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The aim of this study was to investigate the long-term audiological consequences of resistance to thyroid hormone (RTH) syndrome. The cochlea and inner ear express thyroid hormone receptor beta (THRB) in developmental stages. Hearing loss is frequent in subjects with RTH syndrome; however, the long-term impact of insufficient thyroid hormone action in the auditory system remains unknown. Subjects with RTH from the same family, carrying a THRB gene variant, underwent detailed clinical evaluation and serum biochemistry analysis. The genetic assessment involved sequencing of the THRB gene. Hearing loss assessment included (i) meatoscopy, (ii) audiometric tests using pure tone audiometry, (iii) middle ear evaluation by tympanometry, (iv) transient otoacoustic emissions (TOAE), and (v) computed tomography of the mastoids. Genetic sequencing confirmed the THRB gene alteration (p.M442T) in three family members. All affected subjects had clinical and laboratory RTH features. Notably, the older subject with RTH was affected by a bilateral sensorineural hearing loss pattern affected by high frequencies, and cochlear dysfunction was also presented by TOAE analysis, indicating pronounced hearing loss. Hearing loss represents a significant concern in subjects with RTH, emphasizing the need for continuous and comprehensive audiological assessments. These findings underscore the importance of lifelong monitoring, particularly to assess age-related progression of hearing impairment.

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OBJECTIVE: The objective of the study is to evaluate combined hyperbaric oxygen therapy (HBOT) and steroids on hearing in sudden sensorineural hearing loss (SSNHL) patients. METHOD: A total of 50 patients with sudden hearing loss that started within 1 week and who received a combination of intravenous steroid therapy and HBOT in their medical treatment were assigned to the otolaryngology department for 1 week, followed by intravenous steroid therapy at 1 mg/kg/day and then reduced doses for 1 week. They were treated once in a hyperbaric chamber where they breathed 100% oxygen at 2.5 atm pressure for 60 min, for a total of 20 sessions. RESULTS: Hearing loss was observed in 54% of participants in the right ear. Significant improvements were observed in hearing thresholds across all tested frequencies after treatment with a specific intervention (p < 0.001 for each comparison). Combined steroid and HBOT significantly improved hearing across low and high frequencies (p < 0.001). Improvement in hearing at low frequencies was significantly greater than at high frequencies (p < 0.01). Post hoc analysis showed greater hearing improvement at lower frequencies compared to higher ones. CONCLUSIONS: This study demonstrated that combined steroid and HBOT significantly improves hearing thresholds in patients with idiopathic SSNHL, especially at lower frequencies.

OBJETIVO: Evaluar la terapia combinada de oxígeno hiperbárico y corticosteroides en pacientes con pérdida auditiva sensorioneural súbita. MÉTODO: Se incluyeron 50 pacientes con pérdida auditiva súbita iniciada dentro de 1 semana, quienes recibieron un tratamiento médico combinando corticosteroides intravenosos y terapia de oxígeno hiperbárico. Fueron asignados al departamento de otorrinolaringología por 1 semana, seguido de corticosteroides intravenosos a dosis de 1 mg/kg al día y luego dosis reducidas por 1 semana. Se trató a los pacientes en una cámara hiperbárica en la que respiraron oxígeno al 100% a una presión de 2.5 atm durante 60 minutos, para un total de 20 sesiones. RESULTADOS: Se observó pérdida auditiva en el oído derecho en el 54% de los participantes. Se encontraron mejoras significativas en los umbrales auditivos en todas las frecuencias después del tratamiento con una intervención específica (p < 0.001 para cada comparación). La combinación de corticosteroides y terapia de oxígeno hiperbárico mejoró significativamente la audición en frecuencias bajas y altas (p < 0.001). La mejora en la audición en frecuencias bajas fue significativamente mayor que en altas (p < 0.01). Un análisis post hoc mostró una mayor mejora auditiva en las frecuencias bajas comparadas con las altas. CONCLUSIONES: Este estudio demostró que la terapia combinada de corticosteroides y oxígeno hiperbárico mejora significativamente los umbrales auditivos en pacientes con pérdida auditiva sensorioneural súbita idiopática, en especial en frecuencias bajas.

