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Abstract 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.

Resumen 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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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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INTRODUCTION AND OBJECTIVES: Even though the incidence has decreased in recent years, Down syndrome (DS) remains the most common chromosomal disorder today. Despite being a condition with multisystemic involvement, it often tends to affect the head and neck area, making it a frequent reason for consultation with pediatric otolaryngologists or otologists. The purpose of this work is to be one of the first in Spain to characterize and describe the pathology and therapeutic approach typically provided to these patients, analyzing the evolution from a clinical and auditory perspective. MATERIAL AND METHODS: We aim to analyze a sample of 16 pediatric patients recruited over the past 24 years, diagnosed with Down syndrome, and experiencing a wide range of diseases affecting the ear and its auditory function. RESULTS: 62.50% of the patients were women, whose main reason for seeking specialist care was acute and serous otitis media, accounting for 31.25%. These patients have an indication for treatment for various entities within the otological sphere that usually do not differ from those of a healthy child. However, the evolution and response to treatments can take on a torpid character due to the anatomical characteristics of the ears of these patients. CONCLUSIONS: Although the frequency of children with DS in the pediatric otolaryngologist's clinic is decreasing, these patients have a predisposition to ear diseases with auditory repercussions, with variable evolution depending on the disease and the child's intrinsic characteristics.

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BACKGROUND AND OBJECTIVE: Sound localization plays a crucial role in our daily lives, enabling us to recognize voices, respond to alarming situations, avoid dangers, and navigate towards specific signals. However, this ability is compromised in patients with Single-Sided Deafness (SSD) and Asymmetric Hearing Loss (AHL), negatively impacting their daily functioning. The main objective of the study was to quantify the degree of sound source localization in patients with single-sided deafness or asymmetric hearing loss using a Cochlear Implant (CI) and to compare between the two subgroups. MATERIALS AND METHODS: This was a prospective, longitudinal, observational, single-center study involving adult patients diagnosed with profound unilateral or asymmetric sensorineural hearing loss who underwent cochlear implantation. Sound localization was assessed in a chamber equipped with seven speakers evenly distributed from -90º to 90º. Stimuli were presented at 1000 Hz and intensities of 65 dB, 70 dB, and 75 dB. Each stimulus was presented only once per speaker, totaling 21 presentations. The number of correct responses at different intensities was recorded, and angular error in degrees was calculated to determine the mean angular distance between the patient-indicated speaker and the speaker presenting the stimulus. Both assessments were conducted preoperatively without a cochlear implant and two years post-implantation. RESULTS: The total sample comprised 20 patients, with 9 assigned to the SSD group and 11 to the AHL group. The Preoperative Pure Tone Average (PTA) in free field was 31.7 dB in the SSD group and 41.8 dB in the AHL group. There was a statistically significant improvement in sound localization ability and angular error with the use of the cochlear implant at all intensities in both SSD and AHL subgroups. CONCLUSIONS: Cochlear implantation in patients with SSD and AHL enhances sound localization, reducing mean angular error and increasing the number of correct sound localization responses.

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

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

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Introducción: El schwannoma vestibular (SV) es el tumor más frecuente del ángulo pontocerebeloso. La mayor accesibilidad a las pruebas radiológicas ha incrementado su diagnóstico. Teniendo en cuenta las características del tumor, la clínica y la edad del paciente se han propuesto tres estrategias terapéuticas, observación, cirugía o radioterapia. La elección de la más adecuada para cada paciente es un motivo de controversia frecuente. Material y métodos: El presente trabajo incluye una revisión exhaustiva sobre cuestiones relativas al SV que pueden servir de guía clínica en el manejo de pacientes con estas lesiones. La presentación se ha orientado en forma de preguntas que el clínico se hace habitualmente y las respuestas están redactadas y/o revisadas por un panel de expertos nacionales e internacionales consultados por la Comisión de Otología de la SEORL-CCC. Resultados: Se ha elaborado un listado con los 13 bloques temáticos más controvertidos sobre el manejo del SV en forma de 50 preguntas y se han buscado las respuestas a todas ellas mediante una revisión sistemática de la literatura (artículos publicados en PubMed y Cochrane Library entre 1992 y 2023 sobre cada bloque temático). Treinta y tres expertos, liderados por la Comisión de Otología de la SEORL-CCC, han analizado y discutido todas las respuestas. En el Anexo 1 pueden encontrarse 14 preguntas adicionales divididas en cuatro bloques temáticos. Conclusiones: Esta guía de práctica clínica sobre el manejo del SV ofrece respuestas consensuadas a las preguntas más habituales que se plantean sobre este tumor. La ausencia de suficientes estudios prospectivos hace que los niveles de evidencia sobre el tema sean en general medios o bajos. Este hecho incrementa el interés de este tipo de guías de práctica clínica elaboradas por expertos.(AU)

IntroductionVestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. Material and methods: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. Results: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. Conclusions: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.(AU)

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RESUMEN Introducción: La pérdida auditiva afecta a millones globalmente, especialmente a jóvenes que usan auriculares frecuentemente. Objetivo: Establecer la relación entre el uso de auriculares y la audición en estudiantes de medicina en Lima, Perú. Métodos: Se realizó un estudio observacional, analítico y transversal en la Universidad Nacional Federico Villarreal. Participaron 98 estudiantes de medicina (18-32 años) que usaban auriculares regularmente. Se excluyeron aquellos con patologías auditivas previas. La recolección de datos incluyó encuestas sobre el uso de auriculares y audiometrías realizadas en una cabina silente. Los resultados se analizaron mediante estadística descriptiva e inferencial, utilizando el coeficiente de correlación de Rho de Spearman para evaluar la relación entre las variables. Resultados: El 59,2% de los estudiantes eran mujeres. El 39,2% presentó hipoacusia leve a tonos agudos, mientras que el 36,2% tenía audición normal. El 71,4% reportó disminución de la capacidad auditiva y el 56,1% experimentó otalgia. El uso de auriculares mostró una correlación positiva y significativa con la audición (Rho = 0,298, p = 0,003). El tiempo de exposición también fue significativo (Rho = 0,260, p = 0,010), pero la intensidad del sonido no lo fue (Rho = 0,193, p = 0,057). Conclusión: Existe una relación positiva entre el uso de auriculares y la pérdida auditiva en estudiantes de medicina. Se recomienda implementar programas de concienciación sobre el uso seguro de auriculares y realizar evaluaciones auditivas periódicas para prevenir el deterioro auditivo.

ABSTRACT Introduction: Hearing loss affects millions globally, especially young people who frequently use headphones. Objective: To establish the relationship between headphone use and hearing among medical students in Lima, Peru. Methods: An observational, analytical, and cross-sectional study was conducted at the Universidad Nacional Federico Villarreal. Participants included 98 medical students (18-32 years) who regularly used headphones. Those with previous hearing pathologies were excluded. Data collection included surveys on headphone use and audiometries performed in a silent booth. Results were analyzed using descriptive and inferential statistics, employing the Spearman's Rho correlation coefficient to assess the relationship between variables. Results: 59.2% of the students were women. 39.2% had mild hearing loss at high tones, while 36.2% had normal hearing. 71.4% reported decreased hearing capacity, and 56.1% experienced ear pain. Headphone use showed a positive and significant correlation with hearing (Rho = 0.298, p = 0.003). Exposure time was also significant (Rho = 0.260, p = 0.010), but sound intensity was not (Rho = 0.193, p = 0.057). Conclusion: There is a positive relationship between headphone use and hearing loss in medical students. It is recommended to implement awareness programs on safe headphone use and conduct regular hearing evaluations to prevent auditory deterioration.

