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El síndrome de Vogt-Koyanagi-Harada (VKH) es una rara enfermedad granulomatosa multisistémica caracterizada por aparición de panuveítis grave bilateral y desprendimiento seroso de retina; puede acompañarse de un amplio espectro de síntomas extraoculares como los auditivos, y la afección más frecuente es la hipoacusia neurosensorial. Su etiología se reconoce como respuesta autoinmune mediada por células T contra antígenos de melanocitos presentes en coroides, meninges, cóclea y piel. Asimismo, factores genéticos del huésped se han identificado como predisponentes para su aparición, y es la presencia del alelo HLA-DR4, en particular el subtipo HLA-DRB1 0405, el más estudiado hasta la fecha. El tratamiento se basa en administración de corticosteroides sistémicos en dosis altas, sin embargo, es escasa la evidencia que evalúa específicamente la eficacia de estos medicamentos sobre sus manifestaciones audiovestibulares. Este artículo expone un caso de síndrome de VKH con compromiso auditivo concomitante y realiza una breve revisión narrativa de la literatura.
Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic granulomatous disease, characterized by severe bilateral panuveitis and serous retinal detachment; it can be associated with a wide spectrum of extraocular symptoms, such as auditory symptoms, and the most common condition is sensorineural hearing loss. Its etio-logy is recognized as a T-cell-mediated autoimmune response against melanocyte antigens present in the choroid, meninges, cochlea, and skin. Likewise, host genetic factors have been identified as predisposing for its development, specifically the pre-sence of the HLA-DR4 allele, the HLA-DRB1 0405 subtype is the most studied up to date. Treatment is based on the administration of high doses of systemic corticos-teroids, however, there is not much evidence that specifically evaluates the efficacy of these medications on their audiovestibular manifestations. This article presents a clinical case of VKH syndrome with concomitant hearing impairment and carries out a short narrative review of the literature.
Subject(s)
Humans , Male , FemaleABSTRACT
Abstract Objective Serum uric acid is proven to be associated with chronic hearing loss, but its effect on Sudden Sensorineural Hearing Loss (SSNHL) is unclear. This study aims to evaluate the prognostic values of serum uric acid levels in SSNHL patients. Methods The clinical records of SSNHL patients were retrospectively reviewed. Patients were divided into different groups based on hearing recovery and audiogram type, and uric acid levels were compared. Based on uric acid levels, patients were categorized into normouricemia and hyperuricemia groups, and clinical features and hearing recovery were evaluated. Univariate and multivariate analyses were performed to identify prognostic factors. Results In total, 520 SSNHL patients were included in this study, including 226 females and 294 males. In female patients, 186 patients were included in the normouricemia group, and 40 patients were enrolled in the hyperuricemia group. Significant differences were observed in uric acid levels, Total Cholesterol (TC), rate of complete recovery, and slight recovery between the two groups. In male patients, 237 subjects were categorized into the normouricemia group, and 57 patients were included in the hyperuricemia group. The rate of complete recovery and slight recovery was lower in the hyperuricemia group compared to the normouricemia group. All patients were further divided into good recovery and poor recovery groups based on hearing outcomes. The uric acid levels, initial hearing threshold, rate of hyperuricemia, and TC were lower in the good recovery group than the poor recovery group both in female and male patients. Binary logistic regression results showed that uric acid levels, initial hearing threshold, and hyperuricemia were associated with hearing recovery. Conclusion Hyperuricemia might be an independent risk factor for hearing recovery in SSNHL patients. Serum uric acid and initial hearing threshold possibly affected the hearing outcome in males and females with SSNHL. Level of evidence Level 4.
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Abstract Objectives: To review and provide evidence-based recommendations for the diagnosis and treatment of otosclerosis. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on otosclerosis were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: 1) Diagnosis - audiologic and radiologic; 2) Treatment - hearing AIDS, pharmacological therapy, stapes surgery, and implantable devices - bone-anchored devices, active middle ear implants, and Cochlear Implants (CI). Conclusions: The pathophysiology of otosclerosis has not yet been fully elucidated, but environmental factors and unidentified genes are likely to play a significant role in it. Women with otosclerosis are not at increased risk of worsening clinical condition due to the use of contraceptives or during pregnancy. Drug treatment has shown little benefit. If the patient does not want to undergo stapedotomy, the use of hearing aids is well indicated. Implantable systems should be indicated only in rare cases, and the CI should be indicated in cases of profound deafness.