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PURPOSE: To verify the relationship between consistency in the use of hearing aids, auditory speech perception and vocabulary in children using hearing aids. METHODS: The population of Pereira's (2023) study was resumed and the database was analyzed containing information about the Speech Intelligibility Index (SII) for input sounds of 65 dBSPL (with or without hearing aids) of 29 children with neurosensorial hearing loss and hearing aid users bilaterally, with oral language, the number of hours per day of use of the device, four-tone average, results of the repetition of words with or without meaning in 65 dBSPL, Peabody Picture Vocabulary Test - PPVT. The consistency of the use was analyzed through measuring the auditory dosage which takes into account the average of use hours and the audibility with or without the device. RESULTS: The PPVT has a positive correlation with the SII with the device at 65 dBSPL. The four-tone average has a significant negative correlation with the SII in both conditions; and the same happens with the hours on daily usage. The dosage has a significant positive correlation with the SII and negative with the PTA. CONCLUSION: The receptive vocabulary tends to grow alongside with the audibility dosage increment. Results suggest that listening experience, involving the audibility with or without the device and the consistency of the device daily use must be considered throughout the whole process of the rehabilitation.

OBJETIVO: Verificar a relação entre consistência de uso, percepção da fala, e vocabulário receptivo em crianças usuárias de auxiliares de audição, especificamente aparelhos de amplificação sonora individual (AASI). MÉTODO: Foi retomada a população do estudo de Pereira (2023) e analisado o banco de dados com as informações referentes ao Índice de Inteligibilidade de Fala (SII) para sons de entrada de 65 dBNPS (com e sem AASI) de 29 crianças com perda auditiva neurossensorial usuárias de AASI bilateralmente, engajadas em língua oral, em famílias usuárias do português brasileiro. Foram analisados: o número de uso de horas por dia do dispositivo, média quadritonal, resultado da repetição de palavras com e sem sentido em 65 dBNPS, teste Peabody Picture Vocabulary Test ­ PPVT. A consistência de uso foi analisada por meio da medida da dosagem auditiva, que considera média de horas de uso e audibilidade com e sem AASI. RESULTADOS: O PPVT tem correlação positiva com o SII com AASI em 65 dBNPS. A média quadritonal tem correlação significativa negativa com o SII nas duas condições e o mesmo acontece com a média de horas diárias de uso. A dosagem tem correlação positiva significativa com os SII e negativa com a PTA. CONCLUSÃO: O vocabulário receptivo tende a crescer com o aumento da dosagem auditiva. Os resultados sugerem que a experiência auditiva, envolvendo a audibilidade sem e com AASI e a consistência de uso diário do dispositivo, deve ser considerada durante todo o processo de reabilitação.

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We present the case of a 37-year-old woman who underwent bilateral penetrating keratoplasty for congenital hereditary endothelial dystrophy at the age of 10 years. Over the subsequent 27 years, the patient's vision slowly deteriorated. Our examination revealed decompensation of the right corneal graft. We addressed this with regraft surgery. We then learned that the patient had been suffering from progressive hearing loss since adolescence. Tonal audiometry revealed hearing per ceptive deafness of 25 dB, which was more prominent in the left ear. Because the patterns of progressive sensorineural hearing loss and congenital hereditary endothelial dystrophy have both been linked to the same gene, slc4a11, we tested our patient for mutations in this gene. The test was positive for a heterozygous slc4a11 gene fifth exon mutation on chromosome 20p13-p12, which causes a frameshift. A combined clinical and genetic evaluation confirmed a diagnosis of Harboyan syndrome. After the genetic diagnosis of the disease, she was evaluated for the need for a hearing aid due to her hearing loss. The patient was also informed about genetic counseling.

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RESUMEN Se presenta el caso de una mujer joven, trabajadora de salud, residente en una región altoandina del Perú, con exposición reciente a animales de granja y artrópodos, que presenta síndrome febril agudo indiferenciado, trombocitopenia severa y extravasación pulmonar y abdominal. Posteriormente desarrolla meningitis e hipoacusia neurosensorial bilateral de instalación temprana y muestra serología reactiva a infección aguda por Rickettsias sp. Se discuten las consideraciones epidemiológicas y clínicas en el diagnóstico diferencial para un manejo oportuno.

ABSTRACT We present the case of a young female health worker, resident in a high Andean region of Peru, with recent exposure to farm animals and arthropods, who developed acute febrile undifferentiated syndrome, severe thrombocytopenia and pulmonary and abdominal extravasation. Subsequently, the patient developed meningitis and early onset bilateral neurosensorial hypoacusis and showed reactive serology to acute infection by Rickettsia sp. Epidemiological and clinical considerations in the differential diagnosis for early management are discussed.

