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1.
Acta otorrinolaringol. esp ; 75(2): 83-93, Mar-Abr. 2024. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-231380

RESUMO

Introducción: La hipoacusia neurosensorial (HNS) congénita o de inicio precoz es una de las enfermedades hereditarias más frecuentes en nuestro medio y es la deficiencia sensorial más frecuente. Es importante realizar un estudio etiológico de la hipoacusia y el estudio genético mediante la secuenciación de nueva generación (NGS) es la prueba con mayor rendimiento diagnóstico. Nuestro estudio muestra los resultados genéticos obtenidos en una serie de pacientes con HNS congénita/de inicio precoz bilateral. Material y método: Se incluyeron 105 niños diagnosticados de HNS bilateral a los que se les realizó un estudio genético entre los años 2019 y 2022. El estudio genético consistió en una secuenciación masiva del exoma completo, filtrando el análisis para los genes incluidos en un panel virtual de hipoacusia con 244 genes. Resultados: Se obtuvo un diagnóstico genético en 48% (50/105) de los pacientes. Se detectaron variantes patogénicas y probablemente patogénicas en 26 genes diferentes, siendo los genes más frecuentemente afectados el gen GJB2, USH2A y STRC. De las variantes detectadas 52% (26/50) se asociaron a una hipoacusia no sindrómica, 40% (20/50) una hipoacusia sindrómica y 8% restante (4/50) se podían asociar tanto a una hipoacusia sindrómica como no sindrómica. Conclusiones: El estudio genético constituye una parte fundamental del diagnóstico etiológico de la HNS bilateral. Nuestra serie muestra que el estudio genético de la hipoacusia mediante NGS tiene un alto rendimiento diagnóstico y nos proporciona información de gran utilidad en la práctica clínica.(AU)


Introduction: Congenital/early-onset sensorineural hearing loss (SNHL) is one of the most common hereditary disorders in our environment. There is increasing awareness of the importance of an etiologic diagnosis, and genetic testing with next-generation sequencing (NGS) has the highest diagnostic yield. Our study shows the genetic results obtained in a cohort of patients with bilateral congenital/early-onset SNHL. Materials and methods: We included 105 children with bilateral SNHL that received genetic testing between 2019 and 2022. Genetic tests were performed with whole exome sequencing, analyzing genes related to hearing loss (virtual panel with 244 genes). Results: 48% (50/105) of patients were genetically diagnosed. We identified pathogenic and likely pathogenic variants in 26 different genes, and the most frequently mutated genes were GJB2, USH2A and STRC. 52% (26/50) of variants identified produced non-syndromic hearing loss, 40% (20/50) produced syndromic hearing loss, and the resting 8% (4/50) could produce both non-syndromic and syndromic hearing loss. Conclusions: Genetic testing plays a vital role in the etiologic diagnosis of bilateral SNHL. Our cohort shows that genetic testing with NGS has a high diagnostic yield and can provide useful information for the clinical workup of patients.(AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/etiologia , Diagnóstico Pré-Implantação , Otolaringologia , Sequenciamento de Nucleotídeos em Larga Escala
2.
Medicine (Baltimore) ; 103(9): e37317, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38428895

RESUMO

To evaluate the correlation between thallium and diabetes risk among participants with hearing loss. This retrospective cohort study extracted related data such as demographic characteristics, lifestyle factors, and laboratory findings from the National Health and Nutrition Examination Survey (NHANES) database (2013-2018). Logistic regression analysis and interaction analysis were adopted to analyze the correlation between thallium and diabetes risk among patients with hearing loss. Then, the restricted cubic spline was employed to assess the nonlinear relationship between thallium and diabetes risk. The receiver operating characteristic curve and decision curve analysis were used to assess the predictive values of 3 multivariate models with or without thallium for diabetes risk. The Delong test was adopted to assess the significant change of the area under the curves (AUCs) upon thallium addition. A total of 425 participants with hearing loss were enrolled in the study: without diabetes group (n = 316) and diabetes group (n = 109). Patients with hearing loss in the diabetes group had significantly lower thallium (P < .05). The thallium was an independent predictor for diabetes risk after adjusting various covariates (P < .05). The restricted cubic spline (RCS) result showed that there was a linear correlation between thallium and diabetes risk (P nonlinear > .05). Finally, the receiver operating characteristic and decision curve analysis results revealed that adding thallium to the models slightly increased the performance in predicting diabetes risk but without significance in AUC change. Thallium was an independent predictor of diabetes risk among patients with hearing loss. The addition of thallium might help improve the predictive ability of models for risk reclassification. However, the conclusions should be verified in our cohort in the future due to the limitations inherent in the NHANES database.


