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.

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)

Universal neonatal hearing screening and delayed hearing loss or late-developmental hearing loss.

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. METHODS: Retrospective 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. RESULTS: 52 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%). DISCUSSION: Although 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. CONCLUSION: postnatal 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.

Ototoxicidad en pacientes oncológicos: experiencia y propuesta de un protocolo de vigilancia / Ototoxicity in cancer survivors: experience and proposal of a surveillance protocol

Introducción: La ototoxicidad se presenta en diversos porcentajes según estudios tras el tratamiento con quimioterapia basada en platino y/o radioterapia craneal. El objetivo es mostrar nuestra experiencia en la monitorización de la ototoxicidad. Material y métodos: Se realizó una revisión del 1999 al 2019 en el registro de pacientes oncológicos pediátricos de nuestro hospital y remitidos a la Unidad de Hipoacusia Infantil. Resultados: 46 pacientes fueron remitidos a nuestra unidad. 41 pacientes recibieron platinos como parte de su tratamiento, 17 pacientes fueron sometidos a una intervención neuroquirúrgica y 18 pacientes recibieron radioterapia craneal. A todos se les realizó una anamnesis y otoscopia, y la monitorización se llevó a cabo con una audiometría tono-verbal y/o productos de distorsión. Se objetivó una hipoacusia como secuela del tratamiento en ocho pacientes (21,05% de los pacientes remitidos para seguimiento audiológico). Fue imposible determinar la situación audiológica al finalizar el tratamiento en ocho pacientes. La adaptación audioprotésica fue necesaria en dos pacientes. En la coordinación con Oncología Pediátrica, se consideró oportuno el cambio de cisplatino por carboplatino por ototoxicidad importante durante el tratamiento en un único paciente. Conclusión: Es imprescindible una adecuada coordinación con Oncología Pediátrica para realizar una vigilancia activa de la ototoxicidad y modificar, si es posible, la dosificación o el tipo de quimioterápico en caso de verse afectada la audición. En nuestra experiencia, y siguiendo las recomendaciones actuales, realizamos una valoración pretratamiento, una monitorización durante el tratamiento, al finalizarlo y después de forma anual por el riesgo de desarrollo diferido de una hipoacusia. (AU)

Introduction: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present experience in ototoxicity monitoring. Material and methods: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. Results: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. Conclusion: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss. (AU)

Aplicación de la tecnología de enrutamiento inalámbrico contralateral de la señal (CROS) en usuarios de implante coclear unilateral / Application of Wireless Contralateral Routing of Signal (CROS) Technology in Unilateral Cochlear Implant Users

INTRODUCCIÓN: La implantación unilateral en la hipoacusia profunda bilateral deja al paciente en una situación de audición monoaural, con los consiguientes problemas audiológicos, en especial por la incapacidad de percepción de la palabra en ambientes con ruido de fondo, por el efecto sombra de la cabeza. Se puede mejorar su rendimiento auditivo aprovechando los sistemas inalámbricos de enrutamiento de la señal (CROS), para dotarlos de una entrada de sonido bilateral. MÉTODO: Se estudiaron 12 usuarios unilaterales de implante coclear adultos, con experiencia con un procesador de sonido Naída CI Q70 que tiene capacidad de comunicación inalámbrica con el CROS. La comprensión del habla en cabina en silencio y en ruido se llevó a cabo utilizando como estímulos el test de oraciones grabadas en español en 3 condiciones: habla dirigida hacia el oído no implantado con y sin CROS activado y habla de frente con UltraZoom. Tras un mes de uso del dispositivo CROS, se evaluó la satisfacción del usuario utilizando cuestionarios. RESULTADOS: En silencio, la diferencia media entre el implante unilateral solo y el implante unilateral con el dispositivo CROS fue del 27,6%, mientras que en presencia de ruido fue del 32,5%. Si se comparan las puntuaciones entre presentar activada o no la opción UltraZoom al utilizar CROS, se demuestra un beneficio de un 35% de inteligibilidad. Todos los sujetos puntuaron su experiencia de uso como satisfactoria. CONCLUSIÓN: El dispositivo CROS utilizado con implante coclear unilateral produce un beneficio objetivo y subjetivo en el reconocimiento de la palabra cuando la señal se dirige hacia el mismo. En este estudio no se reprodujo la condición de presentación de la señal verbal en el oído implantado con ruido en el adaptado con el CROS por haberse demostrado previamente que la discriminación verbal se deteriora de forma importante con respecto al implante unilateral sin CROS

