Diagnóstico genético de la hipoacusia neurosensorial infantil / Genetic diagnosis of childhood sensorineural hearing loss

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)

Guía de práctica clínica sobre el manejo del schwannoma vestibular / Clinical practice guideline on the management of vestibular schwannoma

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)

El papel de la RMN en el diagnóstico de la patología audiovestibular / The role of MRI in the diagnosis of audio-vestibular pathology

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)

Clinical practice guideline on the management of vestibular schwannoma.

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.

Diagnostic yield of genetic testing in adults with sensorineural hearing loss.

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.

Enlarged vestibular aqueduct as a cause of postneonatal deafness.

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.

Bone cement in ossicular chain reconstruction: systematic review and meta-analysis.

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.

Genetic diagnosis of childhood sensorineural hearing loss.

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.

Clinical experience with the Nurotron™ cochlear implant in a Spanish-speaking postlingual population: Clinical safety and audiological results.

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 ±â€¯18.8 years. The mean follow-up of the group was 31.56 ±â€¯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% ±â€¯16.66; being 69.82% ±â€¯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 ±â€¯1,6 vs postoperative (18months postoperative) = 1.7 ±â€¯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.

Predictors of hearing outcomes in patients undergoing ossicular chain reconstruction in a developing country / Factores predictores de desenlaces auditivos en pacientes llevados a reconstrucción de la cadena osicular en un país en vía de desarrollo

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.

Manifestaciones otorrinolaringológicas del Síndrome VEXAS. Revisión sistemática / Ear, nose, and throat manifestations of VEXAS Syndrome. Systematic review

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)

Importancia del citomegalovirus congénito en el programa de cribado auditivo neonatal / Importance of congenital cytomegalovirus in the neonatal hearing screening program

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)

Subclinical neural hearing alterations in school children with type 1 diabetes mellitus / Alteraciones neuroauditivas subclínicas en escolares con diabetes mellitus tipo 1

Background and objectives Evidence has shown a cause-and-effect relationship between type 1 diabetes mellitus and auditory and cognitive dysfunctions. This study aimed to investigate the effect of type 1 diabetes mellitus (T1DM) on central auditory and cognitive functions in school-age children and adolescents. Methods The study sample consisted of 101 children and adolescents, 50 with T1DM, of both sexes, aged between 7 and 18 years. All participants were selected for a structured interview on hearing, behavioral, and cognitive health and assessment of brainstem auditory evoked potentials (BAEP) and event-related potentials (P300). Results Significant differences were observed in memory (p=0.002) and attention (p=0.021) complaints between participants with and without T1DM. In the BAEP responses, there were differences between wave III latencies in the right (p=0.017) and left (p=0.019) ears and in wave V latencies in the left ear (p=0.001) between the evaluated groups. In addition, there was an association between BAEP findings and metabolic control in the T1DM group in the left ear in waves III (p=0.006) and V (p=0.005) and in the right ear in wave V (p=0.026). No differences were observed in the latencies of P300 between the evaluated groups. Conclusion This study demonstrated the existence of a subclinical finding in the central auditory pathway, offering an increased risk for retrocollear alterations, which may be a consequence of poor metabolic control. (AU)

Justificación y objetivos La evidencia ha demostrado una relación de causa y efecto entre la diabetes mellitus tipo 1 y las disfunciones auditivas y cognitivas. Este estudio tuvo como objetivo investigar el efecto de la diabetes mellitus tipo 1 (DM1) en las funciones cognitivas y auditivas centrales en niños y adolescentes en edad escolar. Métodos La muestra del estudio estuvo constituida por 101 niños y adolescentes, 50 con DM1, de ambos sexos, con edades entre 7 y 18 años. Todos los participantes fueron seleccionados para una entrevista estructurada sobre la salud auditiva, conductual y cognitiva y la evaluación de los potenciales evocados auditivos del tronco encefálico (BAEP) y los potenciales relacionados con eventos (P300). Resultados Se observaron diferencias significativas en quejas de memoria (p=0,002) y atención (p=0,021) entre participantes con y sin DM1. En las respuestas BAEP, hubo diferencias entre las latencias de la onda III en el oído derecho (p=0,017) e izquierdo (p=0,019) y en las latencias de la onda V en el oído izquierdo (p=0,001) entre los grupos evaluados. Además, hubo asociación entre los hallazgos de PEATC y el control metabólico en el grupo de DM1 en el oído izquierdo en las ondas III (p=0,006) y V (p=0,005) y en el oído derecho en la onda V (p=0,026). No se observaron diferencias en las latencias de P300 entre los grupos evaluados. Conclusión Este estudio demostró la existencia de un hallazgo subclínico en la vía auditiva central, ofreciendo un mayor riesgo de alteraciones retrocollar, lo que puede ser consecuencia de un mal control metabólico. (AU)

Hearing results with combination steroid therapy for sudden sensorineural hearing loss.

