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1.
Proc Natl Acad Sci U S A ; 121(15): e2314763121, 2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38557194

RESUMO

Although sudden sensorineural hearing loss (SSNHL) is a serious condition, there are currently no approved drugs for its treatment. Nevertheless, there is a growing understanding that the cochlear pathologies that underlie SSNHL include apoptotic death of sensory outer hair cells (OHCs) as well as loss of ribbon synapses connecting sensory inner hair cells (IHCs) and neurites of the auditory nerve, designated synaptopathy. Noise-induced hearing loss (NIHL) is a common subtype of SSNHL and is widely used to model hearing loss preclinically. Here, we demonstrate that a single interventive application of a small pyridoindole molecule (AC102) into the middle ear restored auditory function almost to prenoise levels in a guinea pig model of NIHL. AC102 prevented noise-triggered loss of OHCs and reduced IHC synaptopathy suggesting a role of AC102 in reconnecting auditory neurons to their sensory target cells. Notably, AC102 exerted its therapeutic properties over a wide frequency range. Such strong improvements in hearing have not previously been demonstrated for other therapeutic agents. In vitro experiments of a neuronal damage model revealed that AC102 protected cells from apoptosis and promoted neurite growth. These effects may be explained by increased production of adenosine triphosphate, indicating improved mitochondrial function, and reduced levels of reactive-oxygen species which prevents the apoptotic processes responsible for OHC death. This action profile of AC102 might be causal for the observed hearing recovery in in vivo models.


Assuntos
Perda Auditiva Provocada por Ruído , Perda Auditiva Neurossensorial , Cobaias , Animais , Audição , Cóclea , Ruído/efeitos adversos , Células Ciliadas Auditivas Externas/fisiologia , Limiar Auditivo
2.
BMC Med Genomics ; 17(1): 89, 2024 Apr 16.
Artigo em Inglês | MEDLINE | ID: mdl-38627775

RESUMO

OBJECTIVE: Branchio-oto-renal syndrome (BOR, OMIM#113,650) is a rare autosomal dominant disorder that presents with a variety of symptoms, including hearing loss (sensorineural, conductive, or mixed), structural abnormalities affecting the outer, middle, and inner ear, branchial fistulas or cysts, as well as renal abnormalities.This study aims to identify the pathogenic variants by performing genetic testing on a family with Branchio-oto-renal /Branchio-otic (BO, OMIM#602,588) syndrome using whole-exome sequencing, and to explore possible pathogenic mechanisms. METHODS: The family spans 4 generations and consists of 9 individuals, including 4 affected by the BOR/BO syndrome. Phenotypic information, including ear malformation and branchial cleft, was collected from family members. Audiological, temporal bone imaging, and renal ultrasound examinations were also performed. Whole-exome sequencing was conducted to identify candidate pathogenic variants and explore the underlying molecular etiology of BOR/BO syndrome by minigene experiments. RESULTS: Intra-familial variability was observed in the clinical phenotypes of BOR/BO syndrome in this family. The severity and nature of hearing loss varied in family members, with mixed or sensorineural hearing loss. The proband, in particular, had profound sensorineural hearing loss on the left and moderate conductive hearing loss on the right. Additionally, the proband exhibited developmental delay, and her mother experienced renal failure during pregnancy and terminated the pregnancy prematurely. Genetic testing revealed a novel heterozygous variant NM_000503.6: c.639 + 3 A > C in the EYA1 gene in affected family members. In vitro minigene experiments demonstrated its effect on splicing. According to the American College of Medical Genetics (ACMG) guidelines, this variant was classified as likely pathogenic. CONCLUSION: This study highlights the phenotypic heterogeneity within the same family, reports the occurrence of renal failure and adverse pregnancy outcomes in a female patient at reproductive age with BOR syndrome, and enriches the mutational spectrum of pathogenic variants in the EYA1 gene.


