therapies should be continued and may decrease incidence of otitis media. Low caregiver perception of hearing loss emphasizes the need for routine hearing screening for HIV-infected children.


Sujet(s)
Surdité , Infections à VIH , Perte d'audition , Otite , Adolescent , Antirétroviraux , Audiométrie tonale , Enfant , Surdité/complications , Infections à VIH/complications , Infections à VIH/traitement médicamenteux , Infections à VIH/épidémiologie , Haïti/épidémiologie , Perte d'audition/diagnostic , Perte d'audition/épidémiologie , Perte d'audition/étiologie , Humains , Otite/complications
14.
Bol Med Hosp Infant Mex ; 79(4): 206-214, 2022.
Article de Anglais | MEDLINE | ID: mdl-36100214

RÉSUMÉ

Hearing loss is the most frequent sensory disorder, with an incidence of 1:1500 live newborns. In more than 50% of patients, it is associated with a genetic cause, while in up to 30% of cases, it is related to syndromic entities. We performed a literature review of studies on congenital hearing loss of genetic origin in the Mexican population. We identified eight reports that showed that the pathogenic variants most frequently associated with hearing loss are related to the GJB2 gene, although in a low percentage (3%). Other mutations were identified in the GJB6, SLC26A4, or CHD23 genes. On this basis, a possible diagnostic strategy in Mexican patients with hearing loss is to consider an initial screening of these three genes. If these genes were negative for pathogenic variants, the following steps would be to consider second-generation sequencing analysis focused on panels of genes associated with hearing loss, isolated or syndromic, and if necessary, to perform exome or whole-genome analysis. Establishing an etiologic cause is critical in clinically evaluating patients with congenital hearing loss and their families. It can help determine rehabilitation strategies, such as hearing aids or cochlear implants and provide information on disease progression and genetic counseling in this population.


La pérdida auditiva es la alteración sensorial más frecuente, con una incidencia de 1:1500 recién nacidos vivos. En más del 50% de los pacientes se asocia con una causa genética, mientras que en más del 30% de los casos se asocia con entidades sindrómicas. Se llevó a cabo una revisión de la literatura de las investigaciones sobre la pérdida auditiva congénita de origen genético en la población mexicana. Se identificaron ocho reportes en los que se demostró que las variantes patogénicas más frecuentemente asociadas con pérdida auditiva se encuentran en el gen GJB2, aunque en un porcentaje bajo (3%). Se identificaron otras mutaciones en los genes GJB6, SLC26A4 o CHD23. Con base en esta información, una posible estrategia diagnóstica en pacientes mexicanos con pérdida auditiva es considerar un primer paso en el tamiz diagnóstico con los tres genes mencionados. Si estos genes fueran negativos para variantes patogénicas, el siguiente paso sería considerar el análisis por secuenciación de segunda generación enfocado en paneles de genes asociados con pérdida auditiva, tanto aislada como sindrómica, y en caso necesario, realizar el análisis del exoma o del genoma completo. Establecer una causa etiológica es un componente crítico en la evaluación clínica de los pacientes con pérdida auditiva congénita, ya que puede ayudar a determinar las estrategias de manejo y rehabilitación, como el uso de auxiliares auditivos o implantes cocleares, proporcionar información sobre la progresión de la enfermedad y dar asesoramiento genético en esta población.


Sujet(s)
Implantation cochléaire , Surdité , Perte d'audition , Connexine-26/génétique , Connexines/génétique , Surdité/épidémiologie , Surdité/génétique , Perte d'audition/étiologie , Perte d'audition/génétique , Humains , Nouveau-né
15.
Surg Radiol Anat ; 44(7): 1041-1044, 2022 Jul.
Article de Anglais | MEDLINE | ID: mdl-35842486

RÉSUMÉ

PURPOSE: To describe an anatomical variant that should be consider in patients with hearing loss. METHODS: An 8-year-old girl underwent to temporal bone computed tomography for the evaluation of bilateral conductive hearing loss and further assessment of possible enlarged vestibular aqueduct or high jugular bulb on brain magnetic resonance imaging (MRI). RESULTS: CT of temporal bone showed a cystic cavity with bony sclerotic margins extending from the right jugular foramen to the vestibular aqueduct. Bony dehiscence of the jugular foramen with the right carotid canal was also noted. On brain MRI, there was no evidence of enlargement of the endolymphatic duct and sac on T2 thin-section gradient echo sequence. Time of flight MR angiography did not show arterial flow in the cavity. Contrast enhanced MR venography confirmed the presence of a high right jugular bulb with a diverticulum extending into the vestibular aqueduct due to jugular bulb-vestibular aqueduct dehiscence. CONCLUSION: Knowledge of high jugular bulb-vestibular aqueduct dehiscence is important in the assessment of patients with otologic symptoms such as vertigo, tinnitus and hearing loss.