BACKGROUND: Sudden Sensorineural Hearing Loss was described by Mc. Cabe in 1979 and, since then, many authors have tried to define, explain and correctly treat this disease. The National Institute on Deafness and Other Communication Disorders defines it as sudden sensorineural hearing loss of at least 30 dB in three contiguous audiometric frequencies in a period of 72 hours. Among the therapeutic strategies, corticosteroids have been shown to have the greatest benefit due to their anti-inflammatory and anti-cellular stress effects. OBJECTIVE: To determine the hearing results with combined steroid therapy in patients with sudden sensorineural hearing loss (SSHL), according to the Siegel recovery criteria scale. METHOD: Study carried out in the otorhinolaryngology and head and neck surgery service of the Centro Médico Naval, Ciudad de México, where 150 patients diagnosed with SSHL and who received combined therapy with intratympanic dexamethasone and systemic prednisone were included. RESULTS: Therapeutic effectiveness was demonstrated by correlating therapeutic success in 82% of cases and therapeutic failure in 18% of cases, by correlating it with the Siegel recovery criteria scale. When evaluating the general average of the pure tone average levels at the beginning and 6 weeks after treatment, a statistically significant difference was obtained (p = 0.001). The average of the speech audiometry at the beginning and 6 weeks later had a statistically significant difference (p = 0.001). CONCLUSIONS: Initial combined steroid treatment for SSHL has been shown to have beneficial results according to Siegel recovery criteria scale.

ANTECENDENTES: La Hipoacusia Neurosensorial Súbita Idiopática fue descrita por Mc. Cabe en 1979 y, desde entonces, muchos autores han tratado de definir, explicar y tratar correctamente esta enfermedad. El Nacional Institute on Deafness and Other Communication Disorders la define como pérdida auditiva neurosensorial brusca de al menos 30 dB en tres frecuencias audiométricas contiguas en un periodo de 72 horas. Entre las estrategias terapéuticas, los corticosteroides han demostrado tener mayor beneficio por sus efectos antiinflamatorios y antiestrés celular. OBJETIVO: Determinar los resultados auditivos con la terapia de esteroides combinados en pacientes con hipoacusia neurosensorial súbita idiopática (HNSI), de acuerdo a la escala de criterios de recuperación de Siegel. MÉTODO: Estudio realizado en el servicio de otorrinolaringología y cirugía de cabeza y cuello del Centro Médico Naval, en Ciudad de México, en el que se incluyeron 150 pacientes con diagnóstico de HNSI y que recibieron terapia combinada con dexametasona intratimpánica y prednisona sistémica. RESULTADOS: Se demostró una efectividad terapéutica al correlacionar el éxito terapéutico en el 82% de los casos y un fracaso terapéutico en el 18% de los casos según la escala de criterios de recuperación de Siegel. Al evaluar el promedio general de los niveles de promedio de tonos puros al inicio y 6 semanas posterior al tratamiento se obtuvo una diferencia estadísticamente significativa (p = 0.001). El promedio de las logoaudiometrías al inicio y 6 semanas posterior al tratamiento tuvo una diferencia estadísticamente significativa (p = 0.001). CONCLUSIONES: El tratamiento combinado con esteroides de manera inicial para la HNSI ha demostrado tener resultados benéficos de acuerdo con la escala de criterios de recuperación de Siegel.

Factores de riesgo relacionados con hipoacusia en recién nacidos mediante tamizaje neonatal auditivo en el departamento del Atlántico (Colombia), 2019-2020