Assuntos
Surdez , Diabetes Mellitus , Perda Auditiva , Humanos , Inquéritos Nutricionais , Tálio , Estudos Retrospectivos , Perda Auditiva/epidemiologia , Perda Auditiva/etiologia , Diabetes Mellitus/epidemiologia
3.
Int J Mol Sci ; 25(5)2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38473883

RESUMO

Congenital cytomegalovirus (CMV) infection is the main cause of non-hereditary sensorineural hearing loss (SNHL). In order to shed light on SNHL pathophysiology, we examined the auditory pathway in CMV-infected fetuses; the temporal lobe, in particular the auditory cortex, and the inner ear. We investigated both inner ears and temporal lobes of 20 human CMV-infected fetuses at 21 weeks of gestation. As a negative group, five fetuses from spontaneous miscarriages without CMV infection were studied. Inner ears and temporal lobes were histologically examined, immunohistochemistry for CMV and CMV-PCR were performed. On the auditory cortex, we evaluated the local microglial reaction to the infection. CMV-positive cells were found in 14/20 brains and the damage was classified as severe, moderate, or mild, according to histological features. Fetuses with severe brain damage had a statistically higher temporal lobe viral load and a higher number of activated microglial cells in the auditory cortex compared to fetuses with mild brain damage (p: 0.01; p: 0.01). In the inner ears, the marginal cells of the stria vascularis were the most CMV positive. In our study, CMV affected the auditory pathway, suggesting a tropism for this route. In addition, in the auditory cortex, microglial activation may favor further tissue damage contributing to hearing loss.


Assuntos
Infecções por Citomegalovirus , Perda Auditiva Neurossensorial , Humanos , Citomegalovirus , Vias Auditivas/patologia , Perda Auditiva Neurossensorial/etiologia , Feto/patologia
4.
BMC Geriatr ; 24(1): 220, 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38438862

RESUMO

OBJECTIVE: To analyse and discuss the association of gender differences with the risk and incidence of poststroke aphasia (PSA) and its types, and to provide evidence-based guidance for the prevention and treatment of poststroke aphasia in clinical practice. DATA SOURCES: Embase, PubMed, Cochrane Library and Web of Science were searched from January 1, 2002, to December 1, 2023. STUDY SELECTION: Including the total number of strokes, aphasia, the number of different sexes or the number of PSA corresponding to different sex. DATA EXTRACTION: Studies with missing data, aphasia caused by nonstroke and noncompliance with the requirements of literature types were excluded. DATA SYNTHESIS: 36 papers were included, from 19 countries. The analysis of 168,259 patients with stroke and 31,058 patients with PSA showed that the risk of PSA was 1.23 times higher in female than in male (OR = 1.23, 95% CI = 1.19-1.29, P < 0.001), with a prevalence of PSA of 31% in men and 36% in women, and an overall prevalence of 34% (P < 0.001). Analysis of the risk of the different types of aphasia in 1,048 patients with PSA showed a high risk in females for global, broca and Wenicke aphasia, and a high risk in males for anomic, conductive and transcortical aphasia, which was not statistically significant by meta-analysis. The incidence of global aphasia (males vs. females, 29% vs. 32%) and broca aphasia (17% vs 19%) were higher in females, and anomic aphasia (19% vs 14%) was higher in males, which was statistically significant (P < 0.05). CONCLUSIONS: There are gender differences in the incidence and types of PSA. The risk of PSA in female is higher than that in male.


