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Otosclerosis is a pathological condition affecting the temporal bone, and is characterized by remodelling of the labyrinthine bone tissue through a dynamic process of osteolysis and osteogenesis. This condition progressively leads to hearing loss, tinnitus, and vertigo. Stapedotomy, a surgical procedure involving the removal of the stapes superstructure and its replacement with a prosthesis, is the treatment of choice to improve hearing in individuals with otosclerosis. However, vestibular dysfunction is a significant complication associated with this procedure, which can occur intraoperatively or postoperatively, ranging from the immediate postoperative period to weeks, months, or even years after surgery. This paper aims to provide a comprehensive review of the most important causes of vertigo associated with otosclerosis and stapes surgery with the goal of minimizing the incidence of this complication. Understanding the underlying factors contributing to vertigo in this context is crucial for the prevention and effective management of vertigo in patients undergoing stapedotomy.
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Sordera , Otosclerosis , Cirugía del Estribo , Humanos , Otosclerosis/complicaciones , Otosclerosis/cirugía , Vértigo/etiología , Cirugía del Estribo/efectos adversos , OsteogénesisRESUMEN
Introduction: During cochlear implantation, electrode insertion can cause cochlear damage, inflammation, and apoptosis, which can affect the residual hearing. Nanoparticles are increasingly studied as a way to increase the availability of inner ear protective factors. We studied the effect on rats of Pluronic-coated gold nanoparticles (Plu-AuNPs) containing dexamethasone, which were applied locally in the rat's middle ear following the implant procedure. Methods: Seven rats were used in the study. The right ear served as a model for the Dex-Plu-AuNP group. Following the intracochlear dummy electrode insertion through the round window, Dex-Plu-AuNPs were placed in the round window niche. In the right ear, following the same insertion procedure, free dexamethasone (Dex) was placed in the same manner. Auditory brainstem response thresholds (click stimulus, pure tones at 8 kHz, 16 kHz, 24 kHz, and 32 kHz) were measured before and one week after the procedure. A two-tailed T-test was used for the variables. Statistical significance was set as p < 0.05. Results: In the Dex-Plu-AuNP group, the threshold shift was less than that in the free dexamethasone group, but no statistical significance was noted between the groups. When compared individually, only the 8 kHz frequency showed statistically significant, better results after one week, in favor of the Dex-Plu-AuNP group. The mean postoperative 8 kHz threshold in the Dex-Plu-AuNPs was significantly lower than that of the control group (p = 0.048, t-test). For the other frequencies, statistical analysis showed no significant differences between the mean threshold shifts of the two cohorts. Conclusions: The local application of Plu-AuNPs containing dexamethasone following cochlear implantation may better protect the residual hearing than dexamethasone alone, but a larger sample size is needed to reach a possible statistical significance. Dex-Plu-AuNPs do not seem to cause ototoxicity and may be used as a carrier for other agents. In a clinical setting, Dex-Plu-AuNPs may have the effect of protecting lower frequencies in patients with partial deafness who are candidates for electric acoustic stimulation (EAS). If we consider this tendency, Dex-Plu-AuNPs may also be beneficial for patients with Ménière's disease.
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Dexamethasone is one of the most often used corticosteroid drugs for sensorineural hearing loss treatment, and is used either by intratympanic injection or through systemic delivery. In this study, a biopolymer lipid hybrid microcarrier was investigated for enhanced local drug delivery and sustained release at the round window membrane level of the middle ear for the treatment of sensorineural hearing loss (SNHL). Dexamethasone-loaded and dexamethasone-free microparticles were prepared using biopolymers (polysaccharide and protein, pectin and bovine serum albumin, respectively) combined with lipid components (phosphatidylcholine and Dimethyldioctadecylammonium bromide) in order to obtain a biopolymer-liposome hybrid system, with a complex structure combining to enhance performance in terms of physical and chemical stability. The structure of the microparticles was evaluated by FTIR, XRD, thermal analysis, optical microscopy, and scanning electron microscopy (SEM). The encapsulation efficiency determination and the in vitro Dexamethasone release study were performed using UV-Vis spectroscopy. The high value of encapsulation efficiency and the results of the release study indicated six days of sustained release, encouraging us to evaluate the in vitro cytotoxicity of Dexamethasone-loaded microparticles and their influence on the cytotoxicity induced by Cisplatin on auditory HEI-OC1 cells. The results show that the new particles are able to protect the inner ear sensory cells.