Objetivos: Identificar los factores de riesgo relacionados con hipoacusia en recién nacido mediante tamizaje neonatal auditivo en el departamento del Atlántico (Colombia) en el periodo 2019-2020. Materiales y métodos: Estudio observacional, analítico, de casos y controles anidado en una cohorte. Los recién nacidos fueron atendidos en 4 instituciones de III nivel de complejidad en Atlántico entre 2019 y 2020. Se incluyeron 32 casos correspondientes a registros según criterios de la Organización Mundial de la Salud (OMS), sugestivos de alteración auditiva mediante la prueba de otoemisiones acústicas y 28 controles seleccionados de la misma población fuente, donde se originaron los casos en el mismo período. Se aplicaron los criterios de exclusión. Las variables cualitativas se presentaron en tablas de frecuencia y el análisis estadístico se realizó en el programa STATGRAPHICS versión 16. Para establecer diferencia entre los 2 grupos se utilizó la prueba (X2), calculándose su respectivo odds ratio, con un intervalo del 95 % de confianza. Resultados: Los principales factores de riesgo encontrados en recién nacido con alteración auditiva sugestiva de hipoacusia con significancia estadística (p<0,05) fueron la estancia en la Unidad de Cuidados Neonatales: OR 4,2 IC95 % (1,4-12,4) y uso de aminoglucósidos: OR 3,2 IC95 % (1,03-10,1). Conclusiones: Se encontró asociación entre la estancia en la Unidad de Cuidados Neonatales y uso de aminoglucósidos con alteración auditiva sugestivo de hipoacusia. Se debe tener en cuenta estos factores de riesgo para ser identificados en forma oportuna y realizar el manejo adecuado, como por ejemplo, disminuyendo tiempo de exposición.

Objectives: Identify risk factors related to hearing loss in newborns through neonatal hearing screening in the Department of Atlantic in the period 2019-2020. Materials and methods: Observational, analytical, case-control study nested in a cohort. Newborns were cared for in 4 level III of complexity health institutions in Atlantic between 2019 and 2020. 32 cases were included corresponding to records according to World Health Organization (WHO) criteria, suggestive of hearing impairment through the otoacoustic emissions test and 28 controls selected from the same source population, where the cases originated in the same period. Exclusion criteria were applied. The qualitative variables were presented in frequency tables and the statistical analysis was carried out in the STAT-GRAPHICS version 16 program. To establish the difference between the 2 groups, the (X2) test was used, calculating their respective odds ratio with a CI 95 %. Results: The main risk factors found in newborns with hearing impairment suggestive of hearing loss with statistical significance (p<0,05) were the stay in the Neonatal Care Unit: OR 4,2 CI95% (1,4-12,4) and use of aminoglycosides: OR 3,2 CI95 % (1,03-10,1). Conclusions: An association was found between the stay in the neonatal care unit and the use of aminoglycosides with hearing impairment suggestive of hearing loss. These risk factors must be taken into account to be identified in a timely manner and to carry out adequate management, such as reducing exposure time.

Evaluation of von Willebrand factor and protein/creatinine ratio in idiopathic sudden hearing loss / Evaluación del Factor Von Willebraund y la Relación Proteína/Creatinina en la Hipoacusia Súbita Idiopática

Objective The objective of this study was to evaluate the levels of von Willebraund Factor (vWF) in plasma and the protein/creatinine ratio in urine in patients with idiopathic sudden acute hearing loss, which we think to be caused by epithelial dysfunction. Materials-Methods Thirty patients with a sudden hearing loss and thirty healthy individuals were included in the study. Before the treatment, blood and urine were collected from the patients and the control group to investigate the levels of the protein/creatinine ratio and the levels of vWF. The test results of the patients group were compared with those of the control group. Results We found that the levels of vWF increased in the patient group, which was statistically significant (P<.05). The protein/creatinine ratio in the urine increased in the patient group, but this was not statistically significant (P>.05). In addition, we found that the vWF and urine protein/creatin ratio of the patients who benefited from treatment were lower than those who did not benefit. Conclusions This study showed that sudden sensorineural hearing loss may result from endothelial dysfunction. However, more studies that include more patients are needed in order to support this. (AU)

Objetivo El objetivo de este estudio fue evaluar los niveles de factor de von Willebraund (vWF) en plasma y la relación proteína/creatinina en orina en pacientes con hipoacusia aguda súbita idiopática, que creemos que es causada por una disfunción epitelial. Material-Método Treinta pacientes con hipoacusia súbita y treinta individuos sanos fueron incluidos en el estudio. Antes del tratamiento, se recogieron sangre y orina de los pacientes y del grupo de control para investigar los niveles de relación proteína/creatinina y los niveles de vWF. Los resultados de las pruebas del grupo de pacientes se compararon con el grupo de control. Resultados En la literatura, se sabe que la cantidad de proteína en la orina y las elevaciones en los niveles de vWF en plasma son las consecuencias de la disfunción endotelial. Encontramos que los niveles de vWF aumentaron en el grupo de pacientes y esto fue estadísticamente significativo. Sin embargo, la relación proteína/creatinina en la orina aumentó en el grupo de pacientes, pero esto no fue estadísticamente significativo. Además, encontramos que el vWF y la relación proteína/creatina en orina de los pacientes que se beneficiaron del tratamiento fueron más bajos que los que no se beneficiaron. Conclusiones Este estudio muestra que la pérdida auditiva neurosensorial súbita puede resultar de una disfunción endotelial. Sin embargo, se necesitan más estudios que incluyan más pacientes para respaldar esto. (AU)