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PURPOSE: The purpose of this study was to investigate the peripheral and central auditory pathways in adult individuals after COVID-19 infection. METHOD: A total of 44 individuals aged between 19 and 58 years, of both genders, post-COVID-19 infection, confirmed by serological tests, with no previous hearing complaints and no risk factors for hearing loss, were assessed. All the participants underwent the following procedures: pure tone audiometry, logoaudiometry, immitanciometry, and Brainstem Auditory Evoked Potentials (BAEP), in addition to answering a questionnaire about auditory symptoms. RESULTS: Thirteen individuals (29.5 %) had some hearing threshold impairment, mainly sensorineural hearing loss. In the BAEP, 18 individuals (40.9 %) presented longer latencies, mainly in waves III and V. According to the questionnaire answers, 3 individuals (9.1 %) reported worsened hearing and 7 (15.9 %) tinnitus that emerged after the infection. As for the use of ototoxic drugs during treatment, 7 individuals (15.9 %) reported their use, of which 5 showed abnormalities in peripheral and/or central auditory assessments. CONCLUSION: Considering the self-reported hearing complaints after COVID-19 infection and the high rate of abnormalities found in both peripheral and central audiological assessments, it is suggested that the new COVID-19 may compromise the auditory system. Due to the many variables involved in this study, the results should be considered with caution. However, it is essential that audiological evaluations are carried out on post-COVID-19 patients in order to assess the effects of the infection in the short, medium, and long term. Future longitudinal investigations are important for a better understanding of the auditory consequences of COVID-19.

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OBJECTIVE: To identify and characterize the population of Pediatric patients referred to our hyperbaric oxygen therapy center. METHODS: Retrospective and observational study, including pediatric patients treated with hyperbaric oxygen therapy, from 2006 to 2021, at the hyperbaric medicine reference center in the north of Portugal. Variables of interest were extracted from electronic medical records. RESULTS: Our study included 134 patients. The most frequent reasons for referral were carbon monoxide poisoning (n=59) and sudden sensorineural hearing loss (n=41). In 75 cases (56%), treatment was initiated in an urgent context. Symptom presentation at Emergency Department varied among patients, the most frequent being headache and nausea/vomiting. Concerning carbon monoxide poisoning, the most common sources were water heater, fireplace/brazier, and boiler. Regarding adverse effects, it was identified one case of intoxication by oxygen and four cases of middle ear barotrauma. CONCLUSIONS: The most frequent cause for referral was carbon monoxide poisoning. All patients evolved favorably, with few side effects being reported, emphasizing the safety of this therapy. While most pediatricians may not be aware of the potential benefits arising with hyperbaric oxygen therapy, it is of upmost importance to promote them, so that this technique is increasingly implemented.

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BACKGROUND: Sensory information obtained from the visual, somatosensory, and vestibular systems is responsible for regulating postural control, and if damage occurs in one or more of these sensory systems, postural control may be altered. OBJECTIVE: To evaluate and compare the postural sway velocity between children with normal hearing and with sensorineural hearing loss (SNHL), matched by sex and age group, and to compare the postural sway velocity between children with normal hearing and with SNHL, with and without vestibular dysfunction. METHODS: Cross-sectional study that evaluated 130 children (65 with normal hearing and 65 with SNHL), of both sexes and aged between 7 and 11 years, from public schools of the city of Caruaru, Pernambuco state, Brazil. The postural sway velocity of the center of pressure (COP) was assessed by a force platform, in two directions, anteroposterior (AP) and mediolateral (ML)), in three positions, namely bipedal support with feet together and parallel (parallel feet (PF)), bipedal support with one foot in front of the other (tandem foot (TF)), and single-leg support (one foot (OF)), evaluated with the eyes open and closed. RESULTS: Children with SNHL demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, with significant differences in the AP direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.003) and closed (PF: p = 0.050; TF: p = 0.005). The same occurred in the ML direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.001) and closed (PF: p = 0.002; TF: p = 0.000). The same occurred in relation to vestibular function, where the children with SNHL with an associated vestibular dysfunction demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, demonstrating significant differences in the AP direction, with the eyes open (TF: p = 0.001; OF: p = 0.029) and eyes closed (PF: p = 0.036; TF: p = 0.033). The same occurred in the ML direction, with the eyes open (TF: p = 0.000) and with the eyes closed (PF: p = 0.008; TF: p = 0.009). CONCLUSIONS: Children with SNHL demonstrated greater instability of postural control than children with normal hearing in all the directions assessed. Children with SNHL and an associated vestibular dysfunction demonstrated the greatest instability of postural control in this study.