Sujet(s)
Surdité , Diverticule , Perte d'audition , Aqueduc du vestibule , Enfant , Diverticule/diagnostic , Diverticule/imagerie diagnostique , Femelle , Perte d'audition/diagnostic , Perte d'audition/étiologie , Perte d'audition/anatomopathologie , Humains , Veines jugulaires/imagerie diagnostique , Os temporal/anatomopathologie , Aqueduc du vestibule/imagerie diagnostique , Aqueduc du vestibule/anatomopathologie
16.
Braz J Otorhinolaryngol ; 88 Suppl 3: S95-S102, 2022.
Article de Anglais | MEDLINE | ID: mdl-35729043

RÉSUMÉ

OBJECTIVE: Previous studies have shown that hearing function is also vulnerable to the effects of diabetes mellitus which can be shown by brainstem auditory evoked potential and distortion product otoacoustic emission recordings. This study aimed to investigate the changes of brainstem auditory evoked potential and distortion product otoacoustic emission in hyperglycemia and whether there is a relationship between reactive oxygen substances production and hearing deterioration in the rat model. METHODS: 25 streptozotocin induced diabetic rats were divided into three groups: control, high blood glucose, and diabetes mellitus. Brainstem auditory evoked potential and distortion product otoacoustic emission were recorded, and thiobarbituric acid reactive substances levels were measured in the brainstem tissue. RESULTS: At 8 kHz, the latencies of I, II, III, IV, and V brainstem auditory evoked potential waves in high blood glucose and diabetes mellitus groups were elongated, at 16 kHz, only these wave latencies of the diabetes mellitus group were prolonged compared with the control group. A significant decrease was also found in distortion product otoacoustic emission amplitudes at 4, 6, 8, and 10 kHz in the high blood glucose and diabetes mellitus groups compared to the control group. There was a significant increase in thiobarbituric acid reactive substances values due to the increase in blood glucose levels in the high blood glucose and diabetes mellitus groups compared to the control group. CONCLUSION: These results suggested that high blood glucose levels may cause hearing impairment not only in the diabetic state but also in the period of hyperglycemia before the onset of manifest diabetes mellitus and reactive oxygen substances may play an important role in the pathophysiology of diabetes mellitus. We suggest that regulating high glucose levels even before the onset of manifest diabetes mellitus may prevent hazardous effects on hearing function. LEVEL OF EVIDENCE: Level 3.


Sujet(s)
Surdité , Diabète expérimental , Perte d'audition , Hyperglycémie , Rats , Animaux , Glycémie , Diabète expérimental/complications , Substances réactives à l'acide thiobarbiturique , Émissions otoacoustiques spontanées/physiologie , Perte d'audition/étiologie , Potentiels évoqués auditifs du tronc cérébral/physiologie , Hyperglycémie/complications , Oxygène
17.
Rev. otorrinolaringol. cir. cabeza cuello ; 82(2): 184-189, jun. 2022. ilus
Article de Espagnol | LILACS | ID: biblio-1389854

RÉSUMÉ

Resumen La fractura aislada del mango del martillo es una entidad clínica poco habitual, pero frecuentemente subdiagnosticada. Lo fundamental es la sospecha clínica. El diagnóstico se confirma con la otoscopia neumática o la otomicroscopia con maniobra de Valsalva, en la cual se observa una movilidad anormal del mango del martillo. El rasgo de fractura se puede demostrar con tomografía computada de alta resolución o cone beam. Existen diferentes opciones de tratamiento como interposición de cartílago o uso de prótesis de reemplazo osicular así como cemento óseo. El cemento ionomérico vidrioso autocurado, muy utilizado en odontología, se ha usado en distintas cirugías otológicas con buenos resultados y biocompatibilidad. A nuestro saber no se ha usado en esta patología por lo que presentamos esta serie de tres casos en los cuales se ha usado esta novedosa técnica con buenos resultados clínicos.