Cribado auditivo neonatal universal e hipoacusia diferida o de desarrollo tardío / Universal neonatal hearing screening and delayed hearing loss or late-developmental hearing loss

Objetivo Determinar el porcentaje de niños que presentan una hipoacusia bilateral permanente posnatal para estudiar su incidencia, los factores de riesgo relacionados, su diagnóstico y su tratamiento.MétodosEstudio retrospectivo de recogida de datos de niños diagnosticados de hipoacusia fuera del periodo neonatal en la Unidad de Hipoacusia del Hospital Universitario Central de Asturias, desde abril de 2014 hasta abril de 2021.ResultadosUn total de 52 casos cumplieron los criterios de inclusión. La tasa de detección de hipoacusias congénitas del programa de cribado neonatal en el mismo periodo de estudio fue de 1,5 niños por cada 1000 recién nacidos al año; sumando las hipoacusias posnatales da como resultado una tasa de hipoacusia bilateral infantil de 2,7 niños por 1000 (55,5% y 44,4%, respectivamente). Presentan factores de riesgo de hipoacusia 35, siendo 23 de riesgo retrococlear. La edad media de la derivación fue de 91,9 meses (18-185). La adaptación audioprotésica se indicó en 44 casos (84,6%). En 8 casos (15,4%) se indicó la implantación coclear.DiscusiónAunque la hipoacusia congénita representa la mayoría de las sorderas en la infancia, la hipoacusia posnatal tiene una incidencia importante. Esta puede responder principalmente a: 1) que el deterioro auditivo puede surgir en los primeros años de vida, 2) que la hipoacusia leve, así como las pérdidas auditivas en frecuencias graves son indetectables por el cribado neonatal en algunos casos, 3) que algunos niños pueden presentar falsos negativos en los resultados.ConclusiónLa hipoacusia posnatal requiere la identificación de factores de riesgo y el seguimiento a largo plazo de los niños que la sufren, ya que es preciso que sea detectada y tratada precozmente. (AU)

Objective To determine the percentage of children with permanent bilateral postnatal hearing loss in order to study its incidence, related risk factors, diagnosis and treatment.MethodsRetrospective study to collect data on children diagnosed with hearing loss outside the neonatal period in the Hearing Loss Unit of the Hospital Universitario Central de Asturias, from April 2014 to April 2021.Results52 cases met the inclusion criteria. The detection rate of congenital hearing loss in the neonatal screening programme in the same study period was 1.5 children per thousand newborns per year, adding postnatal hearing loss results in a rate of infant bilateral hearing loss of 2.7 children per thousand (55.5% and 44.4% respectively). Thirty-five children presented risk factors for hearing loss, of which 23 were at retrocochlear risk. The mean age at referral was 91.9 (18-185) months. Hearing aid fitting was indicated in 44 cases (84.6%). Cochlear implantation was indicated in eight cases (15.4%).DiscussionAlthough congenital hearing loss accounts for the majority of childhood deafness, postnatal hearing loss has a significant incidence. This may be mainly due to: 1) that hearing impairment may arise in the first years of life, 2) that mild hearing loss as well as hearing loss in severe frequencies are undetectable by neonatal screening in some cases, 3) that some children may have false negative results.ConclusionPostnatal hearing loss requires identification of risk factors and long-term follow-up of children with hearing loss, as it needs to be detected and treated early. (AU)

Manifestaciones otológicas de la enfermedad relacionada con IgG4 / Otologic manifestations of IgG4-related disease