Assuntos
Afasia , Acidente Vascular Cerebral , Feminino , Humanos , Masculino , Incidência , Afasia/diagnóstico , Afasia/epidemiologia , Afasia/etiologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/epidemiologia , Cooperação do Paciente
5.
Neurology ; 102(7): e209187, 2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38484225

RESUMO

Kelch-like protein-11 (KLHL11) immunoglobulin G (IgG) is a recently reported paraneoplastic autoantibody associated with rhombencephalitis, which commonly presents with ataxia, diplopia, vertigo, hearing loss, tinnitus, and gaze palsies. The association of this high-risk paraneoplastic autoantibody with testicular germ cell tumors is widely accepted, but it has not been associated with Müllerian tumors. In this study, we report a woman without a known germ cell tumor presenting with signs and symptoms suggesting autoimmune encephalitis. She was found to have metastatic ovarian serous carcinoma with KLHL11 immunoreactivity on histopathology. This case demonstrates a rare cancer association of KLHL11 IgG-seropositive rhombencephalitis with Müllerian tumor and highlights that this autoantibody can also be detected in female patients. Thus, this case expands on the current knowledge of KLHL11-related autoimmune encephalitis including the paraneoplastic presentation, associated tumor types, and management of this syndrome in women.


Assuntos
Doenças Autoimunes do Sistema Nervoso , Surdez , Encefalite , Doença de Hashimoto , Perda Auditiva , Neoplasias Testiculares , Feminino , Humanos , Autoanticorpos , Proteínas de Transporte , Perda Auditiva/etiologia , Imunoglobulina G
6.
Vestn Otorinolaringol ; 89(1): 21-27, 2024.
Artigo em Russo | MEDLINE | ID: mdl-38506021

RESUMO

Chronic sensorineural hearing loss (SNHL) is a common disease that leads to disability of the population. Despite the many reports devoted to SNHL, the question of the pathogenesis of the disease is still open. Many researchers consider the development of SNHL as a manifestation of microangiopathy. The mechanism of development of microangiopathy in SNHL is multifactorial, but most researchers agree that endothelial dysfunction (ED) triggers a complex of pathological changes in the vessels of the inner ear. OBJECTIVE: Review of the results of scientific research in recent years on the problem of etiopathogenesis of sensorineural hearing loss from the perspective of endothelial dysfunction in the formation of auditory disorders.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Humanos , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/epidemiologia
7.
Biometrics ; 80(1)2024 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-38488465

RESUMO

Age-related hearing loss has a complex etiology. Researchers have made efforts to classify relevant audiometric phenotypes, aiming to enhance medical interventions and improve hearing health. We leveraged existing pattern analyses of age-related hearing loss and implemented the phenotype classification via quadratic discriminant analysis (QDA). We herein propose a method for analyzing the exposure effects on the soft classification probabilities of the phenotypes via estimating equations. Under reasonable assumptions, the estimating equations are unbiased and lead to consistent estimators. The resulting estimator had good finite sample performances in simulation studies. As an illustrative example, we applied our proposed methods to assess the association between a dietary intake pattern, assessed as adherence scores for the dietary approaches to stop hypertension diet calculated using validated food-frequency questionnaires, and audiometric phenotypes (older-normal, metabolic, sensory, and metabolic plus sensory), determined based on data obtained in the Nurses' Health Study II Conservation of Hearing Study, the Audiology Assessment Arm. Our findings suggested that participants with a more healthful dietary pattern were less likely to develop the metabolic plus sensory phenotype of age-related hearing loss.


Assuntos
Perda Auditiva , Humanos , Causalidade , Análise de Regressão , Perda Auditiva/diagnóstico , Perda Auditiva/etiologia , Fenótipo
8.
Acta Otolaryngol ; 144(1): 23-29, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38461404

RESUMO

BACKGROUND: There is no comprehensive and up-to-date overview of audiovestibular approach to the posterior fossa tumors in the literature. OBJECTIVE: This paper reviewed the literature relating to tumors at the posterior cranial fossa to find red flags alerting a posterior fossa lesion from audiovestibular perspectives. METHODS: This review was developed from articles published in those journals listed on the journal citation reports. Through the PubMed database, Embase, Google Scholar, and Cochrane library, 60 articles were finally obtained based on the PRISMA guidelines for reporting reviews. RESULTS: The presence of one red flag indicates a positive predictive value of 33% for detecting a posterior fossa lesion. Clinical features, namely, 1) mid-frequency sudden sensorineural hearing loss (SNHL), 2) bilateral sudden SNHL, and 3) rebound nystagmus may indicate a posterior fossa lesion, representing one, two, and three red flags, respectively. CONCLUSION: Those with 1) mid-frequency sudden SNHL, 2) bilateral sudden SNHL, and 3) rebound nystagmus trigger one, two, and three red flags, respectively, alerting clinicians the possibility of a posterior fossa lesion, which warrant MR imaging to exclude life-threatening or treatable conditions. SIGNIFICANCE: Patients with posterior fossa tumors may have potential life-threatening outcome.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Neoplasias Infratentoriais , Nistagmo Patológico , Humanos , Perda Auditiva Neurossensorial/patologia , Nistagmo Patológico/diagnóstico , Nistagmo Patológico/etiologia , Neoplasias Infratentoriais/complicações , Neoplasias Infratentoriais/diagnóstico , Neoplasias Infratentoriais/patologia , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Perda Auditiva Súbita/patologia
9.
Phys Med Rehabil Clin N Am ; 35(2): 419-431, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38514227