Abstract Isolated fracture of the manubrium of the malleus is a rare clinical entity. Clinical suspicion is paramount. The usual clinical presentation is acute otalgia followed by tinnitus and fluctuating hearing loss after a brisk introduction and withdrawal of a finger into the external auditory canal. On physical examination, the eardrum looks normal on otoscopy. Only in pneumatic otoscopy or otomicroscopy with Valsalva an abnormal motility of the manubrium could bee seen. High-resolution computed tomography (CT) or cone beam CT is able to show the fracture line. Several treatment options have been proposed, such as interposition of bone or cartilage between the manubrium and the incus, total or partial ossicular replacement prosthesis; and the use of bone cement. Glass ionomer luting cement, with wide use in dentistry, has been used in several otological procedures with good biocompatibility and results, however, to our best knowledge, it has not been used to repair this type of fractures, so we present this novel material in three cases.


Sujet(s)
Humains , Mâle , Femelle , Adulte , Adulte d'âge moyen , Cément dentaire , Fractures osseuses/imagerie diagnostique , Malléus/chirurgie , Malléus/traumatismes , Ciments osseux , Manoeuvre de Vasalva , Otoscopie , Conduit auditif externe , Otalgie , Tomodensitométrie à faisceau conique , Perte d'audition/étiologie
18.
Rev Saude Publica ; 56: 28, 2022.
Article de Anglais | MEDLINE | ID: mdl-35476106

RÉSUMÉ

OBJECTIVE: To compare the incidence of hearing loss among adults stratified by the occurrence of hypertension, and to investigate the association between hypertension and hearing loss. METHODS: Longitudinal observational study, part of the Estudo Longitudinal da Saúde do Adulto (ELSA-Brasil, Longitudinal Study on Adult's Health). Data from the first and second waves were analyzed, including information from audiological assessment and general health of the subjects. As outcome, we considered the presence of hearing loss (hearing thresholds above 25 dBHL at frequencies from 500 Hz to 8 kHz) and, as exposure variable, hypertension (report of medical diagnosis of hypertension; and/or use of drugs to treat hypertension; and/or pressure systolic blood pressure ≥ 140 mmHg; or diastolic blood pressure ≥ 90 mmHg). As covariables for adjustment were considered: sex, age, education, race / ethnicity, income, smoking, diabetes, and occupational exposure to noise. Poisson regression analysis was conducted, estimating the crude and adjusted relative risks, with 95% confidence intervals, in order to assess the factors associated with hearing loss. RESULTS: In crude analyses, the incidence of hearing loss was higher for subjects with hypertension (9.7% versus 5.4%). The crude relative risks for hearing loss was almost double (1.93; 95%CI: 1.10-3.39) for subjects with hypertension in the right ear. In the adjusted analyses, the relative risks was not significant for the hypertension variable (1.42; 95%CI: 0.75-2.67). Being 60 years or older (RR: 5.41; 95%CI: 2.79-10.50) showed a statistically significant association with hearing loss, indicating that older adults have higher relative risks for hearing loss. CONCLUSION: In the adjusted analyses controlled for multiple risk factors there was no association between hypertension and hearing loss. The dichotomous variable age (being 60 years or older), on the other hand, has shown a significant association with hearing loss.


Sujet(s)
Perte d'audition , Hypertension artérielle , Sujet âgé , Brésil/épidémiologie , Perte d'audition/épidémiologie , Perte d'audition/étiologie , Humains , Hypertension artérielle/épidémiologie , Hypertension artérielle/étiologie , Incidence , Études longitudinales , Adulte d'âge moyen
19.
Braz J Otorhinolaryngol ; 88 Suppl 3: S73-S80, 2022.
Article de Anglais | MEDLINE | ID: mdl-35331657