La enfermedad relacionada con IgG4 (ER-IgG4) constituye un proceso fibroinflamatorio autoinmune sistémico caracterizado por la infiltración multiorgánica de células plasmáticas positivas para IgG4, fibrosis y vasculopatía que determinan la disfunción del órgano afectado. La presente revisión tiene por objetivo caracterizar las manifestaciones otológicas de la enfermedad.Se realizó una revisión sistemática de la literatura biomédica publicada describiendo los casos de ER-IgG4 con manifestaciones otológicas. Para la búsqueda utilizamos las siguientes bases de datos: Pubmed, Web of Science y Scopus. Seleccionamos 48 casos de los que se extrajeron diferentes variables para caracterizar las manifestaciones otológicas de la misma.Alrededor del 52% de los pacientes eran varones con edades comprendidas entre los 19 y los 79 años. La forma más habitual de presentación fue lesiones seudotumorales con presentación clínica similar a una otitis seromucosa, con lesión coclear asociada o una hipoacusia neurosensorial (uni o bilateral). Presentaciones menos frecuentes fueron un síndrome vertiginoso, condritis auricular, otitis eosinofílica o paquimeningitis hipertrófica con afectación cocleovestibular. En 32 pacientes (67%) la manifestación otológica constituyó la forma de presentación de la ER-IgG4.Dieciséis pacientes (33%) fueron tratados mediante cirugías agresivas como consecuencia del retraso diagnóstico y el desconocimiento de esta entidad clínica. Dos pacientes precisaron un implante coclear.Si bien las manifestaciones óticas de la ER-IgG4 son infrecuentes, es importante que el otorrinolaringólogo conozca las formas de presentación otológicas de esta entidad, ya que pueden ser la primera manifestación de la enfermedad, permitiendo un diagnóstico precoz y un tratamiento adecuado, evitando secuelas permanentes posteriores. (AU)

IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease characterized by multiorgan infiltration of IgG4-positive plasma cells, fibrosis and vasculopathy that determine dysfunction of the affected organ. This review aims to characterize the otologic manifestations of the disease.We have conducted a systematic review of the biomedical literature published describing cases of IgG4-RD with otologic manifestations. For the review, the bibliographic databases utilized were Pubmed, Web of Science and Scopus. We selected 48 cases from which we extracted several data collections.About 52% of the patients were male between the ages of 19 and 79 years. Otologic findings were characterized by pseudotumoral lesions that most often caused a clinical presentation similar to otitis media with effusion, with cochlear involvement or sensorineural hearing loss (uni or bilateral). Less frequent presentations included auricular chondritis, eosinophilic otitis, or hypertrophic pachymeningitis. In 32 patients (67%) the otologic manifestation was the first symptom of the ER-IgG4.Sixteen patients (33%) were treated with mastoidectomies as a result of delayed diagnosis and lack of knowledge about this entity. Two patients needed a cochlear implant.Although the otologic manifestations of IgG4-RD are unusual, it is important for the otorhinolaryngologist to know the otologic manifestations of this entity as it can be the onset of the disease, in order to allow early diagnosis and adequate treatment, avoiding permanent sequelae.

Comportamento Auditivo e Validação no Diagnóstico Audiológico e Intervenção em Bebês e Crianças com Deficiência Auditiva / Auditory Behavior and Validation in Audiological Diagnosis and Intervention in Infants and Children with Hearing Impairment / Comportamiento auditivo y validación en el diagnóstico e intervención audiológicos en bebés y niños con deficiencia auditiva

Introdução: A atuação profissional com bebês e crianças pequenas com deficiência auditiva exige conhecimento e técnica específica no que diz respeito à prescrição e adaptação do aparelho de amplificação sonora individual (AASI) e ao processo de desenvolvimento de linguagem. Limitações e imprecisões ao longo do processo diagnóstico poderão comprometer todos os procedimentos subsequentes do processo de intervenção. Objetivo: Analisar a validação do processo de diagnóstico audiológico e intervenção em bebês e crianças com deficiência auditiva a partir da análise comparativa de exames audiológicos, comportamento auditivo e aplicação do princípio de verificação cruzada após adaptação de AASI. Método: Foram sujeitos da pesquisa 12 crianças de até 36 meses de idade, com diagnóstico de perda auditiva neurossensorial bilateral, selecionados a partir da disponibilidade de acesso ao serviço para a avaliação e agrupados em G1 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB até 35%) e G2 (sujeitos com Índice de Inteligibilidade de Fala - SII 65 dB acima de 54%). Resultados: A média de idade do diagnóstico audiológico foi de 4,33 meses. Os resultados audiológicos de todas as crianças tiveram correspondência entre si, com exceção de dois sujeitos do G2. Conclusão: O comportamento auditivo não só permitiu a validação dos processos de diagnóstico e intervenção auditiva dos sujeitos da pesquisa, como também permitiu a identificação de comportamentos não compatíveis com a audibilidade devido ao uso inconsistente dos AASI. A aplicação dos instrumentos de acompanhamento de desenvolvimento mostrou-se adequada para o monitoramento do desenvolvimento de habilidades de audição e linguagem em crianças pequenas. (AU)