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BACKGROUND: The cochlear implant (CI) is effective for rehabilitating patients with severe to profound sensorineural hearing loss. However, its placement and use have been associated with various complications, such as those affecting the vestibular system. The objective of this study was to compare vestibular function using the video head impulse test (vHIT) in pediatric patients before and after CI placement. METHODS: A descriptive and retrospective study was conducted. The outcomes of 11 pediatric patients of both sexes with a history of profound hearing loss were evaluated. The results of vestibular-ocular reflex (VOR) gain, saccades, asymmetry, Pérez Rey (PR) index, and VOR/saccade ratio for both ears obtained by the vHIT test before and after CI placement were compared. RESULTS: Of the 11 patients evaluated, the VOR gain showed that 81.8% had normal function, 18.2% had hypofunction, and no patients had hyperfunction before implantation. No statistically significant differences were found when compared with post-implant off and post-implant on conditions (p > 0.05). The extracted variables, asymmetry, PR index, and the VOR/saccades ratio also showed no statistically significant differences between the pre- and post-implant conditions, whether off or on. CONCLUSIONS: The vestibular function of pediatric patients did not show significant changes before and after CI placement. The vHIT test is a valuable tool for assessing vestibular function and could be considered a criterion for surgical and rehabilitation decisions in patients undergoing CI placement.

INTRODUCCIÓN: El implante coclear es un dispositivo eficaz para la rehabilitación de pacientes con hipoacusia neurosensorial severa a profunda. Sin embargo, su colocación y uso se ha asociado a diversas complicaciones, entre ellas a nivel del sistema vestibular. El objetivo del presente estudio fue comparar la función vestibular mediante la prueba de videoimpulso cefálico (vHIT) de pacientes pediátricos antes y después de la colocación del implante coclear. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo. Se evaluaron los resultados de 11 pacientes pediátricos de ambos sexos con antecedente de hipoacusia profunda. Se compararon los resultados de ganancia del VOR, sacadas, asimetría, índice PR así como la relación VOR/sacadas para ambos oídos obtenidos mediante la prueba vHIT antes y después de la colocación del implante coclear. RESULTADOS: De los 11 pacientes evaluados, la ganancia del VOR mostró que el 81.8% tenía normofunción, 18.2% hipofunción y ningún paciente hiperfunción antes del implante. Al compararlo con la ganancia post implante apagado y post implante encendido no se encontraron diferencias estadísticamente significativas (p > 0.05). Las variables sacadas, asimetría, índice PR así como la relación VOR/sacadas tampoco mostraron diferencias estadísticamente significativas entre las condiciones pre y pos implante ya sea apagado o encendido. CONCLUSIONES: La función vestibular de pacientes pediátricos no mostró cambios significativos previo y posterior a la colocación del implante coclear. La prueba vHIT es una herramienta útil que permite evaluar la función vestibular y que podría considerarse como criterio para tomar decisiones quirúrgicas en pacientes que se encuentran en protocolo para implante coclear.

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PURPOSE: To verify the influence of verbal intellectual-cognitive skills on speech perception in noise, in elderly with sensorineural hearing loss, considering education, age, and degree of hearing loss. METHODS: 36 elderly between 60 and 89 years old with bilateral sensorineural hearing loss participated in the study. After psychological assessment using the Wechsler Intelligence Scale for Adults (WAIS-III), they were grouped into (GI) 24 elderly without cognitive alteration and (GII) 12 elderly with risk of cognitive alteration. They underwent otorhinolaryngological assessment, audiological interview, pure tone audiometry, and assessment of speech perception in noise using the Hearing in Noise Test (HINT-Brazil). The Mann-Whitney U statistical test compared the results between the groups, and the Spearman correlation verified the variable's age, degree of hearing loss, and level of education. RESULTS: There was no difference between the groups in the ability to perceive speech in noise, except in the noise on the left condition, in which GII showed better performance in HINT-Brazil. The degree of hearing loss and level of education influenced the perception of speech in noise. The level of education was correlated with the WAIS-III results. CONCLUSION: The decline in verbal intellectual-cognitive skills did not affect speech perception of noise in the elderly with hearing loss. The degree of hearing loss and level of education influenced the performance of the elderly in the speech perception test in noise. Performance in verbal cognitive skills varied according to the level of education.