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Introducción y objetivo: El schwanoma vestibular es un tumor benigno, de lento crecimiento que aparece en la vaina de mielina que rodea al nervio vestibular. Estos tumores representan el 6% de todos los tumores intracraneales y el 85% de los tumores del ángulo pontocerebeloso. El síntoma de aparición más frecuente es la hipoacusia unilateral, seguida del acúfeno unilateral, inestabilidad, vértigo, cefalea e incluso, en determinados casos, parestesias faciales o parálisis facial. Todo ello depende del tamaño del tumor y su localización. Actualmente, el diagnóstico de estos tumores se realiza mediante la realización de una historia clínica completa, pruebas complementarias audiológicas y vestibulares y, como prueba de imagen, una RMN. Según el American College of Radiology (ACR) la RMN de base de cráneo y conductos auditivos internos es la prueba de elección para el diagnóstico del schwanoma vestibular. Ésta puede ser con y sin contraste (generalmente Gadolinio) y permite detectar tumores de muy pequeño tamaño. Nuestro objetivo con este trabajo es aportar evidencia científica que permita al profesional seguir un protocolo diagnóstico de los schwanomas vestibulares y, consecuentemente, optimizar los recursos hospitalarios. Método: De una muestra total de 685 se revisaron todas las historias clínicas de los pacientes a los que se les había solicitado una RMN por síntomas audiovestibulares (hipoacusia, acúfeno, vértigo, parálisis facial y otros). Se llevó a cabo un estudio descriptivo y observacional en el cual se mostraban los síntomas que había padecido cada paciente, el motivo de petición de la prueba de imagen, el diagnóstico final y el tipo de resonancia magnética empleada. Con toda esta información se creó una base de datos y se analizaron los resultados estadísticamente. Resultados: ... (AU)

Introduction and objective: Vestibular schwannoma is a benign, slow-growing tumor that appears in the myelin sheath surrounding the vestibular nerve. These tumors represent 6% of all intracranial tumors and 85% of tumors in the cerebellopontine angle. The most common initial symptom is unilateral hearing loss, followed by unilateral tinnitus, instability, vertigo, headache, and, in certain cases, facial paresthesia or facial paralysis. All of these symptoms depend on the size and location of the tumor. Currently, the diagnosis of these tumors is made through a complete medical history, complementary audiological and vestibular tests, and, as an imaging test, an MRI. According to the American College of Radiology (ACR), the MRI of the skull base and internal auditory canals is the gold standard for diagnosing vestibular schwannoma. This can be performed with and without contrast (usually Gadolinium) and allows the detection of very small tumors. Our objective with this article is to provide scientific evidence that enables professionals to diagnose vestibular schwannomas and optimize hospital resources. Method: From a total sample of 685 patients, all medical records of them who had been requested an MRI for audiovestibular symptoms (hearing loss, tinnitus, vertigo, facial paralysis, and others) were reviewed. A descriptive and observational study was carried out, showing the symptoms experienced by each patient, the reason for requesting the imaging test, the final diagnosis, and the type of MRI used. With all this information, a database was created, and the results were analyzed statistically. Results: ... (AU)

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Introducción: La hipoacusia súbita (HS) es poco frecuente y su etiopatogenia no está definida. La terapia con corticoides es de elección en base a recomendaciones de expertos por sus efectos teóricos y no en base a utilidad clínica demostrada. Objetivo: Evaluar si existe correlación entre el resultado auditivo final, de pacientes con HS tratados con corticoides, y la respuesta in vitro de sus leucocitos a corticoides, medida como diferencias en la expresión relativa de genes blanco del receptor de glucocorticoides. Material y Método: Estudio de casos (recuperación total) y controles (no recuperados) de pacientes con HS tratados con corticoides en el Hospital Clínico de la Universidad de Chile, durante 2017-2019. Se obtuvo DNA que fue almacenado en el Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Se purificaron y cultivaron leucocitos mononucleares de sangre periférica, expuestos in vitro a hidrocortisona. Se determinó la diferencia en la expresión relativa de genes blanco (IGFBP1, CAT, HSD17B12, APOA2), por Q-RTPCR, entre ambos grupos. Resultados: Se reclutaron 35 pacientes; se incluyeron para análisis 23: 11 casos y 12 controles, con edad promedio de 54,9 y 50,8 años respectivamente, distribución homogénea de sexo. No hubo diferencia estadísticamente significativa en la expresión relativa de los genes blanco, a la exposición in vitro a corticoides, entre ambos grupos. Conclusión: En nuestro estudio, modelo, y sistema de evaluación no se evidenciaron efectos de los corticoides. No podemos descartar que, con un número mayor de pacientes, otros genes blanco u otros protocolos de estudio podrían detectarse diferencias.