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Abstract Introduction Metabolic syndrome (MetS) and its associated components were reported as a possible cause of inner ear dysfunction. However, research about the influence of cardiovascular risk factors on hearing thresholds are conducted mainly in adult patients. Objective The aim of the present study was to investigate auditory function in adolescents with MetS compared with healthy controls. Methods One hundred adolescents with metabolic syndrome and 200 sex- and age-matched controls were recruited from a university pediatric endocrine clinic from May 2018 to July 2020. Hearing loss was defined as hearing level ≥ 15 dB at speech frequency (SFHL) or high frequency (HFHL) in one or both ears. A multivariable conditional logistic regression analysis examined the correlation between MetS components and several important demographic characteristics, and hearing loss. Results A total of 165 (55.0%) boys and 135 (45.0%) girls participated in this study. The rates of SFHL and HFHL in adolescents with MetS were 32.0% and 51.0%, respectively. Those values for controls were 5.0% and 15.5%, respectively. The regression analysis showed high triglycerides as a significant predictor for SFHL (odds ratio 10.87; 95% confidence interval: 1.98, 59.74). Neither predictor of interest was significant for HFHL. Conclusion Hypertriglyceridemia may be an important factor in the pathogenesis of SFHL. However, the strength of the association was not significant with a wide confidence interval. Also, we were unable to find an association between predictors and HFHL with the current sample size. Larger and prospective studies are recommended.
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Objective@#Recent advances in epigenetic studies continue to reveal novel mechanisms of gene regulation and control, however little is known on the role of epigenetics in sensorineural hearing loss (SNHL) in humans. We aimed to investigate the methylation patterns of two regions, one in RB1 and another in GJB2 in Filipino patients with SNHL compared to hearing control individuals. @*Methods@#We investigated an RB1 promoter region that was previously identified as differentially methylated in children with SNHL and lead exposure. Additionally, we investigated a sequence in an enhancer-like region within GJB2 that contains four CpGs in close proximity. Bisulfite conversion was performed on salivary DNA samples from 15 children with SNHL and 45 unrelated ethnically-matched individuals. We then performed methylation-specific real-time PCR analysis (qMSP) using TaqMan® probes to determine percentage methylation of the two regions. @*Results@#Using qMSP, both our cases and controls had zero methylation at the targeted GJB2 and RB1 regions. @*Conclusion@#Our study showed no changes in methylation at the selected CpG regions in RB1 and GJB2 in the two comparison groups with or without SNHL. This may be due to a lack of environmental exposures to these target regions. Other epigenetic marks may be present around these regions as well as those of other HL-associated genes.
Subject(s)
Hearing Loss , MethylationABSTRACT
Introducción: La hipoacusia congénita es una patología frecuente entre los recién nacidos con gran impacto en su calidad de vida si no es diagnosticada y tratada precozmente. Para su pesquisa, se recomienda, internacionalmente, el tamizaje auditivo universal neonatal (TAUN), que desde 2014 se aplica en el Hospital Clínico La Florida Dra. Eloísa Díaz Insunza (HLF). Objetivo: Describir la experiencia del programa de TAUN del Servicio de Otorrinolaringología HLF. Material y Método: Estudio descriptivo, retrospectivo. Se incluyó a todos los recién nacidos vivos (RNV) del establecimiento entre 2015 y 2021, evaluados de acuerdo con el protocolo del programa. Resultados: Fueron evaluados 17.804 RNV. Se obtuvo una cobertura de 97,1% y tasa de referencia de 0,98%. Se confirmaron a 21 pacientes con hipoacusia sensorioneural (HSN), obteniéndose una tasa de HSN de 1,5 cada 1.000 RNV. Conclusión: La tasa de incidencia de HSN congénita fue similar a la estimada a nivel mundial. El programa de TAUN HLF cumple con estándares internacionales en cuanto a cobertura, tiempo de evaluación del tamizaje y tasa de referencia. El trabajo multidisciplinario, mejoría de tecnología y registro adecuado de pacientes, son las principales fortalezas. La dificultad de seguimiento durante horario inhábil y presencia de sólo un profesional con dedicación exclusiva, son aspectos a perfeccionar.
Introduction: Congenital hearing loss is a frequent pathology among newborns with great impact on their quality of life if it is not diagnosed and treated early. The Joint Committee on Infant Hearing recommends universal newborn hearing screening (UNHS) and has been applied at the Hospital Clínico La Florida Dra. Eloísa Díaz Insunza (HLF) since 2014. Aim: To describe the experience of UNHS program at the Otolaryngology Service of the HLF. Material and Method: Descriptive, retrospective study, including all newborns of the HLF between 2015 and 2021. They were evaluated according to the protocol proposed in the program. Results: 17804 newborns were evaluated. Coverage of 97,1% and a referral rate of 0,98% were obtained. Twenty-one patients with sensorineural hearing loss (SNHL) were confirmed, obtaining a SNHL rate of 1.5 per 1000 live births. Conclusion: The incidence rate of congenital SNHL is similar to that estimated worldwide. The UNHS program in HLF complies with international standards in terms of coverage, timing and referral rates. Multidisciplinary work, improved technology and adequate patient registration are the main strengths of the program. The difficulty of follow-ups during the weekends and the presence of only one full-time professional are aspects that can be improved.