OBJETIVO: Verificar a influência das habilidades intelectuais-cognitivas verbais na percepção de fala no ruído, em idosos com perda auditiva sensorioneural, considerando a escolaridade, a idade e o grau da perda auditiva. MÉTODO: Participaram 36 idosos entre 60 e 89 anos com perda auditiva sensorioneural bilateral, que após avaliação psicológica por meio do Wechsler Intelligence Scale for Adults (WAIS III), foram divididos em (GI) 24 idosos sem alteração cognitiva e (GII) 12 idosos com risco de alteração cognitiva. Foram submetidos à avaliação otorrinolaringológica, entrevista audiológica, audiometria tonal liminar e a avaliação da percepção de fala no ruído por meio do Hearing in Noise Test (HINT-Brasil). O teste estatístico U de Mann-Whitney comparou os resultados entre os grupos, e a correlação de Spearman verificou as variáveis idade, grau da perda auditiva e nível de escolaridade. RESULTADOS: Não houve diferença entre os grupos na habilidade de percepção de fala no ruído, exceto na condição ruído à esquerda, no qual o GII apresentou melhor desempenho no HINT-Brasil. O grau da perda auditiva e o nível de escolaridade influenciaram na percepção de fala no ruído. O nível de escolaridade teve correlação com os resultados do WAIS III. CONCLUSÃO: O declínio das habilidades intelectuais-cognitivas verbais não interferiu na percepção de fala no ruído nos idosos com perda auditiva. O grau da perda auditiva e o nível de escolaridade influenciaram no desempenho dos idosos no teste de percepção de fala no ruído. O desempenho nas habilidades cognitivas verbais variou com o nível de escolaridade.

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OBJECTIVE: The objective of the study is to compare the optic coherence tomography (OCT) parameters of the healthy and affected sides of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) and to investigate the relationships between these and the improvement in hearing levels. METHODS: A bilateral eye evaluation of patients diagnosed with ISSNHL was performed with OCT. The ganglion cell complex (GCC) and retina nerve fiber layer (RNFL) thickness values were recorded and the differences between the two eyes were examined. RESULTS: An evaluation was made of 39 patients with a mean age of 44.82 ± 14.90 years. The RNFL thickness of the eyes was determined to be mean 89.87 ± 3.65 µm on the affected side and 103.87 ± 3.98 µm on the healthy control side (p = 0.0001). The mean GCC was determined to be mean 90.46 ± 3.49 µm on the affected side and 103.77 ± 3.96 µm on the healthy control side (p = 0.0001). CONCLUSIONS: A statistically significant difference was observed between the healthy and affected eyes of patients with ISSNHL with respect to mean GCC and mean RNFL thickness. OCT could be a useful technique for measuring this neural degeneration.

OBJETIVO: Comparar e investigar los parámetros de la tomografía de coherencia óptica (OCT) de los lados sanos y afectados de pacientes con pérdida auditiva neurosensorial súbita idiopática (PANSI). MÉTODO: La evaluación ocular bilateral de los pacientes diagnosticados con PANSI se realizó con OCT. Se registraron los valores de espesor del complejo de células ganglionares (CCG) y de la capa de fibras nerviosas de la retina (CFNR), y se examinaron las diferencias entre los dos ojos. RESULTADOS: Se evaluaron 39 pacientes, con una edad media de 44.82 ± 14.90 años. Se determinó que el grosor de la CFNR de los ojos era una media de 89.87 ± 3.65 µm en el lado afectado y 103.87 ± 3.98 µm en el lado de control sano (p = 0.0001). Se determinó que el CCG medio era 90.46 ± 3.49 µm en el lado afectado y 103.77 ± 3.96 µm en el lado de control sano (p = 0.0001). CONCLUSIONES: Se encontró una diferencia estadísticamente significativa entre los ojos sanos y afectados de pacientes con PANSI con respecto al CCG medio y al espesor medio de la CFNR. La OCT podría ser una técnica útil para medir esta degeneración neuronal.