INTRODUCTION: Single cochlear implantation usually provides substantial speech intelligibility benefits but bilaterally deaf, unilaterally implanted subjects will continue to experience limitations due to the head shadow effect, like single-sided deaf individuals. In the treatment of individuals with single-sided deafness one option is contralateral routing of signal (CROS) devices, which constitute a non-surgical intervention of the second ear in unilaterally implanted individuals. METHOD: Twelve experienced adult cochlear implant users with Naída Q70 processor and the CROS device used in combination participated in the study. For the study 3 conditions were provided: cochlear implant only, omnidirectional microphone mode (CROS deactivated); cochlear implant plus CROS activated, omnidirectional microphone mode and cochlear implant plus CROS activated, UltraZoom mode. Speech reception thresholds were determined in quiet and noise. Subjective feedback regarding the practical usability of the CROS device and the perceived benefit were collected. RESULTS: There was a 27.6% improvement in speech understanding in quiet and 32.5% improvement in noise when CROS device was activated. Using advanced directional microphones, a statistically significant benefit of 35% was obtained. The responses to the questionnaires revealed that the subjects perceived benefit in their everyday lives when using the CROS device with their cochlear implants. CONCLUSION: The investigated CROS device used by unilateral CI recipients in cases where bilateral implantation is not an option provides both subjective and objective speech recognition benefit when the signal is directed to the CROS device. Unfavourable conditions where speech is presented from the cochlear implant side and noise from the CROS side or diffusely were not included in this evaluation since the CROS device adds additional noise and performance is expected to decrease as has previously been shown

[Ototoxicity in cancer survivors: experience and proposal of a surveillance protocol]. / Ototoxicidad en pacientes oncológicos: experiencia y propuesta de un protocolo de vigilancia.

INTRODUCTION: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present experience in ototoxicity monitoring. MATERIAL AND METHODS: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. RESULTS: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. CONCLUSION: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss.

Application of Wireless Contralateral Routing of Signal (CROS) Technology in Unilateral Cochlear Implant Users. / Aplicación de la tecnología de enrutamiento inalámbrico contralateral de la señal (CROS) en usuarios de implante coclear unilateral.

INTRODUCTION: Single cochlear implantation usually provides substantial speech intelligibility benefits but bilaterally deaf, unilaterally implanted subjects will continue to experience limitations due to the head shadow effect, like single-sided deaf individuals. In the treatment of individuals with single-sided deafness one option is contralateral routing of signal (CROS) devices, which constitute a non-surgical intervention of the second ear in unilaterally implanted individuals. METHOD: Twelve experienced adult cochlear implant users with Naída Q70 processor and the CROS device used in combination participated in the study. For the study 3 conditions were provided: cochlear implant only, omnidirectional microphone mode (CROS deactivated); cochlear implant plus CROS activated, omnidirectional microphone mode and cochlear implant plus CROS activated, UltraZoom mode. Speech reception thresholds were determined in quiet and noise. Subjective feedback regarding the practical usability of the CROS device and the perceived benefit were collected. RESULTS: There was a 27.6% improvement in speech understanding in quiet and 32.5% improvement in noise when CROS device was activated. Using advanced directional microphones, a statistically significant benefit of 35% was obtained. The responses to the questionnaires revealed that the subjects perceived benefit in their everyday lives when using the CROS device with their cochlear implants. CONCLUSION: The investigated CROS device used by unilateral CI recipients in cases where bilateral implantation is not an option provides both subjective and objective speech recognition benefit when the signal is directed to the CROS device. Unfavourable conditions where speech is presented from the cochlear implant side and noise from the CROS side or diffusely were not included in this evaluation since the CROS device adds additional noise and performance is expected to decrease as has previously been shown.

Evaluación de las coberturas de vacunación en pacientes con implante coclear de un hospital de referencia del norte de España / Evaluation of vaccination coverage in cochlear implant patients at a referral hospital in Northern Spain