RESUMO

Poststroke aphasia, which impacts expressive and receptive communication, can have detrimental effects on the psychosocial well-being and the quality of life of those affected. Aphasia recovery is multidimensional and can be influenced by several baseline, stroke-related, and treatment-related factors, including preexisting cerebrovascular conditions, stroke size and location, and amount of therapy received. Importantly, aphasia recovery can continue for many years after aphasia onset. Behavioral speech and language therapy with a speech-language pathologist is the most common form of aphasia therapy. In this review, the authors also discuss augmentative treatment methodologies, collaborative goal setting frameworks, and recommendations for future research.


Assuntos
Afasia , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Humanos , Qualidade de Vida , Afasia/etiologia , Afasia/terapia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Reabilitação do Acidente Vascular Cerebral/métodos , Fonoterapia/métodos
10.
Int Tinnitus J ; 27(2): 135-140, 2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38507626

RESUMO

BACKGROUND: Tympanic membrane perforation due to inactive mucosal chronic suppurative otitis media is a common problem in otolaryngology, with consequent conductive hearing loss. Still, there is controversy about the relationship between the location of the tympanic membrane perforation and the degree of hearing impairment. AIM OF THE STUDY: To assess the correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss in adult patients with inactive mucosal chronic suppurative otitis media. PATIENTS AND METHODS: A prospective cross-sectional study enrolled 74 adult patients with small tympanic membrane perforations (perforation involves less than one quadrant of the tympanic membrane) and conductive hearing loss (airbone gap ≥ 20 dB HL) due to inactive mucosal chronic suppurative otitis media for at least 3 months. The locations of the tympanic membrane perforations were classified as anterosuperior, anteroinferior, posterosuperior, and poster inferior perforations. Audiometric analysis and a CT scan of the temporal bone were done for all patients. The means of the air and bone conduction pure tone hearing threshold averages at frequencies 500, 1000, 2000, and 4000 Hz were calculated, and consequently, the air-bone gaps were calculated and presented as means. The ANOVA test was used to compare the means of the air-bone gaps, and the Scheffe test was used to determine if there were statistically significant differences regarding the degree of conductive hearing loss in relation to different locations of the tympanic membrane perforation. RESULTS: The ages of the patients ranged from 20 to 43 years (mean = 31.9 ± 6.5 years), of whom 43 (58%) were females and 31 (42%) were males. The means of the air-bone gaps were 32.29 ± 5.41 dB HL, 31.34 ± 4.12 dB HL, 29.87 ± 3.48 dB HL, and 29.30 ± 4.60 dB HL in the posteroinferior, posterosuperior, anteroinferior, and anterosuperior perforations, respectively. Although the air-bone gap's mean was greater in the posteroinferior perforation, statistical analysis showed that it was insignificant (P-value=0.168). CONCLUSION: In adult patients with inactive chronic suppurative otitis media, the anteroinferior quadrant is the most common location of the tympanic membrane perforation, and there was an insignificant correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss.