Introduction: Sudden hearing loss (SHL) is rare and its etiopathogenesis is still not clear. Corticosteroid therapy is of choice based on expert recommendations due to its theoretical effects and no based on proved clinical efficacy. Objectives: To assess whether there is a correlation between the final auditory outcome of patients with SHL treated with corticosteroids and the in vitro response of their leukocytes to corticosteroids, measured as differences in the relative expression of glucocorticoid receptor target genes. Material and Method: Case-control (total recovery and not recovered respectively) study of patients with SHL treated with corticosteroids at Clinical Hospital Universidad de Chile between 2017 and 2019. DNA was obtained and stored in the Biobanco de Tejidos y Fluidos de la Universidad de Chile (BTUCH). Peripheral blood mononuclear leukocytes were purified and cultured and then exposed to hydrocortisone. The difference in the relative expression of target genes (GFBP1, CAT, HSD17B12, APOA2), by Q-RTPCR was determined. Results: Thirty-five patients were recruited, 24 were included for the analysis: 11 cases and 12 controls, with and average age of 54.9 and 50.,8 years respectively, homogeneous sex distribution. There was no statistically significant difference in the relative expression of the target genes, upon in vitro exposure to corticosteroids, between both groups. Conclusion: In our study, model and evaluation system, no effects of corticosteroids were evidenced. With a larger number of patients, other target genes or other study protocols, we cannot rule out that differences could be detected.

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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.

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OBJECTIVES: To compare the hearing outcomes of ossicular chain reconstruction using bone cement versus other materials such as prostheses and autografts. METHODS: This study included articles that compared hearing outcomes in patients with conductive hearing loss who underwent stapes revision surgery or chronic otitis media surgery. A systematic search for articles from January 2000 to February 2022 was conducted in Medline, Embase, and Cochrane Library databases. Only articles in English were included. An effective postoperative air-bone gap (ABG) was defined as ≤20 dB. A bias assessment tool was developed according to Cochrane guidelines, and the chi-square test was used to evaluate the mean age of the samples. RESULTS: Of the 418 studies that met the selection criteria, only seven were eligible for this study, consisting of 187 patients in the bone cement group and 173 in the non-bone cement group. Ossiculoplasty using bone cement yielded significantly better results, with a combined odds ratio (OR) of 2.03 (95% CI: 1.16-3.55, p = 0.01). CONCLUSIONS: The results of this study suggest that the effectiveness of bone cement in ossiculoplasty was greater than that of other materials in chronic otitis media surgery or stapes revision surgery, with a higher number of patients achieving ABG ≤20 dB.

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INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

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INTRODUCTION: The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5-15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located. OBJECTIVE: To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations. METHOD: Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023. RESULTS: Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation. DISCUSSION: The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.

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INTRODUCTION: The contribution of genetic causes to sensorineural hearing loss (SNHL) in adults is less clear than in children, and genetic diagnosis is still not standardized in adults. In this study we present the genetic results obtained in a cohort of adult patients with SNHL. MATERIALS AND METHODS: We included 63 adults with SNHL that received genetic testing between 2019 and 2022. Whole exome sequencing was performed and variants in genes related to hearing loss (virtual panel with 244 genes) were prioritised and analysed. RESULTS: 24% (15/63) of patients were genetically diagnosed: 87% (13/15) of patients had non-syndromic hearing loss and 13% (2/15) had syndromic hearing loss. We identified pathogenic and likely pathogenic variants in 11 different genes. CONCLUSIONS: Our results show that a significant proportion of adults with SNHL have a genetic origin, and that implementation of genetic testing improves diagnostic accuracy and allows personalized management of these patients.