Antecedentes y objetivo: El riesgo de meningitis bacteriana aumenta en los pacientes con implante coclear. Por ello, se indica la vacunación antineumocócica, antigripal y frente a Haemophilus influenzae tipo b en este grupo. El objetivo del presente estudio es conocer el cumplimiento del calendario vacunal en los pacientes implantados en un hospital de referencia. Materiales y métodos: Se incluyeron los pacientes con implante coclear intervenidos entre 2005 y 2015. Se evaluaron las coberturas vacunales frente a gripe estacional, Haemophilus influenzae tipo b, neumococo conjugada de 13 serotipos y neumococo polisacárida de 23 serotipos. Se dividió la muestra en 2 grupos por edad (< 14 años y ≥ 14 años). Se realizó un análisis univariante y bivariante. Resultados: De los 153 pacientes estudiados (28,01% 0-13 años y 71,9% ≥ 14), solo 2 (5,71%) tuvieron un 100% de adherencia al calendario vacunal, mientras que el 65,71% registró un cumplimiento del 50% o menor. Globalmente, la cobertura de vacunación frente a la pauta secuencial de neumococo fue del 48,57%. La población pediátrica superó el 90% de cobertura para la vacuna frente a Haemophilus influenzae tipo b y neumococo conjugada de 13 serotipos, mientras que en los mayores de 14 años apenas superó el 50%. La cobertura frente a gripe estacional fue inferior al 40%. Se obtuvo una correlación inversa entre la edad y el cumplimiento, aunque no estadísticamente significativa. Conclusiones. Las coberturas de vacunación en los pacientes con implante coclear evaluados son más bajas de lo esperado. Se propone la colaboración estrecha entre los servicios de Otorrinolaringología y las Unidades de Vacunas como principal estrategia para la mejora

Background and objective: The risk of bacterial meningitis increases in cochlear implant patients. Therefore, pneumococcal, influenza and Haemophilus influenzae type b vaccination is indicated in this group. The aim of this study was to determine compliance with the vaccination calendar in patients implanted in a referral hospital. Materials and methods: Patients with cochlear implant operated between 2005 and 2015 were included. Vaccine coverage for seasonal influenza, Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes and pneumococcal polysaccharide 23-serotypes was evaluated. The sample was divided into 2 age groups (< 14 years and ≥ 14 years). A univariate and bivariate analysis was performed. Results: Of the 153 patients studied (28.01% 0-13 years old and 71.9% ≥ 14), only 2 (5.71%) had 100% adherence to the vaccination schedule, while 65.71% had compliance of 50% or less. Overall, vaccination coverage against the sequential pneumococcal pattern was 48.57%. The paediatric population exceeded 90% coverage for the vaccine against Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes while in those over 14 years of age it barely exceeded 50%. Influenza coverage was less than 40%. An inverse correlation was obtained between age and compliance, although not statistically significant. Conclusions: Vaccination coverage in patients with cochlear implant is lower than expected. Close collaboration between Otolaryngology departments and the Vaccination Units is proposed as the main strategy for improvement

Comparación de la producción vocal en niños con hipoacusia prelocutiva con adaptación audioprotésica e implante coclear con normooyentes / Vowel production in hearing impaired children: A comparison between normal-hearing, hearing-aided and cochlear-implanted children

Introducción y objetivos: La inadecuada retroalimentación auditiva en los niños con hipoacusia prelocutiva altera la articulación de consonantes y vocales. El propósito de esta investigación es comparar la producción de vocales en niños hipoacúsicos hispanohablantes con implante coclear o adaptación audioprotésica con niños normooyentes por medio del análisis acústico de las frecuencias formánticas y el espacio vocal. Método: Un total de 56 niños con hipoacusia prelocutiva (25 con implante coclear y 31 con audífonos) y 47 niños normooyentes participaron en el estudio. Los 2 primeros formantes (F1 y F2) de las 5 vocales del idioma español se midieron utilizando el programa Praat. Para analizar las diferencias entre los 3 grupos se aplicó el método ANOVA de análisis de la varianza y el test de Scheffé. También se calculó el área del espacio vocal. Resultados: El valor medio del F1 en todas las vocales no mostró diferencias significativas en los 3 grupos de niños. Para las vocales /i/, /o/ y /u/ el valor medio del F2 fue significativamente diferente entre los 2 grupos de niños hipoacúsicos y el grupo de niños normooyentes. Conclusión: Ambos grupos de niños con hipoacusia prelocutiva tienden a presentar sutiles desviaciones en la articulación de las vocales que pueden ser analizadas mediante un programa de análisis acústico

Introduction and objectives: Inadequate auditory feedback in prelingually deaf children alters the articulation of consonants and vowels. The purpose of this investigation was to compare vowel production in Spanish-speaking deaf children with cochlear implantation, and with hearing-aids with normal-hearing children by means of acoustic analysis of formant frequencies and vowel space. Methods: A total of 56 prelingually deaf children (25 with cochlear implants and 31 wearing hearing-aids) and 47 normal-hearing children participated. The first 2 formants (F1 and F2) of the five Spanish vowels were measured using Praat software. One-way analysis of variance (ANOVA) and post hoc Scheffé test were applied to analyze the differences between the 3 groups. The surface area of the vowel space was also calculated. Results: The mean value of F1 in all vowels was not significantly different between the 3 groups. For vowels /i/, /o/ and /u/, the mean value of F2 was significantly different between the 2 groups of deaf children and their normal-hearing peers. Conclusion: Both prelingually hearing-impaired groups tended toward subtle deviations in the articulation of vowels that could be analyzed using an objective acoustic analysis programme