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OBJECTIVES: Research focusing on changes in the clinical practice of audiological diagnosis has become increasingly necessary, particularly in pediatric audiology. The pursuit of accurate and reliable examinations has intensified given the importance of early detection and intervention in cases of childhood hearing loss. Thus, this study aims to investigate the correlation between electrophysiological auditory thresholds, as obtained through frequency-specific auditory brainstem responses with two distinct chirp stimuli (narrow-band CE-Chirp Level Specific and narrow-band iChirp), in children with hearing impairments. In addition, this research set out to correlate these thresholds with behavioral responses while simultaneously comparing the examination durations relative to the type of stimuli and the degree of hearing loss. DESIGN: A cohort of 20 children (aged 6 months to 12 years) with varying degrees of hearing impairment (ranging from mild to profound) were recruited. The participants underwent bilateral measurement of their electrophysiological thresholds via auditory brainstem responses across different frequencies (500, 1000, 2000, and 4000 Hz), and the timeframe for determining these thresholds was carefully recorded. Subsequently, behavioral thresholds were ascertained using pure-tone audiometry or visual reinforcement audiometry based on the child's age. The data collected was subsequently analyzed using Pearson and Spearman correlation coefficients. To compare examination times, the Student t test and the Kruskal-Wallis test were used. RESULTS: There was a pronounced correlation between the thresholds obtained through both narrow-band chirp stimuli. Moreover, a substantial correlation was found between electrophysiological and behavioral thresholds at 1000, 2000, and 4000 Hz, especially when compared with pure-tone audiometry. The mean differences between the electrophysiological and behavioral thresholds were below 6 dB nHL, and the exam duration was relatively consistent across both devices, averaging 47.63 (±19.41) min for the narrow-band CE-Chirp Level Specific and 52.42 (±26) min for the narrow-band iChirp. Notably, variations in exam duration did not relate to varying degrees of hearing loss when using the narrow-band CE-Chirp Level Specific. Nevertheless, the narrow-band iChirp indicated significantly shorter durations in instances of profound degree measurements, demonstrating a statistically significant difference. CONCLUSIONS: The narrow-band CE-Chirp Level Specific and narrow-band iChirp stimuli provided similar estimates of electrophysiological auditory thresholds in children with hearing impairments, giving accurate estimations of behavioral thresholds. The time it took to complete the assessment is comparable between both stimuli. For the narrow-band iChirp, the degree of hearing loss was shown to impact the testing time, and children with profound hearing loss underwent faster exams. Ultimately, this study exhibits significant clinical implications as it reveals that the narrow-band CE-Chirp Level Specific and narrow-band iChirp stimuli could be remarkably promising for clinically exploring electrophysiological thresholds in children with hearing impairments.

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OBJECTIVE: The purpose was to explore the effects of traditional and non-traditional lipid parameters on Sudden Sensorineural Hearing Loss (SSNHL). METHODS: The study included 452 patients diagnosed with SSNHL, among whom 206 patients had a level of hearing improvement ≥10 dB after one month of follow-up. A propensity score-matched (2:1) control group was used. Conditional and unconditional logistic regression were used to analyze the risk factors for SSNHL. RESULTS: Patients with SSNHL had a higher risk of concomitant hypertension and elevated atherosclerogenic lipid levels, with apolipoprotein B and apolipoprotein E identified as independent risk factors for the onset of SSNHL. Additionally, the Lipid Comprehensive Index (LCI) was an independent risk factor for the degree of hearing loss. A positive linear correlation was revealed between triglyceride, non-high-density lipoprotein cholesterol, atherogenic index, Castelli risk index, atherogenic index of plasma, LCI and hearing loss. However, no linear relationship was observed between hearing gain and any lipid parameters. When Total Cholesterol (TC) was in the range of borderline high, the treatment effect was the best. However, the statistical significance disappeared upon adjusting for confounding factors. CONCLUSION: Patients with SSNHL exhibited markedly dysregulated lipid metabolism. Elevated serum lipid levels may be a causative factor in auditory impairment and can influence the extent of hearing loss. Promptly improving cochlear microcirculation may benefit patients with borderline elevated TC.

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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.