Subject(s)
Humans , Infant, Newborn , Neonatal Screening/methods , Hearing Loss/diagnosis , Hearing Loss, Sensorineural/diagnosis , Epidemiology, Descriptive , IncidenceABSTRACT
ABSTRACT Purpose The aim of the study was to verify the level of satisfaction of CI users with long periods of hearing deprivation, highlighting the positive and negative aspects of the use of the device and their quality of life. Methods This is a analytical research, of the type observational cross-sectional study. The study was performed with 24 patients from a private Institute of Otorhinolaryngology. Three surveys were applied: Satisfaction with Amplification in Daily Life (SADL), International Outcome Inventory - Cochlear Implant (IOI - CI) to assess cochlear implant satisfaction and WHOQOL- bref to assess the quality of life. The results in the pre and post lingual groups were compared. Results The highest degree of satisfaction was reported with regard to personal image, positive effects, and how the users feel about their CI. The lowest degree of satisfaction was reported regarding the cost-benefit of the CI and the competitive noise. In the WHOQOL-bref assessment, the highest scores were found in physical, psychological and social relations domains. When comparing the results of the surveys, the pre and post-lingual groups showed no difference in relation to the achieved scores. Conclusion The participants had a high level of satisfaction with the use of cochlear implants. The longer the sensory deprivation time, the greater the degree of satisfaction with the device. The use of the CI electronic device reflects on the individual's quality of life.
RESUMO Objetivo Verificar o nível de satisfação de usuários de IC com longos períodos de privação auditiva, destacando os aspectos positivos e negativos do uso do dispositivo e avaliar a qualidade de vida desses indivíduos. Método Trata-se de uma pesquisa analítica, do tipo estudo observacional transversal. O estudo foi realizado com 24 pacientes de um instituto privado de Otorrinolaringologia. Foram aplicados três questionários: Satisfaction with Amplification in Daily Life (SADL), International Outcome Inventory- Implante Coclear (IOI - IC) para avaliar a satisfação com o implante coclear e WHOQOL-bref para avaliar a qualidade de vida. Os resultados nos grupos pré e pós-lingual foram comparados. Resultados O maior grau de satisfação foi relatado em relação à imagem pessoal, efeitos positivos e como os usuários se sentem em relação ao seu IC. O menor grau de satisfação ocorreu em relação ao custo-benefício do IC e ao ruído competitivo. Na avaliação do WHOQOL-bref, os maiores escores foram encontrados nos domínios físico, psicológico e relações sociais. Os grupos pré e pós-lingual não apresentaram diferença em relação aos escores alcançados. Conclusão Os participantes apresentaram alto nível de satisfação com o uso do implante coclear. Quanto maior o tempo de privação sensorial maior o grau de satisfação com o dispositivo. O uso do dispositivo eletrônico de IC reflete melhora na qualidade de vida do indivíduo.
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Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.
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ABSTRACT Waardenburg syndrome is a rare congenital genetic disorder characterized by sensorineural hearing loss and pigmentary abnormalities of the hair, skin, and eyes. Based on the different clinical presentations, it is divided into four subtypes as in WS1 to WS4. This report describes a 15-year-old boy who presented with low vision and bilateral hearing loss. His visual acuity was 20/200 in both eyes. Slit-lamp examination revealed complete iris heterochromia, with one blue iris and one brown iris. Fundus examination showed symmetrical pigmentation of the retina and choroid, with atrophy of the pigment epithelium in the macular region, notably also in the eye with normal iris pigment illustrating the broad spectrum of the iris and fundus pigmentation as part of this syndrome. A carefully clinical and ophthalmological evaluation should be done to differentiate various types of Waardenburg syndrome and other associated auditory-pigmentary syndrome. Early diagnosis in some cases may be crucial for the adequate development of patients affected with this condition.
RESUMO A síndrome de Waardenburg é uma doença genética congênita rara caracterizada por perda auditiva neurossensorial e anormalidades pigmentares do cabelo, da pele e dos olhos. Com base nas diferentes apresentações clínicas, é dividida em quatro subtipos (WS1 a WS4). Este relato descreve o caso de um menino de 15 anos que apresentava baixa visão e perda auditiva bilateral. Sua acuidade visual era de 20/200 em ambos os olhos. O exame em lâmpada de fenda revelou heterocromia completa da íris, com uma íris azul e uma íris marrom. A fundoscopia mostrou pigmentação simétrica da retina e coroide, com atrofia do epitélio pigmentar na região macular, notadamente também no olho com pigmento de íris normal, ilustrando o amplo espectro de pigmentação de íris e fundo como parte dessa síndrome. Uma avaliação clínica e oftalmológica criteriosa deve ser feita para diferenciar os vários tipos de síndrome de Waardenburg e outras síndromes auditivo-pigmentares associadas. O diagnóstico precoce em alguns casos pode ser crucial para o desenvolvimento adequado dos pacientes acometidos por essa condição.