Validez de las medidas del pico cepstral para la valoración objetiva de la disfonía en sujetos de habla hispana / Validation of the measures of cepstral peak prominence as a measure of dysphonia severity in Spanish-speaking subjects

Introducción y objetivos: La fiabilidad de las calificaciones perceptuales de la calidad global de la voz y sus dimensiones específicas es difícil de alcanzar, por cuanto estos juicios dependen de la subjetividad del examinador. De este modo, la búsqueda de unas medidas clínicas que sean objetivas, válidas y accesibles es una prioridad para incluirlas en los protocolos de evaluación de la voz. El propósito del presente estudio fue: 1) determinar la exactitud diagnóstica de un único parámetro acústico, la prominencia del pico cepstral suavizado (CPPS), para predecir la presencia y severidad de una disfonía en vocales sostenidas y habla conectada utilizando el programa Praat; 2) determinar la relación entre las medidas del CPPS y las calificaciones perceptuales de la calidad vocal; y 3) describir los valores normativos del CPPS. Método: Se obtuvo el valor del CPPS de muestras vocales sostenidas y de habla conectada de 72 sujetos hispanohablantes con trastornos vocales y de 52 sujetos hispanohablantes sanos utilizando el programa Praat. Se llevó a cabo un estudio estadístico completo utilizando el programa SPSS de IBM versión 23. Resultados: Se encontró una sensibilidad de un 70% y una especificidad de un 85%. La severidad estimada de la voz, tanto en vocal sostenida como en habla conectada, se correlacionó de forma importante con las calificaciones perceptuales de severidad global de la disfonía. Conclusiones: Un parámetro acústico único, el CPPS calculado mediante el programa Praat, predice de forma importante el estatus de un trastorno vocal. Se considera la incorporación del CPPS para complementar la valoración clínica de la voz

Introduction and objectives: Perceptual rating of overall voice quality and other more specific perceptual dimensions is difficult, as such judgments depend on the listener's subjectivity. Thus, finding objective, valid, and accessible clinical measures to include in comprehensive voice evaluation protocols is a priority. The purposes of this study were to 1) determine the diagnostic accuracy of a single acoustic measure, smoothed cepstral peak prominence (CPPS), to predict voice disorder status from sustained vowels and connected speech samples using the software Praat; 2) to determine the relationship between measures of CPPS and perceptual ratings of vocal quality; and 3) describe the normative values of CPPS. Method: Measures of CPPS were obtained from connected speech and sustained vowel recordings of 72 Spanish-speaking subjects with voice disorders and 52 nondysphonic Spanish-speaking subjects with no vocal disorders using freely downloadable Praat software. IBM SPSS Statistics software version 23 was used to complete the statistical analyses. Results: results revealed a 70% sensitivity rate, a specificity rate of 85%. Estimated severity for sustained vowels and connected speech were strongly correlated and significantly associated with listener ratings of dysphonia severity. Conclusions: A single acoustic measure of CPPS was highly predictive of voice disorder status using Praat software. Clinicians may consider using CPPS to complement clinical voice evaluation and screening protocols

Evaluation of vaccination coverage in cochlear implant patients at a referral hospital in Northern Spain. / Evaluación de las coberturas de vacunación en pacientes con implante coclear de un hospital de referencia del norte de España.

BACKGROUND AND OBJECTIVE: The risk of bacterial meningitis increases in cochlear implant patients. Therefore, pneumococcal, influenza and Haemophilus influenzae type b vaccination is indicated in this group. The aim of this study was to determine compliance with the vaccination calendar in patients implanted in a referral hospital. MATERIALS AND METHODS: Patients with cochlear implant operated between 2005 and 2015 were included. Vaccine coverage for seasonal influenza, Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes and pneumococcal polysaccharide 23-serotypes was evaluated. The sample was divided into 2 age groups (<14 years and≥14 years). A univariate and bivariate analysis was performed. RESULTS: Of the 153 patients studied (28.01% 0-13 years old and 71.9%≥14), only 2 (5.71%) had 100% adherence to the vaccination schedule, while 65.71% had compliance of 50% or less. Overall, vaccination coverage against the sequential pneumococcal pattern was 48.57%. The paediatric population exceeded 90% coverage for the vaccine against Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes while in those over 14 years of age it barely exceeded 50%. Influenza coverage was less than 40%. An inverse correlation was obtained between age and compliance, although not statistically significant. CONCLUSIONS: Vaccination coverage in patients with cochlear implant is lower than expected. Close collaboration between Otolaryngology departments and the Vaccination Units is proposed as the main strategy for improvement.