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

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OBJECTIVE: to assess clinical safety and postoperative audiological outcomes in postlingual deafness Spanish speaking patients, who underwent surgery with Nurotron™ cochlear implant. MATERIAL AND METHODS: Retrospective descriptive case series study. We performed follow-up of complications and audiological measurements before and after cochlear implantation. Patients with bilateral severe to profound sensorineural hearing loss or patients with unilateral deafness with/without tinnitus were included. Repeated-measures within-subjects for assess pure tone thresholds and speech performance (bilingual test) with a detailed monitoring to establish security or adverse effects were performed. Analysis of variance tests, repetitive measures, were used for statistical analysis. RESULTS: 31 patients were included, 17 (54.8%) men and 14 (45.2%) women. Mean age at the time of surgery was 49.82 ± 18.8 years. The mean follow-up of the group was 31.56 ± 9.57 months (minimum = 19.6 months and maximum = 52.50 months). As major complication one patient (3.23%) had a hard failure that required removal and re-implantation. 25.8% of the patients presented minor complications, the most frequent being vertigo/unsteadiness in 22.6%. The mean of language discrimination (free field at 65 dB SPL) was 62.19% ± 16.66; being 69.82% ± 7.35 in the group of severe to profound bilateral sensorineural hearing loss. A statistically significant reduction was observed in patients with tinnitus, assessed using the visual analogue scale, preoperative = 7.2 ± 1,6 vs postoperative (18months postoperative) = 1.7 ± 1.3 (p < .001). CONCLUSIONS: The Nurotron™ cochlear implant shows satisfactory audiological results, in accordance with what has been reported in the literature. Minor complications were similar to previous studies, but the percentage of hard failure should continue to be observed, which was higher than other reports with comparable follow-up.

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El desarrollo del habla, el lenguaje, la comunicación, la cognición y otros aspectos en la infancia se ven profundamente influenciados por la audición. Cuando un niño presenta pérdida auditiva no detectada o no tratada, se reducen los estímulos y se dificulta el desarrollo de habilidades lingüísticas. La falta de atención temprana puede llevar a retrasos en el desarrollo del lenguaje, afectando la capacidad del niño para comprender y comunicarse. La hipoacusia pediátrica es un problema de salud pública que afecta a un porcentaje significativo de niños en todo el mundo. El aumento de la causa de la hipoacusia infantil en diferentes países se atribuye a factores como la falta de conciencia y educación sobre la importancia de la detección temprana, la disponibilidad y acceso limitados a servicios de detección y diagnóstico, la ausencia de programas de detección temprana en algunos países y la necesidad de contar con profesionales de la salud capacitados en el manejo de la audición pediátrica. Todo ello puede afectar áreas fundamentales del desarrollo, incluyendo el lenguaje y la comunicación, el desarrollo cognitivo, sensorial, motor y adaptativo. Por tanto, este trabajo tuvo el objetivo de llevar a cabo una revisión narrativa de la literatura científica sobre la situación de las alteraciones auditivas en la población pediátrica

The development of speech, language, communication, cognition, and other aspects in childhood is profoundly influenced by hearing. When a child experiences undetected or untreated hearing loss, stimuli are reduced, and the development of linguistic skills becomes challenging. The lack of early attention can lead to language development delays, affecting the child's ability to comprehend and communicate. Pediatric hearing loss is a public health issue that affects a significant percentage of children worldwide. The increasing prevalence of pediatric hearing loss in different countries is attributed to factors such as a lack of awareness and education about the importance of early detection, limited availability and access to screening and diagnostic services, the absence of early detection programs in some countries, and the need for trained healthcare professionals in pediatric audiology. All of this can impact fundamental areas of development, including language and communication, cognitive, sensory, motor, and adaptive development.Therefore, this work aimed to conduct a narrative review of the scientific literature on the status of auditory impairments in the pediatric population

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Objective: To explore the social and clinical factors that predict audiometric outcomes in patients undergoing ossicular chain reconstruction. Methods: A retrospective analytical cohort study was conducted, including patients 18 years of age or older with a history of chronic otitis media (COM) and/or any of its complications, who underwent ossicular chain reconstruction with Partial Ossicular Replacement Prosthesis (PORP) or Total Ossicular Replacement Prosthesis (TORP), at Hospital San José and Hospital infantile Universitario de San José between 2012 and 2020. We excluded patients with ossicular chain malformations and those with incomplete information. Information about sociodemographic and clinical factors was collected. Additionally, the surgery findings information was analyzed using the Ossiculoplasty outcome parameter staging ( OOPS) index. Results: A total of 35 adult patients who underwent ossicular chain reconstruction were retrospectively studied. An improvement was evidenced in the Preoperative Pure-Tone Average (PTA) and postoperative PTA (p-value=0.036), as well as in the pre and postoperative air-bone gap (ABG) (p-value < 0.01). A moderate correlation coefficient was found between the OOPS index and the postoperative PTA (p= 0.429), and between the OOPS index and the postoperative (ABG) (p= 0.653). Conclusion: We found that a higher OOPS score is correlated with worse hearing outcomes postoperatively, and there was no association between the demographic or pathologic factors with a worse postoperative hearing outcome. Therefore, OOPS index can predict audiometric outcomes in patients undergoing ossicular chain reconstruction in a developing country, regardless of the demographic or pathologic factors.