Subject(s)
Humans , Male , Adolescent , Pigmentation Disorders/diagnosis , Retinal Diseases/diagnosis , Retinal Diseases/etiology , Waardenburg Syndrome/complications , Iris Diseases/diagnosis , Iris Diseases/etiology , Pigmentation Disorders/etiology , Waardenburg Syndrome/diagnosis , Visual Acuity , Slit Lamp Microscopy , Fundus Oculi , Hearing Loss, Sensorineural/etiologyABSTRACT
El Síndrome de Alport (SA) es un desorden genético originado por mutaciones en el colágeno tipo IV que es el constituyente principal de las membranas basales. Clínicamente, se caracteriza por nefropatía hereditaria progresiva. En el oído interno, el colágeno IV se encuentra ubicado en la membrana basilar y en el ligamento espiral, por lo que las mutaciones en los genes codificadores provocan hipoacusia de tipo neurosensorial. La presente investigación tiene por objetivo caracterizar el comportamiento de la pérdida de audición en personas diagnosticadas con SA, mediante la revisión de estudios de la literatura. Se realizó una búsqueda en bases de datos con los criterios de inclusión establecidos, incorporando un total de siete artículos para su análisis. Teniendo en cuenta los hallazgos de las diversas investigaciones recopiladas, se concluye que la pérdida auditiva secundaria al SA es heterogénea. No hay un patrón común de presentación pues depende del modo de herencia y del tipo de mutación de la enfermedad, además su grado de severidad y progresión va paralelo a la función renal. Sin embargo, independientemente del momento en que se manifieste el deterioro auditivo, es de vital importancia una intervención audiológica oportuna, con el fin de detectar la hipoacusia lo más temprano posible, hacer un seguimiento riguroso de la funcionalidad auditiva y, en caso de ser necesario, adaptar ayudas auditivas de acuerdo con las necesidades comunicativas del paciente
Alport Syndrome (AS) is a genetic disorder originated by mutations in the collagen type IV which is the main constituent of the basal membranes. Clinically, is characterized by progressive hereditary nephropathy. In the inner ear, the collagen IV is ubicated in the basilar membrane and in the spiral ligament, whereby the mutations in the codifier gens, cause neurosensorial hearing loss. The aim of this investigation is to characterize how hearing loss behaves in people diagnosed with AS, by presenting a reviewing of reported studies in the literature. A database search was performed with the established criteria of inclusion and a total of seven articles were incorporated for its analysis. Considering the findings by the several investigations collected the conclusion is that the secondary hearing loss to AS is heterogenous. There is no a common pattern of presentation as it depends on the mode of heredity and the type of mutation of the disease, and besides of the degree of severity and progression that goes along with the renal function. Nevertheless, regardless of the moment that the auditive impair appears, it is of vital importance an opportune audiological intervention, aiming at detecting an alteration as soon as possible, make a rigorous tracking of the auditive functionality and, if necessary, incorporate hearing aids in accordance with the communicative needs of the patients
Subject(s)
HumansABSTRACT
Objective:To summarize the clinical features and gene variations in children with Townes-Brocks syndrome (TBS).Methods:The clinical data of a female infant diagnosed with TBS caused by human spalt-like transcription factor 1 ( SALL1) gene mutation in Gansu Maternal and Child Health Hospital in May 2022 were analyzed retrospectively. Relevant articles up to July 2022 were retrieved from several databases including CNKI, VIP, Wanfang, Chinese Medical Journal Network and PubMed with the terms of " SALL1 gene" and "Townes-Brocks syndrome". Patients diagnosed with TBS caused by SALL1 gene mutation were retrieved and the clinical phenotype-genotype correlations in patients with TBS caused by frameshift mutation in SALL1 gene were analyzed and summarized. Descriptive statistical analysis was applied. Results:(1) Clinical data: The index patient was a 40-day-old girl exhibiting major clinical manifestations of polycystic kidney dysplasia, congenital external ear deformity, preaxial polydactyly and recto-perineal fistula. Whole exome sequencing and Sanger sequencing revealed a heterozygous variation of c.420delC (p.S141fs*42) in the SALL1 gene, while the same gene was found to be wild type in her parents and sister. The variant was predicted to be pathogenic (PVS1+PS2+PM2). (2) Literature review retrieved 161 cases of TBS, of which 71 were attributable to a frameshift mutation in SALL1 gene. Clinical phenotypes of the 71 cases and the index case were summarized. TBS was mainly characterized by external ear, hand and anal deformities, sometimes accompanied by hearing loss, abnormal kidney development and foot deformity. A small number of affected cases presented with rare clinical phenotypes such as abnormal eyes, hypothyroidism and abnormal development. At present, the human gene mutation database records 110 variations in the SALL1 gene, with a majority located in exon 2. The most common mutation type was frameshift variation, accounting for 52%, followed by missense variation and nonsense variation. Conclusion:TBS should be considered in children with ear, hand and anal malformations, accompanied by renal dysfunction and hearing loss, and genetic testing is recommended for timely diagnosis.