Vowel production in hearing impaired children: A comparison between normal-hearing, hearing-aided and cochlear-implanted children. / Comparación de la producción vocal en niños con hipoacusia prelocutiva con adaptación audioprotésica e implante coclear con normooyentes.

INTRODUCTION AND OBJECTIVES: Inadequate auditory feedback in prelingually deaf children alters the articulation of consonants and vowels. The purpose of this investigation was to compare vowel production in Spanish-speaking deaf children with cochlear implantation, and with hearing-aids with normal-hearing children by means of acoustic analysis of formant frequencies and vowel space. METHODS: A total of 56 prelingually deaf children (25 with cochlear implants and 31 wearing hearing-aids) and 47 normal-hearing children participated. The first 2 formants (F1 and F2) of the five Spanish vowels were measured using Praat software. One-way analysis of variance (ANOVA) and post hoc Scheffé test were applied to analyze the differences between the 3 groups. The surface area of the vowel space was also calculated. RESULTS: The mean value of F1 in all vowels was not significantly different between the 3 groups. For vowels /i/, /o/ and /u/, the mean value of F2 was significantly different between the 2 groups of deaf children and their normal-hearing peers. CONCLUSION: Both prelingually hearing-impaired groups tended toward subtle deviations in the articulation of vowels that could be analyzed using an objective acoustic analysis programme.

Validation of the measures of cepstral peak prominence as a measure of dysphonia severity in Spanish-speaking subjects. / Validez de las medidas del pico cepstral para la valoración objetiva de la disfonía en sujetos de habla hispana.

INTRODUCTION AND OBJECTIVES: Perceptual rating of overall voice quality and other more specific perceptual dimensions is difficult, as such judgments depend on the listener's subjectivity. Thus, finding objective, valid, and accessible clinical measures to include in comprehensive voice evaluation protocols is a priority. The purposes of this study were to 1) determine the diagnostic accuracy of a single acoustic measure, smoothed cepstral peak prominence (CPPS), to predict voice disorder status from sustained vowels and connected speech samples using the software Praat; 2) to determine the relationship between measures of CPPS and perceptual ratings of vocal quality; and 3) describe the normative values of CPPS. METHOD: Measures of CPPS were obtained from connected speech and sustained vowel recordings of 72 Spanish-speaking subjects with voice disorders and 52 nondysphonic Spanish-speaking subjects with no vocal disorders using freely downloadable Praat software. IBM SPSS Statistics software version 23 was used to complete the statistical analyses. RESULTS: results revealed a 70% sensitivity rate, a specificity rate of 85%. Estimated severity for sustained vowels and connected speech were strongly correlated and significantly associated with listener ratings of dysphonia severity. CONCLUSIONS: A single acoustic measure of CPPS was highly predictive of voice disorder status using Praat software. Clinicians may consider using CPPS to complement clinical voice evaluation and screening protocols.

Aplicación del índice acústico de calidad vocal para la cuantificación objetiva de la severidad de la disfonía / Application of the acoustic voice quality index for objective measurement of dysphonia severity

Introducción: En las últimas décadas se han estudiado muchos parámetros acústicos con el fin de cuantificar la disfonía, sin embargo las medidas actuales podrían no ser sensibles a la calidad vocal percibida. En un metaanálisis donde se evaluó la relación entre la calidad global percibida de la voz se identificaron numerosas medidas acústicas que no dependen de la extracción del periodo fundamental tales como las derivadas del cepstrum y que pueden ser usadas tanto en vocales sostenidas como en habla conectada. Un método específico recientemente diseñado para cuantificar la severidad global de la disfonía es el índice acústico de calidad vocal (AVQI) que es un constructo multivariante que combina múltiples marcadores acústicos para generar un único número que se correlaciona razonablemente con la calidad global vocal. Métodos: Esta investigación se basó en un archivo de voces grabadas procedentes de dos grupos de individuos: 60 personas sanas vocalmente y 58 participantes con trastornos vocales. Se grabaron vocales sostenidas y una muestral de habla conectada para su análisis y así obtener los seis parámetros incluidos en el AVQI utilizando el programa Praat. El análisis estadístico de los resultados se llevó a cabo con el programa SPSS para Windows versión 12.0. Resultados: Correspondencia entre el valor del AVQI y el grado de severidad global de la disfonía: correlación r = 0,68 (p < 0,000), y entre el AVQI y el parámetro B del GRABS: r = 0,68 (p < 0,000). Conclusiones: Los resultados obtenidos muestran una buena correlación con la calidad vocal percibida. En conclusión, el AVQI es una medida multivariante, accesible, factible y razonablemente válida para medir clínicamente la severidad global de la severidad de la disfonía (AU)