RÉSUMÉ

OBJECTIVE: Diabetes Mellitus (DM) causes an increase in oxidative stress that leads to deterioration in auditory functions. Astaxanthine (AST) is known to have strong antioxidant effects. In this study, the aim is to investigate the effect of AST against hearing loss that is due to DM. METHODS: This study is an experimental animal study. The study was designed in four groups with 8 animals (n = 8) in each group. The groups were as follows; Control Group (CNT), Diabetic Group (DM), AST applied diabetic group (DM+AST), and AST applied non-diabetic group (AST). Streptozotocin was applied in rats to induce DM. AST was administered by oral gavage. Auditory Brainstem Responses (ABR) and Distortion Product Otoacoustic Emissions (DPOAE) tests were performed on several days of the study. At the end of the study, pro-inflammatory cytokine levels were analyzed in cochlear tissue samples, and Glutathione Peroxidase (GPx), Superoxide Dismutase (SOD), Catalase (CAT) and Malondialdehyde (MDA) levels were measured. RESULTS: When the findings obtained in the ABR and DPOAE tests in the DM group, it was observed that there was a significant deterioration in the hearing sense. This deterioration was not observed in the DM+AST group. In the DM group, GPx, SOD and CAT levels decreased and MDA levels increased in blood and cochlear tissue. Compared to the DM group, it was noted that antioxidant enzyme levels increased and MDA levels decreased in the DM+AST group. Cochlear tissue pro-inflammatory cytokine levels, which increased with DM, were significantly decreased in the DM+AST group. CONCLUSION: Even though the effects of AST were investigated in a diabetic experimental animal model, if this molecule is proven to be effective in diabetic humans, it can be considered an adjunct therapeutic option with its antioxidant effects. LEVEL OF EVIDENCE: The level of evidence of this article is 5. This article is an experimental animal and laboratory study.


Sujet(s)
Diabète expérimental , Perte d'audition , Xanthophylles , Animaux , Rats , Antioxydants/pharmacologie , Antioxydants/usage thérapeutique , Cytokines , Diabète expérimental/complications , Diabète expérimental/traitement médicamenteux , Glutathione peroxidase/métabolisme , Perte d'audition/traitement médicamenteux , Perte d'audition/étiologie , Perte d'audition/prévention et contrôle , Superoxide dismutase , Xanthophylles/usage thérapeutique
20.
Brain ; 145(3): 858-871, 2022 04 29.
Article de Anglais | MEDLINE | ID: mdl-35136969

RÉSUMÉ

Susac syndrome is a disorder thought to be mediated by an autoimmune response towards endothelial cells, leading to a characteristic clinical triad of encephalopathy, visual disturbances due to branch arterial occlusions and sensorineural hearing impairment. Although it is a rare disease, three reasons make it important. First, given its variable presentation, Susac syndrome is underdiagnosed. Second, it is considered an important differential diagnosis in different neurological, psychiatric, ophthalmological and hearing disorders, and consequently is frequently misdiagnosed. Third, in many cases, Susac syndrome is diagnosed and treated late, with significant irreversible sequelae including dementia, blindness and hearing loss. Neuropathology findings derived from both Susac syndrome patient tissue and novel transgenic mouse models indicate cytotoxic CD8+ T cells adhere to microvessels, inducing endothelial cell swelling, vascular narrowing and occlusion, causing microinfarcts. Anti-endothelial cell antibodies are present in serum in 25% of Susac syndrome patients, but it is unclear whether they are aetiologically related to the disease, or an epiphenomenon. The clinical triad comprising encephalopathy, branch arterial occlusions, and sensorineural hearing impairment is considered pathognomonic, although great variability is found in presentation and natural course of disease. At first evaluation, only 13-30% of patients exhibit the full clinical triad, making diagnosis difficult. Retinal fluorescein angiography, optic coherence tomography, MRI and tonal audiometry are helpful methods for diagnosing and monitoring disease activity during treatment. By contrast, there are no reliable objective immune markers to monitor disease activity. Immunosuppression is the current treatment, with high-dose corticosteroid therapy as the mainstay, but additional therapies such as intravenous immunoglobulins, cyclophosphamide, rituximab and mycophenolate mofetil are often necessary, because the disease can be devastating, causing irreversible organ damage. Unfortunately, low rates of disease, variability in presentation and paucity of objective biomarkers make prospective controlled clinical trials for Susac syndrome treatment difficult. Current immunosuppressive treatments are therefore based on empirical evidence, mainly from retrospective case series and expert opinion. In this review, we draw attention to the need to take consider Susac syndrome in the differential diagnosis of different neurological, psychiatric, ophthalmological and hearing disorders. Furthermore, we summarize our current knowledge of this syndrome, in reference to its pathophysiology, diagnosis and management, emphasizing the need for prospective and controlled studies that allow a better therapeutic approach.


Sujet(s)
Encéphalopathies , Perte d'audition , Syndrome de Susac , Animaux , Encéphalopathies/diagnostic , Diagnostic différentiel , Cellules endothéliales/anatomopathologie , Troubles de l'audition/diagnostic , Perte d'audition/diagnostic , Perte d'audition/étiologie , Humains , Imagerie par résonance magnétique , Souris , Études prospectives , Études rétrospectives , Syndrome de Susac/traitement médicamenteux , Syndrome de Susac/thérapie
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