Objetivo: Evaluar los factores sociales y clínicos que predicen los desenlaces audiométricos en pacientes llevados a reconstrucción de cadena osicular en un país envía de desarrollo. Métodos: Se realizo un estudio de cohorte analítico retrospectivo donde se incluyeron pacientes mayores de 18 años, con antecedente de otitis media crónica y/o alguna complicación/secuela de esta, que fueron llevados a reconstrucción de la cadena osicular con prótesis PORP - TORP de la Fundación Universitaria de Ciencias de la Salud entre el año 2012 y 2020, se excluyeron pacientes con malformaciones de la cadena osicular y aquellos con informacion incompleta de su historia clinica y quirurgica. Resultados: La población estudiada fue 35 pacientes, en los cuales se compararon variables demográficas, antecedentes de rinitis o tabaquismo activo, parámetros audiológicos pre y postoperatorios, y hallazgos intraquirurgicos. Se evidenció una diferencia estadísticamente significativa entre el promedio tonal auditivo (PTA) preoperatorio y el PTA postoperatorio (p-valor=0.036), así como en el gap aéreo- oseo pre y post operatorio ( p-valor < 0.01). Se reportó un coeficiente de correlación moderado entre el índice OOPS y el PTA post operatorio (p = 0.429), y entre el índice OOPS y el gap aéreo óseo post operatorio (p = 0.653), lo que indica que a mayor puntaje en el índice OOPS peores desenlaces auditivos. Conclusión: En este estudio un mayor puntaje en el índice OOPS se correlacionó con peores desenlaces auditivos. No se evidenció correlación entre los factores demográficos u otras comorbilidades descritas y un peor desenlace auditivo post operatorio. Aunque se obtuvo un GAO postoperatorio ≤20dB en el 48.5% de los pacientes, se observó una disminución en el GAO estadísticamente significativo.

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Introducción y objetivo: El síndrome VEXAS (SV) es una entidad de descripción reciente que afecta fundamentalmente a varones, y se debe a una mutación somática en el gen UBA1. Puede cursar con múltiples manifestaciones sistémicas, siendo la afectación de cabeza y cuello muy frecuente. El objetivo de este estudio fue describir las manifestaciones otorrinolaringológicas del SV, que contribuyan a un diagnóstico y tratamiento temprano de la enfermedad. Método: Se realizó una revisión de la literatura médica, utilizando los criterios PRISMA adaptados al tipo de estudio, de las manifestaciones otorrinolaringológicas del SV, utilizando la base de datos PubMed. Resultados: Fueron incluidos en nuestro trabajo 81 artículos que cumplían los criterios de inclusión del mismo, los cuales describían 133 casos. Los resultados mostraron que el SV se produce, sobre todo, en varones mayores de 50 años de edad, presentando en más de la mitad de los casos, manifestaciones de cabeza y cuello, entre las que destacan la CA, la condritis nasal y el edema periorbitario. Conclusiones: Con frecuencia el SV es confundido, en los pacientes con manifestaciones otorrinolaringológicas, con la policondritis recurrente. El conocimiento por parte del otorrinolaringólogo de las manifestaciones de cabeza y cuello asociadas al mismo, puede contribuir a un diagnóstico y tratamiento temprano mejorando el pronóstico de la enfermedad. (AU)