Introduction: Over the past several decades, many acoustic parameters have been studied as sensitive to and to measure dysphonia. However, current acoustic measures might not be sensitive measures of perceived voice quality. A meta-analysis which evaluated the relationship between perceived overall voice quality and several acoustic-phonetic correlates, identified measures that do not rely on the extraction of the fundamental period, such the measures derived from the cepstrum, and that can be used in sustained vowel as well as continuous speech samples. A specific and recently developed method to quantify the severity of overall dysphonia is the acoustic voice quality index (AVQI) that is a multivariate construct that combines multiple acoustic markers to yield a single number that correlates reasonably with overall vocal quality. Methods: This research is based on one pool of voice recordings collected in two sets of subjects: 60 vocally normal and 58 voice disordered participants. A sustained vowel and a sample of connected speech were recorded and analyzed to obtain the six parameters included in the AVQI using the program Praat. Statistical analysis was completed using SPSS for Windows, version 12.0. Results: Correlation between perception of overall voice quality and AVQI: A significant difference exists (t (95) = 9.5; p<.000) between normal and dysphonic voices. Conclusions: The findings of this study demonstrate the clinical feasibility of the AVQI as a measure of dysphonia severity (AU)

Application of the acoustic voice quality index for objective measurement of dysphonia severity. / Aplicación del índice acústico de calidad vocal para la cuantificación objetiva de la severidad de la disfonía.

INTRODUCTION: Over the past several decades, many acoustic parameters have been studied as sensitive to and to measure dysphonia. However, current acoustic measures might not be sensitive measures of perceived voice quality. A meta-analysis which evaluated the relationship between perceived overall voice quality and several acoustic-phonetic correlates, identified measures that do not rely on the extraction of the fundamental period, such the measures derived from the cepstrum, and that can be used in sustained vowel as well as continuous speech samples. A specific and recently developed method to quantify the severity of overall dysphonia is the acoustic voice quality index (AVQI) that is a multivariate construct that combines multiple acoustic markers to yield a single number that correlates reasonably with overall vocal quality. METHODS: This research is based on one pool of voice recordings collected in two sets of subjects: 60 vocally normal and 58 voice disordered participants. A sustained vowel and a sample of connected speech were recorded and analyzed to obtain the six parameters included in the AVQI using the program Praat. Statistical analysis was completed using SPSS for Windows, version 12.0. RESULTS: Correlation between perception of overall voice quality and AVQI: A significant difference exists (t(95) = 9.5; p<.000) between normal and dysphonic voices. CONCLUSIONS: The findings of this study demonstrate the clinical feasibility of the AVQI as a measure of dysphonia severity.

Fiabilidad de los potenciales evocados auditivos de estado estable en la fase diagnóstica del cribado neonatal universal de la hipoacusia / Auditory-steady-state response reliability in the audiological diagnosis after neonatal hearing screening

Introducción y objetivos: La audiometría convencional es el patrón de referencia para cuantificar y describir una hipoacusia. Sin embargo, se precisan métodos alternativos para los pacientes muy jóvenes que no pueden responder con fiabilidad. Los potenciales evocados auditivos de tronco (PEATC) es el método más extendido para determinar de forma objetiva los umbrales auditivos, sin embargo, no informan específicamente en cada frecuencia. La llegada de los potenciales evocados de estado estable (PEAEE) permite conseguir una determinación más específica en frecuencias. El presente trabajo describe y compara los umbrales obtenidos mediante PEATC, PEAEE y audiometría convencional en un grupo de niños que presentan varios grados de pérdida auditiva. Métodos: Se llevó a cabo una comparación entre los umbrales de PEATC, PEAEE y audiométricos obtenidos de 35 niños detectados en el programa de cribado auditivo neonatal. Resultados: La diferencia media (±DE) entre los umbrales de los PEATC y la banda de 4.000Hz de los PEAEE fueron 11,2 dB (±13) para el oído derecho y 10,2 dB (±11) para el izquierdo. Las correlaciones de Pearson entre los umbrales de los PEAEE y audiométricos fue 0,80 y 0,91 (500Hz); 0,84 y 0,82 (1.000Hz); 0,85 y 0,84 (2.000Hz); 0,83 y 0,82 (4.000Hz), respectivamente para oído derecho e izquierdo. Conclusión: La técnica de los PEAEE es una técnica a tener en cuenta para su inclusión en la batería de test que se emplea en el diagnóstico audiológico de los niños con hipoacusia (AU)