Assuntos
Síndrome Brânquio-Otorrenal , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Insuficiência Renal , Humanos , Gravidez , Feminino , Síndrome Brânquio-Otorrenal/genética , Síndrome Brânquio-Otorrenal/patologia , Peptídeos e Proteínas de Sinalização Intracelular/genética , Proteínas Tirosina Fosfatases/genética , Perda Auditiva/genética , Linhagem , Proteínas Nucleares/genética
3.
Cereb Cortex ; 34(4)2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38610087

RESUMO

Hearing is critical to spoken language, cognitive, and social development. Little is known about how early auditory experiences impact the brain structure of children with bilateral sensorineural hearing loss. This study examined the influence of hearing aid use and residual hearing on the auditory cortex of children with severe to profound congenital sensorineural hearing loss. We evaluated cortical preservation in 103 young pediatric cochlear implant candidates (55 females and 48 males) by comparing their multivoxel pattern similarity of auditory cortical structure with that of 78 age-matched children with typical hearing. The results demonstrated that early-stage hearing aid use preserved the auditory cortex of children with bilateral congenital sensorineural hearing loss. Children with less residual hearing experienced a more pronounced advantage from hearing aid use. However, this beneficial effect gradually diminished after 17 months of hearing aid use. These findings support timely fitting of hearing aids in conjunction with early implantation to take advantage of neural preservation to maximize auditory and spoken language development.


Assuntos
Córtex Auditivo , Auxiliares de Audição , Perda Auditiva Neurossensorial , Feminino , Masculino , Humanos , Criança , Perda Auditiva Neurossensorial/terapia , Audição , Encéfalo
4.
Neurology ; 102(9): e209358, 2024 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-38593395

RESUMO

We present a case study of a 24-year-old man who reported mild balance and walking difficulties for 2 years. He had a history of recurrent fever, skin lesions, headache, and elbow pain, but most of these events resolved spontaneously. There was no significant family history. On examination, we observed frontal bossing, sensorineural hearing loss, and gait ataxia. This case underscores the significance of identifying clinical indicators in patients with neurologic symptoms, particularly recurrent fever, to establish a precise and thorough differential diagnosis.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Masculino , Humanos , Adulto Jovem , Adulto , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/diagnóstico , Cefaleia , Marcha , Raciocínio Clínico
5.
J Pak Med Assoc ; 74(3): 476-479, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38591281

RESUMO

Objectives: To analyse the demographic and clinical variables in children having undergone cochlear implant surgery because of deafness. METHODS: The cross-sectional study was conducted from January to November 2022 at the Centre for Research in Experimental and Applied Medicine laboratory of the Department of Biochemistry and Molecular Biology, Army Medical College, Rawalpindi, Pakistan, in collaboration with the Ear, Nose and Throat Department of Combined Military Hospital, Rawalpindi, and comprised children of eith gender aged up to 10 years who had received cochlear implant. Data was collected through questionnaire-based detailed interviews. Syndromic Hearing Loss, Non-Syndromic Hearing Loss, and Acquired Hearing Loss were identified among the subjects. Data was analysed using SPSS 22. RESULTS: Of the 250 cases, 147(58.8%) were boys, 146(58.4%) were aged 0-5 years, 219(87.6%) had prelingual onset of disease, and 202(80.8%) had a non-progressive disease course. In 203(81.2%) cases, normal developmental milestones were seen. Parental consanguinity was observed in 219(87.6%) cases. However, 63(25.2%) patients had a first-degree relative who had a history of deafness. In 170(68%) cases, hearing loss was hereditary, whereas in 80(32%) it was acquired. Meningitis was the most commonly identified risk factor 55(68.75%). Acquired risk factors and family history had significant association with hearing loss (p<0.05). Speech perception significantly improved in all 219(100%) patients with prelingual hearing loss who underwent cochlear implantation. CONCLUSIONS: Majority of the cases were found to be male, had a prelingual disease onset and a non-progressive disease course. Family history was a significant factor, while meningitis was the most common acquired cause of hearing loss.


Assuntos
Implante Coclear , Implantes Cocleares , Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Meningite , Criança , Humanos , Masculino , Feminino , Implantes Cocleares/efeitos adversos , Implante Coclear/efeitos adversos , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva Neurossensorial/etiologia , Estudos Transversais , Perda Auditiva/epidemiologia , Perda Auditiva/complicações , Surdez/epidemiologia , Surdez/cirurgia , Meningite/complicações , Demografia
6.
Trials ; 25(1): 256, 2024 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-38610038