Introduction: Professional work with infants and young children with hearing impairment requires specific knowledge and technique regarding the prescription and adaptation of the individual sound amplification device (PSAD) and the language development process. Limitations and inaccuracies throughout the diagnostic process may compromise all subsequent procedures of the intervention process. Purpose: To analyze the validation of the process of audiological diagnosis and intervention in infants and children with hearing impairment based on the comparative analysis of audiological tests, auditory behavior and application of the cross-checking principle after adaptation of hearing aids. Method: The research subjects were 12 children aged up to 36 months, with a diagnosis of bilateral sensorineural hearing loss, selected from the availability of access to the service for the evaluation and grouped into G1 (subjects with Speech Intelligibility Index - SII 65 dB up to 35%) and G2 (subjects with Speech Intelligibility Index - SII 65 dB above 54%). Results: The average age of the audiological diagnosis was 4.33 months. The audiological results of all children corresponded to each other, except for two subjects from G2. Conclusion: The auditory behavior not only allowed the validation of the processes of diagnosis and auditory intervention of the research subjects, but also allowed the identification of behaviors that are not compatible with audibility due to the inconsistent use of hearing aids. The application of developmental monitoring instruments proved to be adequate for monitoring the development of hearing and language skills in young children. (AU)

Introducción: El trabajo profesional con lactantes y niños pequeños con discapacidad auditiva requiere conocimientos y técnica específicos respecto a la prescripción y adaptación del dispositivo individual de amplificación del sonido (PSAD) y el proceso de desarrollo del lenguaje. Las limitaciones e imprecisiones a lo largo del proceso de diagnóstico pueden comprometer todos los procedimientos posteriores del proceso de intervención. Propósito: Analizar la validación del proceso de diagnóstico e intervención audiológica en lactantes y niños con discapacidad auditiva a partir del análisis comparativo de pruebas audiológicas, conducta auditiva y aplicación del principio de cruce tras adaptación de audífonos. Método: Los sujetos de investigación fueron 12 niños de hasta 36 meses, con diagnóstico de hipoacusia neurosensorial bilateral, seleccionados de la disponibilidad de acceso al servicio para la evaluación y agrupados en G1 (sujetos con Índice de inteligibilidade del Habla - SII 65 dB hasta 35%) y G2 (sujetos com Índice de inteligibilidade del Habla - SII 65 dB por encima del 54%). Resultados:La edad promedio del diagnóstico audiológico fue de 4,33 meses. Los resultados audiológicos de todos los niños se correspondieron entre sí, a excepción de dos sujetos del G2. Conclusión: La conducta auditiva no sólo permitió validar los procesos de diagnóstico e intervención auditiva de los sujetos de investigación, sino que también permitió identificar conductas no compatibles con la audibilidad debido al uso inconsistente de audífonos. La aplicación de instrumentos de seguimiento del desarrollo demostró ser adecuada para controlar el desarrollo de las habilidades auditivas y lingüísticas en niños pequeños. (AU)

Subclinical neural hearing alterations in school children with type 1 diabetes mellitus.

BACKGROUND AND OBJECTIVES: Evidence has shown a cause-and-effect relationship between type 1 diabetes mellitus and auditory and cognitive dysfunctions. This study aimed to investigate the effect of type 1 diabetes mellitus (T1DM) on central auditory and cognitive functions in school-age children and adolescents. METHODS: The study sample consisted of 101 children and adolescents, 50 with T1DM, of both sexes, aged between 7 and 18 years. All participants were selected for a structured interview on hearing, behavioral, and cognitive health and assessment of brainstem auditory evoked potentials (BAEP) and event-related potentials (P300). RESULTS: Significant differences were observed in memory (p=0.002) and attention (p=0.021) complaints between participants with and without T1DM. In the BAEP responses, there were differences between wave III latencies in the right (p=0.017) and left (p=0.019) ears and in wave V latencies in the left ear (p=0.001) between the evaluated groups. In addition, there was an association between BAEP findings and metabolic control in the T1DM group in the left ear in waves III (p=0.006) and V (p=0.005) and in the right ear in wave V (p=0.026). No differences were observed in the latencies of P300 between the evaluated groups. CONCLUSION: This study demonstrated the existence of a subclinical finding in the central auditory pathway, offering an increased risk for retrocollear alterations, which may be a consequence of poor metabolic control.