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Hearing loss is the most common neurosensory disorder, and with the constant increase in etiological factors, combined with early detection protocols, numbers will continue to rise. Cochlear implantation has become the gold standard for patients with severe hearing loss, and interest has shifted from implantation principles to the preservation of residual hearing following the procedure itself. As the audiological criteria for cochlear implant eligibility have expanded to include patients with good residual hearing, more attention is focused on complementary development of otoprotective agents, electrode design, and surgical approaches. The focus of this review is current aspects of preserving residual hearing through a summary of recent trends regarding surgical and pharmacological fundamentals. Subsequently, the assessment of new pharmacological options, novel bioactive molecules (neurotrophins, growth factors, etc.), nanoparticles, stem cells, and gene therapy are discussed.
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Implantación Coclear , Implantes Cocleares , Sordera , Pérdida Auditiva , Implantación Coclear/métodos , Implantes Cocleares/efectos adversos , Pérdida Auditiva/complicaciones , Pérdida Auditiva/cirugía , HumanosRESUMEN
Background-Children with genetic disorders have multiple anatomical and physiological conditions that predispose them to obstructive sleep apnea syndrome (OSAS). They should have priority access to polysomnography (PSG) before establishing their therapeutic protocol. We analyzed the prevalence and the severity of OSAS in a particular group of children with genetic disorders and strengthened their need for a multidisciplinary diagnosis and adapted management. Methods-The retrospective analysis included children with genetic impairments and sleep disturbances that were referred for polysomnography. We collected respiratory parameters from sleep studies: apnea-hypopnea index (AHI), SatO2 nadir, end-tidal CO2, and transcutaneous CO2. Subsequent management included non-invasive ventilation (NIV) or otorhinolaryngological (ENT) surgery of the upper airway. Results-We identified 108 patients with neuromuscular disorders or multiple congenital anomalies. OSAS was present in 87 patients (80.5%), 3 of whom received CPAP, 32 needed another form of NIV during sleep, and 15 patients were referred for ENT surgery. The post-therapeutic follow-up PSG parameters confirmed the success of the treatment. Conclusions-The upper airway obstruction diagnostics and management for children with complex genetic diseases need a multidisciplinary approach. Early detection and treatment of sleep-disordered breathing in children with genetic disorders is a priority for improving their quality of life.
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Vestibular sensorial input is essential for psychomotor development of the very small children. In consequence, possible vestibular impairment induced by cochlear implantation in deaf children could affect the balance and walking learning process. Some of cochlear implanted children can present congenital vestibular deficit. The anatomical and embryological relation between auditory and vestibular system explains why congenital neurosensorial hearing loss may associate vestibular impairment. The cochlear implant surgery presents a vestibular lesion risk. Bilateral vestibulopathy, as it appears in early childhood, has a poor prognosis for the psychomotor and cognitive development. Even probably rare, bilateral vestibulopathy induced by simultaneous bilateral cochlear implantation can delay the acquisition of motor skills. This pathology can be avoided by an appropriate surgical indication related to the vestibular preoperative status. This study reports the vestibular saccular functional modifications after the cochlear implantation in children. The cervical vestibular evoked myogenic potentials (cVEMPs) were performed in children before and after the cochlear implantation. Since previous studies report different vestibular impairment related to the portelectrode insertion approach, another objective of our study was to assess the saccular postoperative status depending of the insertion by cochleostomy (CO) or through the round window (RW). We performed cVEMPs for 80 patients (135 cochlear implanted ears) before and after cochlear implantation. We have detected preoperative saccular areflexia in 33 (24.4%) ears. In the group of 102 (75.6%) ears with preoperative normal saccular function, 72 (70.6%) ears preserved the cVEMP response after the surgery, while in 30 (29.4%) ears the cVEMP response was lost. Reporting our findings to the portelectrode insertion method, we found normal saccular function in 73.3% of the cochlear implanted ears by RW surgical approach and in 68.42% ears by CO approach. These results suggest that the RW portelectrode insertion is the recommended strategy in order to avoid the saccular vestibular impairment.