RESUMO

BACKGROUND: Teleaudiology can potentially improve access to hearing healthcare services. Remote hearing aid fittings offer a new mode of service delivery that removes barriers of geography and access to an audiologist. Real-ear measurements (REMs) are the gold standard for hearing aid output verification but require in-clinic appointments. This study will investigate whether remote hearing aid fittings can provide clinically equivalent outcomes when compared to current, in-clinic, best practice guidelines. RESEARCH DESIGN: A repeated measure, double-blinded crossover design will be used. Participants will be randomly allocated to one of two groups to determine order of intervention, balanced for degree of hearing loss. STUDY SAMPLE: Sixty adults with mild to moderate hearing loss and at least 1 year of experience with hearing aids will be recruited. DATA COLLECTION AND ANALYSIS: Participants will complete two hearing aid fitting protocols, one using an in-clinic fitting process and the other using a remote (at-home) fitting process. In-clinic fittings will include REMs with adjustments to standard (NAL-NL2) prescription targets. The two fitting protocols will then be randomly assigned to participants in a crossover design, so participants and researchers will be blinded to the order of the two fitting protocols. Participants will then have a 4-week period with follow-up appointments for participant-directed gain adjustment. For each fitting protocol, participants will complete objective measurements of final hearing aid output with REMs, speech-in-noise testing, subjective measurements of hearing aid performance, and quality of life measurements. They will then begin an identical period of living with, adjusting, and objective assessment with the other fitting protocol. Data will be analysed as repeated measures with statistical control for potential confounding variables. RESULTS: Data will compare the four-frequency average real-ear aided response (4FREAR) for hearing aids programmed in-clinic and hearing aids programmed remotely, after participant-directed gain adjustments. Secondary measures will assess clinically significant differences in estimated speech intelligibility, hearing-related quality of life, hearing aid benefit, sound quality and preference, and speech-in-noise ability. CONCLUSIONS: This study will inform the development of best practice guidelines for remote hearing aid fittings. If no clinically significant differences are found between in-clinic and remote fit hearing aids, it has the potential to expand teleaudiology initiatives. TRIAL REGISTRATION: Australian New Zealand Clinical Trial Registry, ACTRN12623000028606p . Date of registration: 12 January 2023.


Assuntos
Auxiliares de Audição , Perda Auditiva Neurossensorial , Perda Auditiva , Adulto , Humanos , Qualidade de Vida , Austrália , Instituições de Assistência Ambulatorial , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/terapia , Ensaios Clínicos Controlados Aleatórios como Assunto
7.
Sci Rep ; 14(1): 8326, 2024 04 09.
Artigo em Inglês | MEDLINE | ID: mdl-38594301

RESUMO

The MYO7A gene is known to be responsible for both syndromic hearing loss (Usher syndrome type1B:USH1B) and non-syndromic hearing loss including autosomal dominant and autosomal recessive inheritance (DFNA11, DFNB2). However, the prevalence and detailed clinical features of MYO7A-associated hearing loss across a large population remain unclear. In this study, we conducted next-generation sequencing analysis for a large cohort of 10,042 Japanese hearing loss patients. As a result, 137 patients were identified with MYO7A-associated hearing loss so that the prevalence among Japanese hearing loss patients was 1.36%. We identified 70 disease-causing candidate variants in this study, with 36 of them being novel variants. All variants identified in autosomal dominant cases were missense or in-frame deletion variants. Among the autosomal recessive cases, all patients had at least one missense variant. On the other hand, in patients with Usher syndrome, almost half of the patients carried biallelic null variants (nonsense, splicing, and frameshift variants). Most of the autosomal dominant cases showed late-onset progressive hearing loss. On the other hand, cases with autosomal recessive inheritance or Usher syndrome showed congenital or early-onset hearing loss. The visual symptoms in the Usher syndrome cases developed between age 5-15, and the condition was diagnosed at about 6-15 years of age.


Assuntos
Perda Auditiva Neurossensorial , Síndromes de Usher , Humanos , Pré-Escolar , Criança , Adolescente , Síndromes de Usher/epidemiologia , Síndromes de Usher/genética , Prevalência , Miosinas/genética , Miosina VIIa/genética , Mutação , Linhagem
9.
Artigo em Chinês | MEDLINE | ID: mdl-38563177

RESUMO

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube(ET). Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography(HRCT) of six patients(nine ears) with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and(14.7±6.4) years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss(average bone and air conduction thresholds were 13.7 dB and 71.3 dB), three had mixed hearing loss(average bone and air conduction thresholds were 27.7 dB and 83.7 dB), and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were(22.61±2.94) mm and(6.50±2.33) mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.