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INTRODUCTION: Studies have identified a greater risk of sensory neural hearing loss in individuals with chronic obstructive pulmonary disease (COPD) compared to healthy individuals, but it is unclear whether they are at increased risk of hearing loss with impaired speech recognition. The aim of this study was to assess whether COPD is associated with hearing loss that affects speech recognition. METHODS: This is a case-control study. We screened individuals from health facilities in the municipality of Jundiai. We enrolled a test group of individuals with COPD and an age-matched control group composed of individuals with asthma. The selected individuals attended an appointment with a chest physician, responded to questionnaires, and underwent tonal and speech audiometry. Adjusted binary logistic regression analysis evaluated whether COPD was associated with reduced speech recognition. RESULTS: We enrolled 36 individuals with COPD and 72 with asthma. Individuals with COPD were more likely to have a reduced speech recognition compared to asthmatic individuals (reduced recognition of three-syllable words: adjusted OR 3.72, 95 CI [1.38-10.02]) (reduced recognition of monosyllable words: adjusted OR 4.74, 95 CI [1.52-14.76]). CONCLUSION: We conclude that individuals with COPD from primary and secondary healthcare facilities have at least 38% greater risk of hearing loss with reduced speech recognition compared to an age-matched control group of individuals with asthma recruited from the same facilities. We recommend that longitudinal studies evaluate whether regular screening could contribute to the prevention or early treatment of hearing loss in individuals with moderate-severe COPD.

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Hereditary connective tissue disorders include more than 200 conditions affecting different organs and tissues, compromising the biological role of the extracellular matrix through interference in the synthesis, development, or secretion of collagen and/or its associated proteins. The clinical phenotype includes multiple signs and symptoms, usually nonspecific but of interest to rheumatologists because of musculoskeletal involvement. The patient´s journey to diagnosis is long, and physicians should include these disorders in their differential diagnoses of diseases with systemic involvement. In this review, insights for the diagnosis and treatment of osteogenesis imperfecta, hypermobility spectrum disorder/Ehlers-Danlos syndrome, Marfan, Loeys-Dietz, and Stickler syndromes are presented.

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OBJECTIVE: To analyze the results of auditory assessment in standard (SA) and extended high-frequency (EHF) audiometry, associating the findings with sudden tinnitus and mental health of patients with unilateral sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Prospective, cohort study. SETTING: Outpatient otology clinic in a tertiary care hospital. SUBJECTS AND METHODS: Patients experiencing unilateral SSNHL were evaluated with pure-tone audiometry performed at frequencies of 250 to16,000 Hz, tinnitus pitch and loudness matching tests, Tinnitus Handicap Inventory (THI), Analog and Visual Scale (AVS) for bothersome tinnitus, and the Hospital Anxiety and Depression Scale (HADS). RESULTS: Eighteen patients with unilateral SSNHL were assessed. After starting treatment, there was a significant improvement in the SA (71.1 dB to 50 dB; p < 0.001*) and EHF audiometry (64.5 dB to 54.4 dB; p < 0.001*) thresholds at 15 days, and this persisted at 30 days of follow-up. Significant improvements were seen for tinnitus in loudness, VAS, and THI and for mental health in the realms of anxiety and depression by HADS. Despite improvements in SA, persistent EHF hearing loss was accompanied by persistent tinnitus, but it was of diminished loudness. CONCLUSION: Despite improvement in pure-tone thresholds by SA, a subset of unilateral SSNHL patients did not experience hearing recovery in EHF thresholds and reported persistent tinnitus. We postulate that their diminished anxiety and better mental health may be related to both hearing improvement in standard audiometry and reduction in tinnitus loudness. This pilot prospective study investigates the utility of performing EHF audiometry to better understand outcomes in patients with SSNHL.

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El citomegalovirus congénito (CMVc) es la infección congénita más común y la principal causa no genética de hipoacusia congénita. Gran parte de los recién nacidos (RN) con CMVc sintomático desarrolla secuelas graves permanentes, donde la hipoacusia es la más frecuente. Sin embargo, el 90% de los casos se presenta en forma asintomática, pudiendo desarrollar secuelas auditivas tardías. El diagnóstico precoz de CMVc requiere un alto índice de sospecha. Actualmente, técnicas eficientes para su detección están disponibles, lo que facilita el diagnóstico en las primeras 3 semanas de vida. La terapia antiviral es la primera línea de tratamiento para el CMVc sintomático, logrando buenos resultados auditivos. A pesar de los avances en los métodos de detección y beneficios del tratamiento, los RN no son tamizados para CMVc. El tamizaje selectivo de CMVc en pacientes que no pasan el screening auditivo facilita la intervención precoz en los casos identificados, pero no permite detectar el número significativo de niños que presenta hipoacusia de aparición tardía. El tamizaje universal permite hacer seguimiento auditivo a los pacientes en riesgo de desarrollar hipoacusia sensorioneural (HSN) por CMVc, identificando así los casos de hipoacusia de aparición tardía, pero la costo-efectividad es aún controversial. Es necesario avanzar en una estrategia local para el tamizaje de CMVc, buscando reducir su impacto a nivel nacional.