Assuntos
Surdez , Perda Auditiva , Otite Média Supurativa , Perfuração da Membrana Timpânica , Adulto , Masculino , Feminino , Humanos , Otite Média Supurativa/complicações , Perfuração da Membrana Timpânica/diagnóstico , Perfuração da Membrana Timpânica/etiologia , Perda Auditiva Condutiva/diagnóstico , Perda Auditiva Condutiva/etiologia , Estudos Prospectivos , Estudos Transversais , Membrana Timpânica
11.
Int Tinnitus J ; 27(2): 119-125, 2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38507624

RESUMO

BACKGROUND: Noise-Induced Hearing Loss (NIHL) is a prevalent occupational hazard among healthcare professionals, including medical students. Despite its detrimental effects, the awareness and utilization of hearing protection measures among medical students in Saudi Arabia remain understudied. OBJECTIVE: Is to determine the level of awareness and understanding of NIHL among medical students in Saudi Arabia, as well as their knowledge and usage of hearing protection measures and to identify potential barriers and facilitators for hearing protection utilization. METHODS: A mixed-methods approach was employed, involving a questionnaire survey and semi-structured interviews. The survey collected data on demographics, knowledge of NIHL, and hearing protection practices among medical students. Subsequently, a semi-structured interview was conducted to obtain in-depth insights into the students' experiences, attitudes, and beliefs regarding NIHL and the use of hearing protection. RESULTS: The level about NIHL was 59.32%. Better access to information is associated with increased odds of awareness (odds ratio=3.07, p=0.012). Having relatives with hearing loss increases the odds of awareness (odds ratio =2.49, p=0.034). Individuals with hearing loss or impairment have higher odds of awareness (odds ratio =2.27, p=0.046). Ear Pain, temporary hearing loss, tinnitus, or ringing in the ear: These factors are not significantly associated with awareness of noise-induced hearing loss (p>0.05). Using hearing aids is strongly associated with increased odds of awareness (odds ratio =3.94, p=0.006).The quantitative analysis provided statistical information on the prevalence rates and factors influencing hearing protection usage, while the qualitative analysis uncover nuanced perspectives and experiences. CONCLUSION: This research will contribute to the understanding of NIHL and hearing protection practices among medical students in Saudi Arabia. Improving hearing protection awareness and practices among medical students can ultimately reduce the incidence of NIHL and promote a healthier work environment within the healthcare sector.


Assuntos
Surdez , Perda Auditiva Provocada por Ruído , Ruído Ocupacional , Estudantes de Medicina , Zumbido , Humanos , Perda Auditiva Provocada por Ruído/epidemiologia , Perda Auditiva Provocada por Ruído/etiologia , Perda Auditiva Provocada por Ruído/prevenção & controle , Arábia Saudita/epidemiologia , Zumbido/etiologia , Audição , Ruído Ocupacional/efeitos adversos , Ruído Ocupacional/prevenção & controle
12.
Zh Nevrol Psikhiatr Im S S Korsakova ; 124(3. Vyp. 2): 18-22, 2024.
Artigo em Russo | MEDLINE | ID: mdl-38512090

RESUMO

Aphasia is a systemic disorder of formed speech that develops as a result of local brain lesions. Most aphasias are characterized by damage to secondary cortical fields, which in turn are responsible for the performance of the functions of gnosis and praxis, which explains the variability in the manifestations of speech disorders in patients with acute cerebrovascular accidents. However, it is necessary in each case to diagnose the central pathological mechanism, which underlies the development of the entire syndrome and determines the entire clinical picture. The most important task of a speech therapist-aphasiologist is to qualify the defect, namely to isolate the mechanism and analyze the syndrome in order to select individual methods of corrective restoration. This article presents a case of a patient with an ischemic stroke in the left posterior cerebral artery with the development of amnestic aphasia in combination with alexia without agraphia.


Assuntos
Afasia , Transtornos Cerebrovasculares , Acidente Vascular Cerebral , Humanos , Artéria Cerebral Posterior/diagnóstico por imagem , Afasia/diagnóstico , Afasia/etiologia , Transtornos Cerebrovasculares/complicações , Transtornos Cerebrovasculares/diagnóstico , Distúrbios da Fala , Síndrome
13.
Ugeskr Laeger ; 186(10)2024 Mar 04.
Artigo em Dinamarquês | MEDLINE | ID: mdl-38445342

RESUMO

Swimming-induced pulmonary oedema (SIPE) is a rare but potentially life-threatening condition which occurs in otherwise healthy swimmers. This is a case report of a 62-year-old female developing severe dyspnoea, haemoptysis and hypoxia during open-water swimming. The report provides the current perspectives of SIPE regarding clinical presentation and treatment and presents theories on the pathophysiology of the condition.