Assuntos
Surdez , Tuba Auditiva , Perda Auditiva Neurossensorial , Vestíbulo do Labirinto , Masculino , Humanos , Feminino , Tuba Auditiva/diagnóstico por imagem , Estudos Retrospectivos , Orelha Média/cirurgia , Perda Auditiva Neurossensorial/diagnóstico
10.
Artigo em Chinês | MEDLINE | ID: mdl-38563182

RESUMO

Various inner ear diseases such as sensorineural deafness and Meniere's disease bring about problems such as speech communication disorders and decreased work efficiency, which seriously affect the life quality of patients. Due to the special anatomical structure and blood-labyrinth barrier in the inner ear, the current drug administration methods are often unable to achieve satisfactory results. Nanocarriers are the forefront and hot spot of nanotechnology research. In recent years, a lot of research progress has been made in the field of targeted delivery of the inner ear, which is expected to be eventually applied to the treatment of clinical diseases of the inner ear. This review focuses on the advantages, main research achievements and limitations of various nanocarriers in the targeted delivery of the inner ear, hoping to provide new ideas for related research.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Doenças do Labirinto , Doença de Meniere , Humanos , Doença de Meniere/tratamento farmacológico , Qualidade de Vida
12.
PLoS Med ; 21(4): e1004296, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38573882

RESUMO

BACKGROUND: Patients with severe-to-profound hearing loss may benefit from management with cochlear implants. These patients need a referral to a cochlear implant team for further assessment and possible surgery. The referral pathway may result in varied access to hearing healthcare. This study aimed to explore referral patterns and whether there were any socioeconomic or ethnic associations with the likelihood of referral. The primary outcome was to determine factors influencing referral for implant assessment. The secondary outcome was to identify factors impacting whether healthcare professionals had discussed the option of referral. METHODS AND FINDINGS: A multicentre multidisciplinary observational study was conducted in secondary care Otolaryngology and Audiology units in Great Britain. Adults fulfilling NICE (2019) audiometric criteria for implant assessment were identified over a 6-month period between 1 July and 31 December 2021. Patient- and site-specific characteristics were extracted. Multivariable binary logistic regression was employed to compare a range of factors influencing the likelihood of implant discussion and referral including patient-specific (demographics, past medical history, and degree of hearing loss) and site-specific factors (cochlear implant champion and whether the hospital performed implants). Hospitals across all 4 devolved nations of the UK were invited to participate, with data submitted from 36 urban hospitals across England, Scotland, and Wales. Nine hospitals (25%) conducted cochlear implant assessments. The majority of patients lived in England (n = 5,587, 86.2%); the rest lived in Wales (n = 419, 6.5%) and Scotland (n = 233, 3.6%). The mean patient age was 72 ± 19 years (mean ± standard deviation); 54% were male, and 75·3% of participants were white, 6·3% were Asian, 1·5% were black, 0·05% were mixed, and 4·6% were self-defined as a different ethnicity. Of 6,482 submitted patients meeting pure tone audiometric thresholds for cochlear implantation, 311 already had a cochlear implant. Of the remaining 6,171, 35.7% were informed they were eligible for an implant, but only 9.7% were referred for assessment. When adjusted for site- and patient-specific factors, stand-out findings included that adults were less likely to be referred if they lived in more deprived area decile within Indices of Multiple Deprivation (4th (odds ratio (OR): 2·19; 95% confidence interval (CI): [1·31, 3·66]; p = 0·002), 5th (2·02; [1·21, 3·38]; p = 0·05), 6th (2·32; [1·41, 3·83]; p = 0.05), and 8th (2·07; [1·25, 3·42]; p = 0·004)), lived in London (0·40; [0·29, 0·57]; p < 0·001), were male (females 1·52; [1·27, 1·81]; p < 0·001), or were older (0·97; [0·96, 0·97]; p < 0·001). They were less likely to be informed of their potential eligibility if they lived in more deprived areas (4th (1·99; [1·49, 2·66]; p < 0·001), 5th (1·75; [1·31, 2·33], p < 0·001), 6th (1·85; [1·39, 2·45]; p < 0·001), 7th (1·66; [1·25, 2·21]; p < 0·001), and 8th (1·74; [1·31, 2·31]; p < 0·001) deciles), the North of England or London (North 0·74; [0·62, 0·89]; p = 0·001; London 0·44; [0·35, 0·56]; p < 0·001), were of Asian or black ethnic backgrounds compared to white patients (Asian 0·58; [0·43, 0·79]; p < 0·001; black 0·56; [0·34, 0·92]; p = 0·021), were male (females 1·46; [1·31, 1·62]; p < 0·001), or were older (0·98; [0·98, 0·98]; p < 0·001). The study methodology was limited by its observational nature, reliance on accurate documentation of the referring service, and potential underrepresentation of certain demographic groups. CONCLUSIONS: The majority of adults meeting pure tone audiometric threshold criteria for cochlear implantation are currently not appropriately referred for assessment. There is scope to target underrepresented patient groups to improve referral rates. Future research should engage stakeholders to explore the reasons behind the disparities. Implementing straightforward measures, such as educational initiatives and automated pop-up tools for immediate identification, can help streamline the referral process.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Perda Auditiva , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Perda Auditiva Neurossensorial/cirurgia , Perda Auditiva/cirurgia , Escolaridade
13.
Zool Res ; 45(2): 284-291, 2024 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-38485498