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Implantación Coclear/métodos , Sordera/cirugía , Sáculo y Utrículo/fisiopatología , Preescolar , Femenino , Humanos , MasculinoRESUMEN
Background and objectives: The cochlear implant is not only meant to restore auditory function, but it also has a series of benefits on the psychomotor development and on the maturation of central auditory pathways. In this study, with the help of neuropsychological tests and cortical auditory potentials (CAEPs), we intend to identify a series of instruments that allow us to monitor children with a cochlear implant, and later on, to admit them into an individualized rehabilitation program. Materials and methods: This is a longitudinal study containing 17 subjects (6 boys and 11 girls) diagnosed with congenital sensorineural hearing loss. The average age for cochlear implantation in our cohort is 22 months old. Each child was tested before the cochlear implantation, tested again 3 months after the implant, and then 6 months after the implant. To test the general development, we used the Denver Developmental Screening Test (DDST II). CAEPs were recorded to assess the maturation of central auditory pathways. Results: The results showed there was progress in both general development and language development, with a significant statistical difference between the overall DQ (developmental quotient) and language DQ before the cochlear implantation and three and six months later, respectively. Similarly, CAEP measurements revealed a decrease of positive-going component (P1) latency after cochlear implantation. Conclusion: CAEPs and neuropsychological tests prove to be useful instruments for monitoring the progress in patients with cochlear implants during the rehabilitation process.
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Corteza Auditiva/crecimiento & desarrollo , Enfermedades Auditivas Centrales/complicaciones , Implantes Cocleares/normas , Enfermedades Auditivas Centrales/cirugía , Preescolar , Implantación Coclear/métodos , Implantes Cocleares/efectos adversos , Femenino , Crecimiento y Desarrollo , Humanos , Lactante , Estudios Longitudinales , Masculino , Trastornos del Neurodesarrollo/complicaciones , Trastornos del Neurodesarrollo/cirugíaRESUMEN
Children suffering from conductive or mixed hearing loss may benefit from a bone-anchored hearing aid system (BAHA Attract implantable prosthesis). After audiological rehabilitation, different aspects of development are improving. The objective of this case report is to propose a comprehensive framework for monitoring cortical auditory function after implantation of a bone-anchored hearing aid system by using electrophysiological and neuropsychological measurements. We present the case of a seven-year-old boy with a congenital hearing loss due to a plurimalformative syndrome, including outer and middle ear malformation. After the diagnosis of hearing loss and the audiological rehabilitation with a BAHA Attract implantable prosthesis, the cortical auditory evoked potentials were recorded. We performed a neuropsychological evaluation using the Wechsler Intelligence Scale for Children - Fourth Edition, which was applied according to a standard procedure. The P1 latency was delayed according to the age (an objective biomarker for quantifying cortical auditory function). The neuropsychological evaluation revealed that the child's working memory and verbal reasoning abilities were in the borderline range comparing with his nonverbal reasoning abilities and processing abilities, which were in the average and below-average range, respectively. Cortical auditory evoked potentials, along with neuropsychological evaluation, could be an essential tool for monitoring cortical auditory function in children with hearing loss after a bone-anchored hearing aid implantation.