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La otitis media crónica (OMC) activa y colesteatomatosa se consideraban una contraindicación para el uso de implante coclear (IC) en el pasado. Actualmente, se han propuesto múltiples opciones quirúrgicas para el IC, de acuerdo con las características clínicas del paciente y el tipo de patología otológica prexistente. La cirugía del IC se puede realizar en un tiempo o en etapas, siendo fundamental intervenir un oído seco y estable previo a la instalación definitiva del electrodo para reducir complicaciones y obtener resultados auditivos satisfactorios. El IC es un tratamiento seguro y eficaz en pacientes con OMC. Múltiples reportes internacionales han evidenciado baja tasa de complicaciones mayores y óptimos resultados auditivos funcionales en pacientes con hipoacusia y OMC.
Supurative and cholesteatomatous chronic otitis media (COM) were considered a contraindication to cochlear implant (CI) use in the past. Currently, multiple surgical options have been proposed for CI according to the clinical characteristics of the patient and the type of pre-existing otological pathology. Cochlear implant surgery can be performed in a single time or in stages, being essential to intervene a dry and stable ear prior to the definitive installation of the electrode to reduce complications and obtain satisfactory hearing results. CI is a safe and effective treatment in patients with COM. Multiple international reports have shown a low rate of major complications and optimal functional hearing results in patients with hearing loss and COM.
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Abstract Objective: To investigate the impact of certain clinical parameters on likelihood of hearing recovery after SSNHL and to detect potential variables that predict its outcome. Methods: A retrospective study was conducted in order to identify the clinical parameters which influence hearing recovery after SSNHL. Medical charts of 87 patients diagnosed with SSNHL and treated in the clinic from January 2015 to December 2019 were retrospectively reviewed. The effects of several parameters on the success of the treatment were statistically evaluated. Such parameters studied were age, gender, the severity of hearing loss, audiometric curve pattern, the time treatment was initiated, and metabolic factors. Results: Patient categorization according to tonal audiometric curve results statistically differed in terms of recovery (complete, partial, no recovery) (x2 = 32.5; p< 0.001). There are 2× more diabetic patients in the group no recovery than in the group partial recovery (OR = 3.1; 95% CI 0.95-10; p = 0.061), and 5.3× more than in the group complete recovery (OR = 10.4; 95% CI 2.3-45; p = 0.002) (χ2 = 11.2; p = 0.004). There is a statistically significant correlation between onset of therapy and recovery on a scale of significance of 93% (χ2 = 5.3, p= 0.069). Conclusion: In our study tonal audiometry results, diabetes mellitus and onset of therapy were shown to be a statistically significant negative predictive factors for recovery. Results of pure tone audiometry suggest a flat profound curve is statistically associated with the no recovery rates, and a down sloping curve proved to be statistically associated with complete recovery rates. Level of evidence: Level 1.
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Abstract Introduction: Impaired cochlear perfusion is a major etiological factor in idiopathic sudden sensorineural hearing loss. Oxidative stress has been shown to be a risk factor for oxidative damage. Objectives: We investigated the role of oxidative stress in idiopathic sudden sensorineural hearing loss by comparing serum levels of oxidant and antioxidant molecules including thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, arylesterase, ceruloplasmin and myeloperoxidase in patients who did and did not recover after treatment. Methods: The amount of dynamic disulfide was calculated by determining half of the difference between the total thiols and native thiols. After the determination of native, total thiol, and disulfide amounts, the disulfide/total thiol percent ratio, native thiol/total thiol ratio and disulfide/native thiol percent ratio were calculated and then compared between the two groups. Additionally, clinical relationship between audiological recovery and native thiol, disulfide, disulfide/native thiol percent ratio, and disulfide/total thiol percent ratio levels was investigated. Blood samples were also analyzed for the assessment of thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, arylesterase, ceruloplasmin, and myeloperoxidase levels. Results: A significant difference was found between the two groups with regard to total oxidant status disulfide, disulfide/native thiol percent ratio, disulfide/total thiol percent ratio, and native thiol/total thiol ratio levels (p = 0.001, p = 0.001, p = 0.001, p = 0.003, p = 0.001, p = 0.002, respectively). However, no significant difference was found between the two groups with regard to thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, ceruloplasmin, and myeloperoxidase levels (p > 0.05 for all). Conclusion: The results supported the common hypothesis that vascular pathologies are the primary cause of idiopathic sudden sensorineural hearing loss and that other etiological factors ultimately result in vascular pathologies. The oxidant-antioxidant and thiol-disulfide balances were impaired in the idiopathic sudden sensorineural hearing loss group.