Introduction and objectives: Conventional audiometry is the gold standard for quantifying and describing hearing loss. Alternative methods become necessary to assess subjects who are too young to respond reliably. Auditory evoked potentials constitute the most widely used method for determining hearing thresholds objectively; however, this stimulus is not frequency specific. The advent of the auditory steady-state response (ASSR) leads to more specific threshold determination. The current study describes and compares ASSR, auditory brainstem response (ABR) and conventional behavioural tone audiometry thresholds in a group of infants with various degrees of hearing loss. Methods: A comparison was made between ASSR, ABR and behavioural hearing thresholds in 35 infants detected in the neonatal hearing screening program. Results: Mean difference scores (±SD) between ABR and high frequency ABR thresholds were 11.2 dB (±13) and 10.2 dB (±11). Pearson correlations between the ASSR and audiometry thresholds were 0.80 and 0.91 (500Hz); 0.84 and 0.82 (1000Hz); 0.85 and 0.84 (2000Hz); and 0.83 and 0.82 (4000Hz). Conclusion: The ASSR technique is a valuable extension of the clinical test battery for hearing-impaired children (AU)

Auditory-steady-state response reliability in the audiological diagnosis after neonatal hearing screening. / Fiabilidad de los potenciales evocados auditivos de estado estable en la fase diagnóstica del cribado neonatal universal de la hipoacusia.

INTRODUCTION AND OBJECTIVES: Conventional audiometry is the gold standard for quantifying and describing hearing loss. Alternative methods become necessary to assess subjects who are too young to respond reliably. Auditory evoked potentials constitute the most widely used method for determining hearing thresholds objectively; however, this stimulus is not frequency specific. The advent of the auditory steady-state response (ASSR) leads to more specific threshold determination. The current study describes and compares ASSR, auditory brainstem response (ABR) and conventional behavioural tone audiometry thresholds in a group of infants with various degrees of hearing loss. METHODS: A comparison was made between ASSR, ABR and behavioural hearing thresholds in 35 infants detected in the neonatal hearing screening program. RESULTS: Mean difference scores (±SD) between ABR and high frequency ABR thresholds were 11.2 dB (±13) and 10.2 dB (±11). Pearson correlations between the ASSR and audiometry thresholds were 0.80 and 0.91 (500Hz); 0.84 and 0.82 (1000Hz); 0.85 and 0.84 (2000Hz); and 0.83 and 0.82 (4000Hz). CONCLUSION: The ASSR technique is a valuable extension of the clinical test battery for hearing-impaired children.

Adenocarcinomas nasosinusales tipo intestinal. Perfil inmunohistoquímico de 66 casos / Intestinal-type sinonasal adenocarcinomas. Immunohistochemical profile of 66 cases

Introducción y objetivos: Los adenocarcinomas nasosinusales tipo intestinal son tumores epiteliales malignos, que suponen el 8-25% de los tumores malignos nasosinusales. Se relacionan con la exposición al polvo de la madera. Se subdividen histológicamente en papilares, colónicos, sólidos y mucinosos. Realizamos un estudio patológico e inmunohistoquímico con el fin de establecer características con significado pronóstico, diagnóstico e incluso terapéutico, así como comparar con estudios previos. Métodos: Estudiamos 66 muestras tumorales mediante matrices tisulares. Realizamos tinciones inmunohistoquímicas para p53, p16, beta-catenina, E-cadherina, receptor del factor de crecimiento epidérmico (EGFR), receptor 2 de factor de crecimiento epidérmico humano (HER2/neu) y ciclooxigenasa 2 (COX-2). Resultados: Un 63% de los casos son positivos para p53, el 37% para beta-catenina nuclear, el 100% para E-cadherina, el 98% para beta-catenina membranosa, el 7% para EGFR, el 8% para HER2/neu, el 52% para COX-2 y el 59% pierden la expresión de p16. Conclusiones: La invasión intracraneal es el factor clínico pronóstico más importante. Los tumores de tipo sólido y mucinoso son los que muestran un comportamiento más agresivo, siendo los mucinosos los que mayor invasión intracraneal muestran. No existen diferencias inmunohistoquímicas entre los distintos subtipos histológicos, únicamente la tinción débil para E-cadherina y beta-catenina, más frecuente en los de tipo mucinoso. El EGFR, HER2/neu y COX-2 muestran una positividad menos frecuente que en series previas. La positividad para p16 se asocia a una menor supervivencia y mayor frecuencia de enfermedad metastásica.Palabras clave Adenocarcinoma nasosinusal. Carcinoma nasosinusal. Inmunohistoquímica. p53. p16. beta-catenina. E-cadherina. Receptor del factor de crecimiento epidérmico. Receptor 2 de factor de crecimiento epidérmico humano. Cyclooxigenasa-2 (AU)