Assuntos
Surdez , Edema Pulmonar , Feminino , Humanos , Pessoa de Meia-Idade , Edema Pulmonar/diagnóstico por imagem , Edema Pulmonar/etiologia , Natação , Nível de Saúde , Hemoptise
14.
Brain Behav ; 14(3): e3450, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38450998

RESUMO

INTRODUCTION: Aphasia and neglect in combination with hemiparesis are reliable indicators of large anterior vessel occlusion (LAVO). Prehospital identification of these symptoms is generally considered difficult by emergency medical service (EMS) personnel. Therefore, we evaluated the simple non-paretic-hand-to-opposite-ear (NPE) test to identify aphasia and neglect with a single test. As the NPE test includes a test for arm paresis, we also evaluated the diagnostic ability of the NPE test to detect LAVO in patients with suspected stroke. METHODS: In this prospective observational study, we performed the NPE test in 1042 patients with suspected acute stroke between May 2021 and May 2022. We analyzed the correlation between the NPE test and the aphasia/neglect items of the National Institutes of Health Stroke Scale. Additionally, the predictive values of the NPE test for LAVO detection were calculated. RESULTS: The NPE test showed a strong, significant correlation with both aphasia and neglect. A positive NPE test result predicted LAVO with a sensitivity of 0.70, a specificity of 0.88, and an accuracy of 0.85. Logistic regression analysis showed an odds ratio of 16.14 (95% confidence interval 10.82-24.44) for predicting LAVO. CONCLUSION: The NPE test is a simple test for the detection of both aphasia and neglect. With its predictive values for LAVO detection being comparable to the results of LAVO scores in the prehospital setting, this simple test might be a promising test for prehospital LAVO detection by EMS personnel. Further prospective prehospital validation is needed.


Assuntos
Afasia , Serviços Médicos de Emergência , Acidente Vascular Cerebral , Estados Unidos , Humanos , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico , Afasia/diagnóstico , Afasia/etiologia , Mãos , Razão de Chances
15.
Otol Neurotol ; 45(4): 362-375, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38437804

RESUMO

OBJECTIVE: To examine the otologic and neurotologic symptoms, physical examination findings, and imaging features secondary to hematologic malignancies. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, databases, including PubMed, Scopus, and CINAHL, were searched for articles including patients with otologic manifestations of leukemia, lymphoma and multiple myeloma. Data collected included patient and study demographics, specific hematologic malignancy, timing and classification of otologic symptoms, physical examination findings, imaging features and methods of diagnosis. Pooled descriptive analysis was performed. RESULTS: Two hundred seventy-two articles, of which 255 (93.8%) were case reports and 17 (6.2%) were case series, reporting on 553 patients were identified. Otologic manifestations were reported on 307 patients with leukemia, 204 patients with lymphoma and 42 patients with multiple myeloma. Hearing loss and unilateral facial palsy were the most common presenting symptoms for 111 reported subjects with leukemia (n = 46, 41.4%; n = 43, 38.7%) and 90 with lymphoma (n = 38, 42.2%; n = 39, 43.3%). Hearing loss and otalgia were the most common presenting symptoms for 21 subjects with multiple myeloma (n = 10, 47.6%; n = 6, 28.6%). Hearing loss and unilateral facial palsy were the most common otologic symptoms indicative of relapse in subjects with leukemia (n = 14, 43.8%) and lymphoma (n = 5, 50%). CONCLUSION: Hearing loss, facial palsy, and otalgia might be the first indication of a new diagnosis or relapse of leukemia, lymphoma, or multiple myeloma. Clinicians should have a heightened level of suspicion of malignant etiologies of otologic symptoms in patients with current or medical histories of these malignancies.