Resumo Introdução: A perfusão coclear prejudicada é um fator etiológico importante na perda auditiva neurossensorial súbita idiopática (PANSSI). O estresse oxidativo mostrou ser um fator de risco para danos oxidativos. Objetivos: Investigamos o papel do estresse oxidativo na PANSSI mediante a comparação dos níveis séricos de moléculas oxidantes e antioxidantes, inclusive homeostase de tiol/dissulfeto, paraoxonase, paraoxonase estimulada, arilesterase, ceruloplasmina e mieloperoxidase em pacientes com e sem recuperação após o tratamento. Método: A quantidade de dissulfeto dinâmico foi calculada mediante a determinação de metade da diferença entre os tiois totais e os tiois nativos. Após a determinação das quantidades de tiol nativo, tiol total e dissulfeto, as razões percentuais de dissulfeto/tiol total, tiol nativo/tioltotal e dissulfeto/tiol nativo foram calculadas e depois comparadas entre os dois grupos. Além disso, a relação clínica entre a recuperação audiológica e os níveis de tiol nativo, tiol nativo/tiol total, dissulfeto, dissulfeto/tiol nativo e dissulfeto/tiol total foi investigada. Amostras de sangue também foram analisadas para avaliar os níveis de paraoxonase, paraoxonase estimulada, arilesterase, ceruloplasmina e mieloperoxidase. Resultados: Uma diferença significante foi encontrada entre os dois grupos em relação ao estado oxidante total e aos níveis de dissulfeto, dissulfeto/tiol nativo, dissulfeto/tiol total, tiol nativo/tiol total (p = 0,001, p = 0,001, p = 0,001, p = 0,003, p = 0,001, p = 0,002, respectivamente). Porém, não foi encontrada diferença significante entre os dois grupos em relação aos níveis de paraoxonase, paraoxonase estimulada, ceruloplasmina e mieloperoxidade (p> 0,05 para todos). Conclusão: Os resultados apoiaram a hipótese comum de que as doenças vasculares são a principal causa de PANSSI e que, em última análise, outros fatores etiológicos resultam em doenças vasculares. Os equilíbrios de oxidante-antioxidante e tiol-dissulfeto estavam prejudicados no grupo PANSSI.
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Introducción: La infección congénita por citomegalovirus es causa de pérdida auditiva y alteraciones cognitivas. La infección perinatal por este virus es más frecuente en neonatos< 1500 g y produce menos secuelas neurológicas. Objetivo: Describir la evaluación neurológica en el primer año de vida en niños muy bajo peso al nacer con infección por citomegalovirus. Métodos: Estudio descriptivo y longitudinal en el que se incuyeron 14 neonatos< 1500 g, con diagnóstico de infección congénita o perinatal por citomegalovirus; a los cuales se les realizó evaluación del neurodesarrollo, ultrasonido craneal, potenciales evocados auditivos de tallo cerebral y potenciales visuales a las 40 semanas, a los seis meses y al año de edad gestacional corregida. En la primera evaluación se realizó además, electroencefalograma. Resultados: El 43 por ciento tuvo infección congénita y 57 por ciento infección perinatal. A las 40 semanas se evaluaron completamente 79 % de los casos, a los seis meses 64 por ciento y al año 36 por ciento. No se observaron anormalidades en el ultrasonido craneal, ni en el electroencefalograma. Al año de edad corregida, se detectaron alteraciones ligeras del neurodesarrolo en 33,3 por ciento del total de casos (2/6) y con igual porcentaje en los niños con infección congénita (1/3) y perinatal (1/3). En ningún paciente evaluado se detectó sordera neurosensorial, ni daño del nervio visual. Conclusiones: Las alteraciones del neurodesarrollo encontradas al año de edad corregida pueden estar relacionadas con la prematuridad o la infección por citomegalovirus. El seguimiento a mediano y largo plazo es necesario para detectar otras secuelas neurológicas de debut tardío(AU)