Introduction and objectives: Intestinal-type sinonasal adenocarcinomas are malignant epithelial tumours. Around 8-25% of all sinonasal malignant tumours are intestinal-type adenocarcinomas, which are related to wood dust exposure. Four histological subtypes have been described: papillary, colonic, solid and mucinous. We performed a pathological and immunohistochemical study in order to describe characteristics with prognostic, diagnostic and therapeutic value, and also to compare our results with previous studies. Methods: Sixty six tumour samples were analysed and protein expression of p53, p16, E-cadherin, beta-catenin, epidermal growth factor receptor (EGFR), human epidermal growth factor receptor 2 (HER2/neu) and cyclooxygenase-2 (COX-2) was performed by tissue microarray blocks. Results: The 63% of cases were p53 positive; 37% showed nuclear staining with beta-catenin and 100% with E-cadherin, while 98% showed membrane staining with beta-catenin, 7% with EGFR, 8% with HER2/neu and 52% with COX-2; and 59% of the cases lost p16 expression. Conclusions: Intracranial invasion was the worst prognostic associated event. Solid and mucinous tumours were the most aggressive histological subtypes. Intracranial invasion was more frequent in mucinous subtype tumours. Immunohistochemical results were similar in all tumour subtypes, except for mucinous tumours, which showed weak expression of E-cadherin and beta-catenin. Comparing with previous studies, we found a lower expression of EGFR, HER2/neu and COX-2. The p16 expression was associated with worse survival and metastatic disease (AU)

Medullary thyroid carcinoma and 2q37 deletion in a patient with nevoid basal cell carcinoma syndrome: clinical description and genetic analysis.

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, inheritable, multisystem disorder characterized by numerous basal cell carcinomas (BCCs), maxillary keratocyst, and musculoskeletal malformations. Occasionally, it is associated with malignancies like rhabdomyoma, melanoma, and sinonasal undifferentiated carcinoma, to name a few. METHODS: A patient presented with NBCCS with a medullary thyroid carcinoma. Clinical, surgical details, and germline genetic analysis are herein described. RESULTS: A 32-year-old woman was referred to our department with suspicion of medullary thyroid carcinoma, which was confirmed by histopathological examination. The patient was diagnosed as also having NBCCS. Germline mutation analysis indicated wild-type genes PTCH1 and RET. DNA copy number analysis by high resolution microarray comparative genomic hybridization (CGH) revealed a small interstitial loss at chromosomal band 2q37.3. CONCLUSION: To our knowledge, this is the first described patient with NBCCS carrying a medullary thyroid carcinoma and a 2q37 deletion, which confirms that this syndrome can be associated with many different malignancies.

Intestinal-type sinonasal adenocarcinomas. Immunohistochemical profile of 66 cases.

INTRODUCTION AND OBJECTIVES: Intestinal-type sinonasal adenocarcinomas are malignant epithelial tumours. Around 8-25% of all sinonasal malignant tumours are intestinal-type adenocarcinomas, which are related to wood dust exposure. Four histological subtypes have been described: papillary, colonic, solid and mucinous. We performed a pathological and immunohistochemical study in order to describe characteristics with prognostic, diagnostic and therapeutic value, and also to compare our results with previous studies. METHODS: Sixty six tumour samples were analysed and protein expression of p53, p16, E-cadherin, ß-catenin, epidermal growth factor receptor (EGFR), human epidermal growth factor receptor 2 (HER2/neu) and cyclooxygenase-2 (COX-2) was performed by tissue microarray blocks. RESULTS: The 63% of cases were p53 positive; 37% showed nuclear staining with ß-catenin and 100% with E-cadherin, while 98% showed membrane staining with ß-catenin, 7% with EGFR, 8% with HER2/neu and 52% with COX-2; and 59% of the cases lost p16 expression. CONCLUSIONS: Intracranial invasion was the worst prognostic associated event. Solid and mucinous tumours were the most aggressive histological subtypes. Intracranial invasion was more frequent in mucinous subtype tumours. Immunohistochemical results were similar in all tumour subtypes, except for mucinous tumours, which showed weak expression of E-cadherin and ß-catenin. Comparing with previous studies, we found a lower expression of EGFR, HER2/neu and COX-2. The p16 expression was associated with worse survival and metastatic disease.