RESUMO

Hereditary hearing loss (HHL), a genetic disorder that impairs auditory function, significantly affects quality of life and incurs substantial economic losses for society. To investigate the underlying causes of HHL and evaluate therapeutic outcomes, appropriate animal models are necessary. Pigs have been extensively used as valuable large animal models in biomedical research. In this review, we highlight the advantages of pig models in terms of ear anatomy, inner ear morphology, and electrophysiological characteristics, as well as recent advancements in the development of distinct genetically modified porcine models of hearing loss. Additionally, we discuss the prospects, challenges, and recommendations regarding the use pig models in HHL research. Overall, this review provides insights and perspectives for future studies on HHL using porcine models.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Perda Auditiva , Doenças dos Suínos , Animais , Suínos/genética , Qualidade de Vida , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/veterinária , Perda Auditiva/genética , Perda Auditiva/terapia , Perda Auditiva/veterinária , Modelos Animais
14.
J Vis Exp ; (205)2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38497652

RESUMO

The ear is the organ most susceptible to explosion overpressure, and cochlear injuries frequently occur after blast exposure. Blast exposure can lead to sensorineural hearing loss (SNHL), which is an irreversible hearing loss that negatively affects the quality of life. Detailed blast-induced cochlear pathologies, such as the loss of hair cells, spiral ganglion neurons, cochlear synapses, and disruption of stereocilia, have been previously documented. However, determining cochlear sensorineural deterioration after a blast injury is challenging because animals exposed to blast overpressure usually experience tympanic membrane perforation (TMP), which causes concurrent conductive hearing loss. To evaluate pure sensorineural cochlear dysfunction, we developed an experimental animal model of blast-induced cochlear injury using a laser-induced shock wave. This method avoids TMP and concomitant systemic injuries and reproduces the functional decline in the SNHL component in an energy-dependent manner after LISW exposure. This animal model could be a platform for elucidating the pathological mechanisms and exploring potential treatments for blast-induced cochlear dysfunction.


Assuntos
Traumatismos Craniocerebrais , Perda Auditiva Neurossensorial , Animais , Explosões , Qualidade de Vida , Cóclea , Lasers
15.
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
16.
J Int Adv Otol ; 20(1): 89-93, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38454296

RESUMO

Cochlear implantation has become a standard of care for a child diagnosed with bilateral profound sensorineural hearing loss with a structured surgical standard operating procedure. A 3-year-old boy with bilateral profound prelingual sensorineural deafness underwent a Med-EL Sonata Ti100 implant. We faced a peculiar situation intraoperatively after inserting the electrodes and closing the wound. The impedance recording indicated high ground path impedance with short-circuiting of few electrodes. As a bionic implant, its electronic components may at times malfunction both intraoperatively and/or postoperatively; therefore, neural response telemetry (NRT) was invented to check it. By using NRT and a few milliliters of normal saline, we were able to diagnose as well as rectify the malfunctioning of the implant.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva Neurossensorial , Pré-Escolar , Humanos , Masculino , Cóclea/cirurgia , Implante Coclear/efeitos adversos , Implante Coclear/métodos , Implantes Cocleares/efeitos adversos , Perda Auditiva Bilateral/cirurgia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/cirurgia , Solução Salina , Telemetria/métodos
17.
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
18.
Ther Deliv ; 15(4): 237-252, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38469721

RESUMO

Aim: Excessive free radicals contribute to oxidative stress and mitochondrial dysfunction in sensorineural hearing loss (SNHL). The antioxidant probucol holds promise, but its limited bioavailability and inner ear barriers hinder effective SNHL treatment. Methodology: We addressed this by developing probucol-loaded nanoparticles with polymers and lithocholic acid and tested them on House Ear Institute-Organ of Corti cells. Results: Probucol-based nanoparticles effectively reduced oxidative stress-induced apoptosis, enhanced cellular viability, improved probucol uptake and promoted mitochondrial function. Additionally, they demonstrated the capacity to reduce reactive oxygen species through the nuclear factor erythroid 2-related factor 2/heme oxygenase-1 pathway. Conclusion: This innovative nanoparticle system holds the potential to prevent oxidative stress-related hearing impairment, providing an effective solution for SNHL.


