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.

Desarrollo y validación de la versión abreviada del Transsexual Voice Questionnaire for Male-to-Female Transsexuals en español / Development and validation of the Spanish abbreviated version of the Transsexual Voice Questionnaire for Male-to-Female Transsexuals

OBJETIVO: El objetivo de este estudio es crear y validar un cuestionario abreviado de la versión española del Transsexual Voice Questionnaire for Male-to-Female Transsexuals (VeTVQMtF). METODOLOGÍA: El estudio fue dirigido por 2 hospitales de referencia para la feminización quirúrgica de la voz y por un departamento universitario de psicología y rehabilitación vocal, todos ellos en España. MATERIAL Y MÉTODOS: Se realizó un estudio prospectivo de 51 mujeres transexuales a las que se les intervino de feminización quirúrgica de la voz entre enero 2017 y diciembre 2018. La VeTVQMtF fue rellenada por las mujeres transexuales antes y después de la cirugía y los 10 ítems de este cuestionario que más variaron fueron seleccionados por un grupo de expertos en feminización de la voz, para desarrollar la versión abreviada de la VeTVQMtF (VeTVQMtF-10). Se estudió la correlación entre la puntuación total y la puntuación de cada ítem en la VeTVQMtF y en la VeTVQMtF-10. Se analizó la consistencia interna de la VeTVQMtF-10. RESULTADOS: Se encontró una buena correlación entre los 2 cuestionarios (coeficiente de Pearson > 0,90), una buena correlación entre la puntuación total y la puntuación de cada ítem de la VeTVQMtF-10 y una correlación negativa entre la de la VeTVQMtF y la frecuencia fundamental de la voz tras la cirugía. El alfa Cronbach fue de 0,79. CONCLUSIÓN: La VeTVQMtF-10 es una versión abreviada válida de la VeTVQMtF y podría usarse para valorar la calidad de vida relacionada con la voz en mujeres transexuales en el idioma español

OBJECTIVE: The aim of this study was to create and validate an abbreviated version of the Spanish Transsexual Voice Questionnaire for Male-to-Female Transsexuals (SvTVQMtF). SETTING: The study was conducted by two referral hospitals for voice feminization surgery and by a university department of psychology and speech therapy, all in Spain. Subjects and methods: We prospectively studied 51 male-to-female transsexuals who underwent voice feminization surgery between January 2017 and December 2018. The SvTVQMtF was completed before and after surgery, and the 10 items with the greatest variation were selected by clinical consensus of an expert panel to develop the short version of the SvTVQMtF (SvTVQMtF-10). The correlation between the total score and the score for each item on the SvTVQMtF and the SvTVQMtF-10 was studied. The internal consistency of the SvTVQMtF-10 was analysed. RESULTS: Good correlation (Pearson coefficient above .90) was found between the two questionnaires. A significant correlation was found between the total SvTVQMtF-10 score and the score for each item. A significant negative correlation was found between the SvTVQMtF and fundamental frequency after voice feminization surgery. Cronbach's Alpha was .79. CONCLUSION: The SvTVQMtF-10 is a valid short version of the SvTVQMtF and can be used to quantify voice-related quality of life in MtF transsexuals

Development and validation of the Spanish abbreviated version of the Transsexual Voice Questionnaire for Male-to-Female Transsexuals. / Desarrollo y validación de la versión abreviada del Transsexual Voice Questionnaire for Male-to-Female Transsexuals en español.

OBJECTIVE: The aim of this study was to create and validate an abbreviated version of the Spanish Transsexual Voice Questionnaire for Male-to-Female Transsexuals (SvTVQMtF). SETTING: The study was conducted by two referral hospitals for voice feminization surgery and by a university department of psychology and speech therapy, all in Spain. SUBJECTS AND METHODS: We prospectively studied 51 male-to-female transsexuals who underwent voice feminization surgery between January 2017 and December 2018. The SvTVQMtF was completed before and after surgery, and the 10 items with the greatest variation were selected by clinical consensus of an expert panel to develop the short version of the SvTVQMtF (SvTVQMtF-10). The correlation between the total score and the score for each item on the SvTVQMtF and the SvTVQMtF-10 was studied. The internal consistency of the SvTVQMtF-10 was analysed. RESULTS: Good correlation (Pearson coefficient above .90) was found between the two questionnaires. A significant correlation was found between the total SvTVQMtF-10 score and the score for each item. A significant negative correlation was found between the SvTVQMtF and fundamental frequency after voice feminization surgery. Cronbach's α was .79. CONCLUSION: The SvTVQMtF-10 is a valid short version of the SvTVQMtF and can be used to quantify voice-related quality of life in MtF transsexuals.

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

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.