Assuntos
Paralisia de Bell , Surdez , Paralisia Facial , Perda Auditiva , Neoplasias Hematológicas , Leucemia , Linfoma , Mieloma Múltiplo , Humanos , Dor de Orelha , Paralisia Facial/complicações , Mieloma Múltiplo/complicações , Perda Auditiva/etiologia , Neoplasias Hematológicas/complicações , Surdez/complicações , Leucemia/complicações , Paralisia de Bell/complicações , Linfoma/complicações , Recidiva
16.
Expert Rev Neurother ; 24(3): 267-271, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38323346

RESUMO

INTRODUCTION: Aphasia is a communication disorder resulting from stroke and/or neurodegenerative conditions which involve the left cerebral hemisphere. It is a debilitating disorder affecting a person's ability to speak, understand, read, and write. Its impact on daily life necessitates therapeutic strategies to aid patients with aphasia. AREAS COVERED: In this special report, the authors speculate whether current pharmacotherapeutic strategies are effective in treating aphasia. The authors look at aphasia caused by different conditions and how this could impact therapy before providing the reader with their expert perspectives. The aim of this paper is for the reader to gain a clearer understanding of the efficacy of the current pharmacotherapeutic treatment paradigms as well as potential future developments. EXPERT OPINION: The exploration of pharmacotherapy for aphasia in vascular brain disorders and neurodegenerative diseases has received much attention in recent years with various therapeutic strategies having been put forward. In terms of whether pharmacotherapy is effective for the treatment of aphasia, there is still no clear-cut answer. Further research is needed with more studies requiring a greater emphasis on language and communication deficits. Biomarkers may also help clinicians provide their patients with a more personalized treatment plan.


Assuntos
Afasia , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Humanos , Afasia/tratamento farmacológico , Afasia/etiologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/tratamento farmacológico , Encéfalo
17.
BMJ Case Rep ; 17(2)2024 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-38413140

RESUMO

Rare causes of stroke-like presentations can be difficult to diagnose. We report a case of a man in his 40s who first presented with stroke symptoms, but whose clinical course was not typical for a stroke. A detailed investigation of the patient's medical history revealed bilateral sensorineural hearing loss which prompted a wider diagnostic assessment.Furthermore, lack of vascular risk factors and a normal angiogram strengthened our suspicion of an unusual underlying condition. Raised lactic acid levels and genetic analysis confirmed a diagnosis of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome.


Assuntos
Acidose Láctica , Síndrome MELAS , Acidente Vascular Cerebral , Masculino , Adulto , Humanos , Acidose Láctica/diagnóstico , Síndrome MELAS/complicações , Síndrome MELAS/diagnóstico , Síndrome MELAS/genética , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia , Ácido Láctico , Perda Auditiva Bilateral
18.
Niger J Clin Pract ; 27(1): 74-81, 2024 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-38317038

RESUMO

BACKGROUND: Hearing loss is a neurological sequelae associated with sickle cell disease (SCD) and probably sickle cell trait (SCT) in children and adults but remains understudied. AIM: This study aimed to compare the hearing impairment among children and adults living with SCD or SCT. METHODS: A comparative cross-sectional study conducted in four departments with SCD outpatient clinic in a tertiary hospital in Nigeria. Participants with Sickle cell disease (HbSS) and Sickle cell trait (HbAS) (cohort) and HbAA (control) had comprehensive ear and hearing assessments for sensorineural hearing loss. Audiometric results were categorized according to WHO classifications and data analysed with Statistical Analysis System (SAS 9.4). RESULTS: A total of 212 participants (106 cohort and control, respectively), aged 6 months to 55 years, were enrolled. Of these, 35% of children with SCD and 25% with SCT had hearing impairment, while 30% of adults with SCD, 36.1% with SCT, and 11% with HbAA had hearing impairment. There was asymmetry in the hearing impairment, with the left ear more affected in children and the right ear in adults. The odds ratio (OD) of hearing impairment was higher in HbSS (2.48 (95% confidence interval (CI):1.51-4.14); P = 0.0004) and HbAS (2.28 (95% CI: 1.1-4.58); P = 0.02) participants compared with HbAA but was not statistically significant when adjusted for frequency of hospitalization, crises, blood transfusion and routine drugs in HbAS (P = 0.49) unlike HbSS (P = 0.03). CONCLUSION: The prevalence of hearing loss among children and adults with SCD is higher than in those with HbA genotype. The frequency of hospitalization, crises, blood transfusion and taking routine drugs may influence hearing impairment in SCT but may not in SCD.