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Corteza Auditiva/fisiología , Fenómenos Electrofisiológicos , Audífonos , Niño , Oído Externo/anomalías , Oído Externo/fisiopatología , Potenciales Evocados/fisiología , Humanos , Anomalías Maxilomandibulares/fisiopatología , Masculino , Microstomía/fisiopatologíaRESUMEN
INTRODUCTION: Advances in molecular biology have opened the door to a wide range of research material through the usage of genetic testing on certain variables within the human genome known as single-nucleotide polymorphisms (SNPs).The purpose of this article is to present a review on the influence variants within SNPs have on the outcome of laryngeal cancer when associated with different variables, such as external toxins or survival rate. Amongst these toxins, the most frequent and most studied have been alcohol and tobacco consumption, with a proven increased rate of overall cancer risk within the aero-digestive tract. MATERIALS AND METHODS: The review was realized utilizing available studies on the subject of genetic polymorphisms analysis of deoxyribonucleic acid (DNA) samples using polymerase chain reaction (PCR) assays and laryngeal cancer published in the PubMed database. RESULTS: Statistical analysis of 262 polymorphisms shows a predominantly positive association between two genetic variants of the human genome (mutant homozygote and heterozygote) and cancer risk with significant influence on patient outcome and survival. Genotype combinations were divided into two categories depending on the individual 'at risk' and 'protective' allele within the loci of each inherited gene block. Amongst the genes involved with aero-digestive cancers, the most studied were those belonging to the xenobiotic metabolism, nucleotide excision repair (NER) and DNA repair pathways. The presence of toxins has a distinct cumulative effect within the genotype-phenotype relationship, which further influences the presence of malignancy depending on the adaptability of each individual genome. CONCLUSIONS: Laryngeal cancer evolution is linked to inherited risk factors found within the genetic code. Most studied were the genes belonging to NER pathways, DNA repair and xenobiotic metabolism, which all favored mutant homozygote and heterozygote variants, as high risk factors. Only five articles focused on overall survival rates with insufficient results to undisputedly predict the risk variants. The consumption of external toxins has a positive effect on the overall cancer risk in consumers. Most articles affirmed further evaluation or replication was needed in a larger scale population to conclusively validate their results.
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Neoplasias Laríngeas/genética , Predisposición Genética a la Enfermedad , Humanos , Neoplasias Laríngeas/patología , Pronóstico , Resultado del TratamientoRESUMEN
OBJECTIVES: Chloral hydrate is a sedative that has been used for many years in clinical practice and, under proper conditions, gives a deep and long enough sleep to allow performance of objective hearing tests in young children. The reluctance to use this substance stems from side effects reported over time that can vary, depending on dose, procedure settings and immediate life supporting intervention when needed. Our study adds to those that have appeared in recent years, showing that chloral hydrate is an effective and safe substance when is used in proper conditions. METHODS: The study included 322 children who needed sedation for objective hearing tests, from April 2014 to March 2018. Parents were instructed to bring the child tired and fasted for at least 2â¯h before sedation. The sedative was administered by trained staff in the hospital, and the child was monitored until awaking. RESULTS: In our study group, over half of the children were in the age 1-4 years group, and only 15% were older than 4 years. The dose of chloral hydrate ranged between 50 and 83â¯mg/kg body weight, with an average of 75â¯mg. Successful sedation occurred in 94.1% of children; 0.9% of children awoke during testing and required supplemental sedation or rescheduling of the testing. The most common side effects were vomiting, agitation, prolonged sleep, and failure to fall asleep. CONCLUSIONS: Comparing the side effects of chloral hydrate in our study with those from other studies, ours were similar to those described in the literature. In our study chloral hydrate was effective and had only limited adverse effects. The use of chloral hydrate under hospital conditions with proper monitoring could be a practical and safe solution for outpatients or those with short-term hospitalisation.
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Atención Ambulatoria , Hidrato de Cloral/administración & dosificación , Pruebas Auditivas , Hipnóticos y Sedantes/administración & dosificación , Niño , Preescolar , Hidrato de Cloral/efectos adversos , Femenino , Humanos , Hipnóticos y Sedantes/efectos adversos , Lactante , Masculino , Monitoreo FisiológicoRESUMEN
We report a case in which we quantified the maturation of the central auditory pathway in children with hearing loss and associated disabilities; the audiological intervention was performed using the BAHA softband. The hearing aid was applied according to the international clinical protocols. The presented case reveals the importance of using the P1 CAEP biomarker in clinical practice along with a neuropsychological evaluation to assess the maturation of the central auditory pathways and to objectively quantify the results of auditory rehabilitation in children with hearing loss and associated disabilities.