Introduction and objectives: VEXAS syndrome (VS) is a recently described clinical entity that mainly affects males, and is due to a somatic mutation in the UBA1 gene. It can present with multiple systemic manifestations, especially hematological and dermatological, being the affectation of the head and neck very frequent, especially, auricular chondritis (AC). The objective of this study was to describe the otorhinolaryngological manifestations of VS, knowledge of which by the otolaryngologist could contribute to early diagnosis and treatment of the disease. Methods: A review of the medical literature was carried out, using the PRISMA criteria adapted to the type of study, of the otorhinolaryngological manifestations of VS, from its description in the year 2020 to December 2022, using the Pubmed database. Results: 81 articles that met the inclusion criteria were included in our work, which described 133 cases in detail. The results showed that VS occurs mainly in men over 50 years of age, presenting in more than half of the cases head and neck manifestations, among which AC, nasal chondritis and periorbital edema stand out. Conclusions: Since VS can affect any part of the body, mimicking the clinical pictures of other diseases, it has often been confused, in patients with otorhinolaryngological manifestations, especially with recurrent polychondritis. Knowledge by the otolaryngologist of the head and neck manifestations associated with it can contribute to early diagnosis and treatment, improving the prognosis of the disease. (AU)

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Introducción En recién nacidos, la hipoacusia secundaria a una infección congénita por citomegalovirus (CMVc), pese a su baja prevalencia, puede generar un grave problema en el desarrollo personal y la integración social de los pacientes. Por ello, es importante incluir la determinación del ADN de CMV como herramienta del cribado neonatal. Materiales y métodos Hemos realizado un estudio retrospectivo de 5 años, mediante la descripción de las CMVc en la Comunidad Autónoma del País Vasco en los recién nacidos que no superaron el cribado auditivo en el programa de detección precoz de hipoacusia. Se describen los tiempos de detección, confirmación (incidencia) e intervención (tratamiento). Resultados De 18.782 sujetos estudiados, 58 (3 por cada 1.000 nacidos vivos) presentaron hipoacusia. De ellos, se confirmó CMVc en 4pacientes (una mujer y 3hombres).El tiempo promedio para el cribado auditivo fue de 6,5 días (DE:±3,69) y para detectar el CMV mediante reacción en cadena de la polimerasa en orina y saliva fue de 4,2 días (DE:±3,94).El tiempo para confirmar la hipoacusia mediante PEATC e intervención audiológica fue de 2,2 meses (DE:±0,957) y 5 meses (DE:±3,741), respectivamente. Se realizaron 4adaptaciones audioprotésicas y un implante coclear. Discusión y conclusión El cribado auditivo neonatal se ha consolidado como un buen programa de salud pública. La determinación del ADN viral permite un diagnóstico y tratamiento precoz, específico e interdisciplinar, en el que la otorrinolaringología tiene un papel fundamental. Nuestro estudio resalta la importancia de incluir la reacción en cadena de la polimerasa del CMV como herramienta de cribado universal. (AU)

Introduction In newborns, hearing loss secondary to congenital cytomegalovirus (CMVc) infection, despite its low prevalence, can cause a serious problem in the personal development and social integration of patients. Therefore, it is important to include the determination of CMV DNA as a neonatal screening tool. Materials and methods We have carried out a 5-year retrospective study, by describing the CMVc in the Autonomous Community of the Basque Country (Spain) in newborns who did not pass the hearing screening in the early hearing loss detection program. The times of detection, confirmation (incidence) and intervention (treatment) are described. Results Of 18,782 subjects studied, 58 (3 per 1,000 live births) presented hearing loss. Of these, CMVc is guaranteed in 4patients (one woman and 3men). The mean time to hearing screening was 6.5 days (SD: ±3.69) and to detect CMV by polymerase chain reaction in urine and saliva was 4.2 days (SD: ±3.94). Time to confirm hearing loss by BAEP and audiological intervention 2.2 (SD: ±0.957) and 5 months (SD: ±3.741), respectively. Four hearing aid adaptations and one cochlear implant were performed. Discussion and conclusion Neonatal hearing screening has established itself as a good public health program. The determination of viral DNA allows an early, specific and interdisciplinary diagnosis and treatment, in which otorhinolaryngology plays a fundamental role. Our study highlights the importance of including CMV polymerase chain reaction as a universal screening tool. (AU)

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