Assuntos
Anemia Falciforme , Perda Auditiva Neurossensorial , Perda Auditiva , Traço Falciforme , Adulto , Criança , Humanos , Traço Falciforme/epidemiologia , Estudos Transversais , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Perda Auditiva/epidemiologia , Perda Auditiva/etiologia
19.
Otol Neurotol ; 45(4): 352-361, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38361317

RESUMO

OBJECTIVE: To determine if cochlear implant (CI) is safe and effective in patients with radiation therapy (XRT)-induced sensorineural hearing loss and to discuss considerations in this population through a retrospective cohort review, systematic review, and meta-analysis. DATABASES REVIEWED: PubMed, Cochrane Library, and Embase. METHODS: We retrospectively reviewed all CI cases after head and neck (HN) XRT at our institution, noting intraoperative findings, postoperative complications, and hearing outcomes. Change in speech discrimination scores (SDSs) was the primary outcome measure. Systematic review was performed to identify all cases of CI after HNXRT. A meta-analysis was performed to assess SDS change. RESULTS: The retrospective cohort review identified 12 patients who underwent CI after HNXRT. One patient with HN cancer (HNC) and one with central nervous system pathology (CNSP) received bilateral implants. Six had HNC, three had CNSP, and one had Langerhans cell histiocytosis. Eleven had abnormal findings during CI. There were no postoperative complications. Twenty articles with an additional 97 patients were suitable for systematic review inclusion. Of the 109 patients, 67 (61.5%) had HNC and 18 (16.5%) had CNSP. Abnormal intraoperative findings were common (30.3%), most frequently in the mastoid (66.7%). Postoperative complications, including wound dehiscence and infection with some requiring explantation, occurred in 10.1% of patients. Sixty-six patients were included in the meta-analysis. All demonstrated SDS improvement (mean increase, 56.2%). CONCLUSION: Patients with prior HNXRT benefit from CI. Paying careful attention to surgical planning and technique, postoperative care, and patient expectations is imperative, as complications are not uncommon.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Percepção da Fala , Humanos , Implante Coclear/métodos , Estudos Retrospectivos , Perda Auditiva Neurossensorial/cirurgia , Implantes Cocleares/efeitos adversos , Complicações Pós-Operatórias/etiologia
20.
Stroke ; 55(3): 705-714, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38328930

RESUMO

BACKGROUND: Evidence from systematic reviews confirms that speech and language interventions for people with aphasia during the chronic phase after stroke (>6 months) improve word retrieval, functional communication, and communication-related quality of life. However, there is limited evidence of their cost-effectiveness. We aimed to estimate the cost per quality-adjusted life year gained from 2 speech and language therapies compared with usual care in people with aphasia during the chronic phase (median, 2.9 years) after stroke. METHODS: A 3-arm, randomized controlled trial compared constraint-induced aphasia therapy plus (CIAT-Plus) and multimodality aphasia therapy (M-MAT) with usual care in 216 people with chronic aphasia. Participants were administered a standardized questionnaire before intervention and at 12 weeks after the 2-week intervention/control period to ascertain health service utilization, employment changes, and informal caregiver burden. Unit prices from Australian sources were used to estimate costs in 2020. Quality-adjusted life years were estimated using responses to the EuroQol-5 Dimension-3 Level questionnaire. To test uncertainty around the differences in costs and outcomes between groups, bootstrapping was used with the cohorts resampled 1000 times. RESULTS: Overall 201/216 participants were included (mean age, 63 years, 29% moderate or severe aphasia, 61 usual care, 70 CIAT-Plus, 70 M-MAT). There were no statistically significant differences in mean total costs ($13 797 usual care, $17 478 CIAT-Plus, $11 113 M-MAT) and quality-adjusted life years (0.19 usual care, 0.20 CIAT-Plus, 0.20 M-MAT) between groups. In bootstrapped analysis of CIAT-Plus, 21.5% of iterations were likely to result in better outcomes and be cost saving (dominant) compared with usual care. In contrast, 72.4% of iterations were more favorable for M-MAT than usual care. CONCLUSIONS: We observed that both treatments, but especially M-MAT, may result in better outcomes at an acceptable additional cost, or potentially with cost savings. These findings are relevant in advocating for the use of these therapies for chronic aphasia after stroke.


Assuntos
Afasia , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Humanos , Pessoa de Meia-Idade , Análise Custo-Benefício , Qualidade de Vida , Resultado do Tratamento , Austrália , Afasia/etiologia , Afasia/terapia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Terapia da Linguagem
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