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Vías Auditivas/fisiopatología , Biomarcadores/análisis , Evaluación de la Discapacidad , Potenciales Evocados Auditivos/fisiología , Pérdida Auditiva/fisiopatología , Niño , Femenino , Audífonos , HumanosRESUMEN
PURPOSE: The role of cochlear implant in deaf children development is well-known. However, the results are highly variable and depend on several factors. The most important role belongs to the family, family environment in which the child develops. The aim of the study was to evaluate the features of the family environment in which cochlear-implanted children develop. METHODS: The questionnaire "Family Environment Scale" was sent to 108 families who had cochlear-implanted child with more than 6 months of experience. One of the parents was asked to fill out the questionnaire which also included general information about the child and the family. RESULTS: A total of 58 families responded to the questionnaire and accepted to participate in the study. Mean values were higher compared with normal families in the areas of cohesion, expressiveness, intellectual-cultural orientation and organization, but the cohesion and organization score mean value exceeded the normal values (between 40 and 60). The independence and the conflict areas had lower values than normal families but did not exceed the lower limit of normal (40). CONCLUSIONS: The relationship between the family and the auditory-verbal development potential of the cochlear-implanted child is obvious. However, further studies are needed to establish correlations between the characteristics of the family environment and the level of auditory-verbal and psycho-cognitive development of the cochlear-implanted child so we can act on the family environment through education or therapy, to get maximum benefit with these children.
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Implantación Coclear , Implantes Cocleares , Sordera/terapia , Relaciones Familiares , Relaciones Interpersonales , Adolescente , Niño , Preescolar , Sordera/psicología , Femenino , Humanos , Masculino , Encuestas y CuestionariosRESUMEN
Middle ear aneurysms are rare and difficult to treat. The case of a 50-year-old female who presented with left otorrhagia caused by an internal carotid aneurysm is reported. She had no medical history of tinnitus, vertigo, otalgia or otorrhea. Middle ear surgery was effective in resolving bleeding and did not cause any permanent neurological deficit. High resolution computed tomography angiography is the technique of choice and, in some cases, can be complemented with a magnetic resonance angiography. Misdiagnosis of the internal carotid artery aneurysm may lead to serious morbidity because of bleeding or vascular occlusion. The use of modern imaging techniques explain the current relative increase in frequency.
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OBJECTIVES: Cochlear implantation is a well established treatment method in severe to profound hearing impaired people. Hearing devices do not cure hearing loss, but correct the disability, so it is important to assess the benefits not only on auditory-verbal performances but in terms of health-related quality of life. MATERIALS AND METHODS: We evaluated the health-related quality of life in a cochlear implant group (84 patients), split into two subgroups, according to the age of implantation and compared with a hearing aided group (50 patients). We used the Nijmegen cochlear implant HRQoL questionnaire which was sent to the parents. In the study group, all patients had unilateral MedEl device and at least 6 months of experience with the speech processor. RESULTS: Although there were differences between hearing aided and implanted children in all areas of quality of life, in the physical area, these differences were greater than those in the psychological and social domains. HRQoL was positively correlated with auditory performance, speech intelligibility and negatively correlated with implantation age. The correlation coefficient, R=0.78, indicates that between these three variables, implantation age, SIR and CAP and quality of life, there was a very good linear and direct proportional correlation. According to the determination coefficient (R(2) adjusted=0.59), 59.5% of quality of life's variation was explained by the variation of these three parameters. CONCLUSIONS: Cochlear implant improves the auditory performance and speech production much more than hearing aids. Associated diseases have a negative effect on the evolution of cochlear implanted children but the cochlear implant may have an important impact on these children quality of life. It is well known that children implanted at a young age evolve better than older ones, but we should take into consideration that even older children can get good results, good performances if they are properly selected and well trained.