Congenital cytomegalovirus (cCMV) is the most common congenital infection and the main non-genetic cause of congenital hearing loss. A significant number of newborns (NB) with symptomatic cCMV will develop permanent serious sequelae, being hearing loss the most frequent. However, 90% of the cases are asymptomatic and may develop late auditory sequelae. Early diagnosis of cCMV requires a high index of suspicion. Currently, efficient detection techniques for its detection are available, which facilitates diagnosis within the first 3 weeks of life. Antiviral therapy is the first line of treatment for symptomatic cCMV, achieving good hearing results. Despite advances in detection methods and the benefits of antiviral therapy, NB are not routinely screened for cCMV. Selective screening for cCMV in patients who fail newborn hearing screening facilitates early intervention in identified cases but fails to detect a significant number of children with late onset hearing loss. Universal screening allows hearing follow up in patients at risk of developing sensorineural hearing loss (SNHL) due to cCMV, thus identifying late-onset hearing loss cases, but cost-effectiveness is still controversial. It is necessary to advance in a local strategy for cCMV screening, aiming to reduce its national impact.

RESUMO

OBJECTIVE: To assess the efficacy of valganciclovir in infants with hearing loss and clinically inapparent congenital cytomegalovirus infection (cCMV), as there is no consensus on treatment of this group. STUDY DESIGN: A nationwide, nonrandomized controlled trial, comparing 6 weeks of oral valganciclovir to no treatment in infants with cCMV, recruited after newborn hearing screening resulted in referral to an audiologist. The choice whether to treat was left to parents of subjects. Eligible subjects were full term infants aged <13 weeks with sensorineural hearing loss and diagnosed with cCMV through dried blood spot testing. The primary outcome, measured by linear and ordinal logistic regression, was change in best-ear hearing from baseline to follow-up at 18-22 months of age. RESULTS: Thirty-seven participants were included in the final analysis, of whom 25 were in the treatment group and 12 in the control group. The majority of subjects in both groups had neuroimaging abnormalities, which were mostly mild. Hearing deterioration was more likely in the control group compared with the treatment group (common OR 0.10, 95% CI 0.02-0.45, P = .003). Mean best-ear hearing deteriorated by 13.7 dB in the control group, compared with improvement of 3.3 dB in the treatment group (difference 17 dB, 95% CI 2.6 - 31.4, P = .02). CONCLUSIONS: We investigated treatment in children with hearing loss and clinically inapparent cCMV. Although our study was nonrandomized, it is the first prospective and controlled trial in this population. Valganciclovir-treated children with hearing loss and inapparent cCMV had less hearing deterioration at 18 through 22 months of age than control subjects. EUDRACT REGISTRY NUMBER: 2013-003068-30.

RESUMO

OBJECTIVE: The objective of this study was to determine if valganciclovir initiated after 1 month of age improves congenital cytomegalovirus-associated sensorineural hearing loss. STUDY DESIGN: We conducted a randomized, double-blind, placebo-controlled phase 2 trial of 6 weeks of oral valganciclovir at US (n = 12) and UK (n = 9) sites. Patients of ages 1 month through 3 years with baseline sensorineural hearing loss were enrolled. The primary outcome was change in total ear hearing between baseline and study month 6. Secondary outcome measures included change in best ear hearing and reduction in cytomegalovirus viral load in blood, saliva, and urine. RESULTS: Of 54 participants enrolled, 35 were documented to have congenital cytomegalovirus infection and were randomized (active group: 17; placebo group: 18). Mean age at enrollment was 17.8 ± 15.8 months (valganciclovir) vs 19.5 ± 13.1 months (placebo). Twenty (76.9%) of the 26 ears from subjects in the active treatment group did not have worsening of hearing, compared with 27 (96.4%) of 28 ears from subjects in the placebo group (P = .09). All other comparisons of total ear or best ear hearing outcomes were also not statistically significant. Saliva and urine viral loads decreased significantly in the valganciclovir group but did not correlate with change in hearing outcome. CONCLUSIONS: In this randomized controlled trial, initiation of antiviral therapy beyond the first month of age did not improve hearing outcomes in children with congenital cytomegalovirus-associated sensorineural hearing loss. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT01649869.