Introduction: Congenital cytomegalovirus infection is a cause of hearing loss and cognitive impairments. Perinatal infection by this virus is more frequent in neonates< 1500 g and produces fewer neurological sequelae. Objective: To describe neurological evaluation in the first year of life in very low birth weight children with cytomegalovirus infection. Methods: A descriptive and longitudinal study involving 14 neonates< 1500 g, with a diagnosis of congenital or perinatal cytomegalovirus infection; to which neurodevelopmental evaluation, cranial ultrasound, auditory brain stem evoked potentials and visual potentials were performed at 40 weeks, six months and one year of corrected gestational age. In the first evaluation, electroencephalogram was also performed. Results: 43 percent had congenital infection and 57 percent perinatal infection. At 40 weeks, 79 percent of cases were fully evaluated, at six months 64 percent and at one year 36 percent. No abnormalities were observed on the cranial ultrasound or electroencephalogram. At one year of corrected age, slight alterations in neurodevelopment were detected in 33.3 percent of all cases (2/6) and with the same percentage in children with congenital (1/3) and perinatal (1/3) infection. In no patient evaluated, sensorineural deafness or visual nerve damage was detected. Conclusions: The neurodevelopmental alterations found at one year of corrected age may be related to prematurity or cytomegalovirus infection. Medium- and long-term follow-up is necessary to detect other late-onset neurological sequelae(AU)
Subject(s)
Humans , Infant, Newborn , Aftercare/methods , Cytomegalovirus Infections/etiology , Infant, Very Low Birth Weight/growth & development , Hearing Loss, Sensorineural , Epidemiology, Descriptive , Longitudinal Studies , Cytomegalovirus/genetics , Observational Studies as TopicABSTRACT
Abstract Introduction Idiopathic sudden sensorineural hearing loss (ISSHL) is a disabling otologic urgency whose ethiopathogenesis is still controversial. Only in recent years metabolic syndrome (MetS) has been implicated as a possible aggravating factor in the prognosis of recovery from ISSHL. Objective To assess whether the preexistence of MetS interferes on hearing recovery levels. Methods Retrospective cohort study composed of adult (> 18 years old) ISSHL patients admitted for treatment between January 2015 and December 2019. To diagnose ISSHL, we used pure-tone audiometry, and identified MetS patients based on the criteria of the United States National Cholesterol Education Program Adult Treatment Panel III (NCEPATP III). The treatment protocol comprised hospitalization for five days for the intravenous administration of dexamethasone, audiometric surveillance, imaging and blood analyses, and, based on recovery, the planning of rescue treatments (intratympanic administration of dexamethasone and/or hyperbaric oxygen). The Siegel criteria were used to evaluate the hearing outcomes. Results The final sample was composed of 81 patients, 48 without MetS (nMetS) and 33 with MetS. Regarding the Siegel recovery category, the nMetS group had significantly better results (p = 0.001), with 44% of complete recoveries against 6% in the MetS, and 58% of the MetS patients had the worst outcome, contrasting with 27% in the nMetS group. The nMetS group had an overall better evolution in terms of hearing recovery and had a significant improvement in the median hearing gain (20.6 dB versus 8.8 dB; p = 0.008). Additionally, the multivariate analysis revealed that the presence of MetS is a significant risk factor for a worse outcome (odds ratio [OR] = 0.30; 95% confidence interval [95%CI] = 0.10-0.85). Conclusion Regardless of age, gender, the initial audiometry threshold, and autoimmunity, MetS is a clear risk factor for a worse outcome regarding the recovery of hearing after ISSHL.