Hearing loss affects millions of people worldwide, and its prevalence is expected to double by 2050. Current treatments have limitations, pushing researchers to explore new options. Oxidative stress is a key player in hearing loss and is known to damage inner ear hair cells. While antioxidants, known for their protective effects, hold promise, delivering them effectively to the inner ear is challenging. Scientists have been testing nanoparticles loaded with the antioxidant probucol to fight hearing loss. In this study, these particles protected inner ear cells in cell studies, offering potential hope for preventing hearing problems. This research is a significant step toward finding better treatments for hearing loss.


Assuntos
Orelha Interna , Perda Auditiva Neurossensorial , Nanopartículas , Humanos , Probucol/farmacologia , Estresse Oxidativo , Antioxidantes/farmacologia , Perda Auditiva Neurossensorial/terapia
19.
Int Tinnitus J ; 27(2): 217-224, 2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38507638

RESUMO

INTRODUCTION: Tinnitus is a frequent condition that indicates the sensation of sound in the absence of a corresponding external stimulus and can significantly impair the quality of life. The main risk factor for developing tinnitus is hearing loss. The diagnosis of tinnitus is based on history, assessment of tinnitus severity, clinical examination, and audiological tests. The main purpose of this research was to examine the relationship between the presence and level of hearing loss and the characteristics of tinnitus in patients with bilateral subjective tinnitus. METHODS: Total number of 50 participants, 20 men, and 30 women were included in the research. Demographic data, data on hearing impairment obtained by tone audiometry, and data on difficulties caused by tinnitus obtained in two questionnaires - Tinnitus Handicap Inventory (THI) and Tinnitus Functional Index (TFI) were used. RESULTS: Age above 30 years is significantly associated with tinnitus with hearing loss. Hearing impairment is also significantly more often associated with an auditory TFI index >6.7, a total THI index >20, and an emotional THI index >3. Hearing loss was noted in 76% of patients. CONCLUSION: Tinnitus represents a significant burden for patients, therefore it is important to assess the impact of tinnitus on daily activities and quality of life.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Zumbido , Masculino , Humanos , Feminino , Adulto , Zumbido/diagnóstico , Zumbido/epidemiologia , Zumbido/complicações , Qualidade de Vida , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/complicações , Perda Auditiva/complicações
20.
Biochem Pharmacol ; 222: 116115, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38460910

RESUMO

In recent years, extensive research has been conducted on the pathogenesis of sensorineural hearing loss (SNHL). Apoptosis and necrosis have been identified to play important roles in hearing loss, but they cannot account for all hearing loss. Autophagy, a cellular process responsible for cell self-degradation and reutilization, has emerged as a significant factor contributing to hearing loss, particularly in cases of autophagy deficiency. Autophagy plays a crucial role in maintaining cell health by exerting cytoprotective and metabolically homeostatic effects in organisms. Consequently, modulating autophagy levels can profoundly impact the survival, death, and regeneration of cells in the inner ear, including hair cells (HCs) and spiral ganglion neurons (SGNs). Abnormal mitochondrial autophagy has been demonstrated in animal models of SNHL. These findings indicate the profound significance of comprehending autophagy while suggesting that our perspective on this cellular process holds promise for advancing the treatment of SNHL. Thus, this review aims to clarify the pathogenic mechanisms of SNHL and the role of autophagy in the developmental processes of various cochlear structures, including the greater epithelial ridge (GER), SGNs, and the ribbon synapse. The pathogenic mechanisms of age-related hearing loss (ARHL), also known as presbycusis, and the latest research on autophagy are also discussed. Furthermore, we underscore recent findings on the modulation of autophagy in SNHL induced by ototoxic drugs. Additionally, we suggest further research that might illuminate the complete potential of autophagy in addressing SNHL, ultimately leading to the formulation of pioneering therapeutic strategies and approaches for the treatment of deafness.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva , Animais , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Neurossensorial/metabolismo , Células Ciliadas Auditivas/metabolismo , Perda Auditiva/metabolismo , Modelos Animais de Doenças , Autofagia
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