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Hemodialysis as a treatment modality for Stage V chronic kidney disease (CKD) has significantly decreased mortality. However, there have been several studies reporting sensorineural hearing loss (SNHL) in these patients which affects their quality of life. The role of hemodialysis in its etiology has been widely debated and the strength of available evidence has not yet been evaluated. A systematic literature review was conducted to identify a possible association of hemodialysis to hearing loss and guide in making informed decisions in the management of CKD patients. Relevant articles from PubMed, ScienceDirect, EBSCO Medline, SCOPUS, Google Scholar, and Clinical Key were identified using Preferred Reporting Items for Systematic Review and Meta-Analyses framework. Studies that were published since the inception of the database to May 2020 and written in the English language were analyzed. A total of 36 articles on hemodialysis and sudden or SNHL were retrieved. Many were observational (13 cohorts, 10 case-control, seven case series, and six cross-sectional) studies involving 99,020 participants with ages ranging from 1 to 80 years. This review elucidates the occurrence of SNHL in CKD patients on hemodialysis. Yet, the available literature fails to provide conclusive evidence on hemodialysis as a causative agent for SNHL. Audiological assessment of all CKD patients before hemodialysis and regular follow-up may help in early diagnosis and better management of hearing loss
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Background: Diabetes is a chronic metabolic disorder and its complications pose a significant healthcare burden. Basic pathophysiology of diabetic complications is angiopathy leading to neuropathy. Angiopathy of small vessels of cochlea and neuropathy of cochlear nerve may lead to hearing impairment. Aim and Objectives: The present study was taken up with an objective to evaluate the changes in the auditory brainstem evoked potentials in type 2 diabetic patients leading to hearing loss compared to healthy controls. Materials and Methods: A total of 40 subjects in the age group of 40–60 were enrolled into the study and were categorized into 2 groups of 20 each. In the Group 1, age- and sex-matched healthy controls were included and in the Group 2 subjects with type 2 diabetes of more than 5 years duration were included in the study. Any hearing impairment caused by a known disease, drug or injury either traumatic, iatrogenic or noise induced were excluded from the study. They were subjected to auditory brainstem response test Brainstem evoked response audiometry. Absolute latency of wave I-V and interpeak latencies (IPL) I-III, III-V, and I-V was recorded. The data obtained were evaluated using VassarStats. Results: There was no significant difference in the wave latency of wave I and II between the groups; however, the latencies of waves III, IV, and V was higher in the diabetic group compared to controls on both right and left ear stimulation and it was statistically significant. With respect to the IPL comparison, it was observed that IPL I-III, III-V, and I-V were significantly increased in diabetics with both right and left ear stimulation were statistically significant on comparison with the controls. Conclusion: The delayed transmission of the auditory pathway at the level of brainstem and midbrain observed in the study advocates the presence of central neuropathy in patients with type 2 diabetes mellitus.
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Objective:To investigate the efficacy of intratympanic injection versus intravenous drip of prednisolone acetate in the treatment of sudden hearing loss. Methods:A total of 120 patients with sudden hearing loss who received treatment in the Department of Otolaryngology, Mingzhou Hospital between January 2017 and January 2020 were included in this study. They were divided into intratympanic injection group (intratympanic injection of prednisolone acetate, n = 60) and intravenous injection group (intravenous injection of prednisolone acetate, n = 60) according to route of drug administration. After 8 days of treatment, clinical efficacy was compared between the two groups. The hearing thresholds at 500 Hz and 1 000 Hz in both groups were detected using pure tone audiometry. The levels of procalcitonin and high-sensitivity C-reactive protein and adverse drug reactions were compared between the two groups. Results:After treatment, total response rate in the intratympanic injection group was significantly higher than that in the intravenous injection group (93.33% vs. 80.00%, χ2 = 4.61, P < 0.05). The hearing threshold at 500 Hz in the intratympanic injection group was significantly lower than that in the intravenous injection group [(38.69 ± 3.56) vs. (42.36 ± 4.36), t = 5.05, P < 0.001). The hearing threshold at 1 000 Hz in the intratympanic injection group was significantly lower than that in the intravenous injection group [(32.36 ± 3.36) vs. (40.15 ± 4.12), t = 11.35, P < 0.001). After treatment, procalcitonin level in the intratympanic injection group was significantly lower than that in the intravenous injection group [(0.65 ± 0.12) μg/L vs. (0.98 ± 0.15) μg/L, t = 13.30, P < 0.001)]. High-sensitivity C-reactive protein level in the intratympanic injection group was significantly lower than that in the intravenous injection group [(3.28 ± 0.36) mg/L vs. (5.26 ± 0.56) mg/L, t = 23.03, P < 0.001]. There was no significant difference in incidence of adverse reactions between intratympanic injection and intravenous injection groups (8.33% vs. 10.00%, χ2 = 0.10, P > 0.05). Conclusion:Compared with intravenous drip of prednisolone acetate, intratympanic injection of prednisolone acetate can improve the clinical symptoms of patients with sudden hearing loss and enhance clinical efficacy.
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We have reported cases of idiopathic sensorineural hearing loss treated with Japanese Kampo medicine in the past, but they all exhibited deficiency pattern. This time, we experienced cases of idiopathic sensorineural hearing loss, which exhibited medium pattern and excess pattern. Case 1 is a 57-year-old man. He had a chief complaint of ringing and hearing loss in his right ear from 3 days ago and was diagnosed with right idiopathic sensorineural hearing loss Grade3b. He exhibited medium pattern with liver qi depression and blood stasis, and was administered shosaikoto and keishibukuryogan. He was cured after 2 weeks. Case 2 is a 48-year-old man. He had a chief complaint of hearing loss in his right ear and dizziness from 2 days ago and was diagnosed with right idiopathic sensorineural hearing loss Grade4a. He exhibited excess pattern with liver qi depression and blood stasis, and was administered daisaikoto and tokakujokito for 1 week. After that, we switched tokakujokito to keishibukuryogan and continued treatment, but there remained a slight difference in hearing around the treble part, and it stopped at a remarkable recovery.