Psychometric properties of the Adolescents’Academic Motivation Scale (AAMS)in a representative sample of Dominican Republic high school students

Entre el creciente cuerpo de investigación que se ha centrado en el éxito académico, la motivación académica ha captado considerable atención. El objetivo de esta investigación es presentar la primera validación de la Escala de Motivación Académica de los Adolescentes (EMAA). La muestra total estuvo compuesta por 1712 estudiantes de secundaria de dos distritos de la República Dominicana. La medida principal fue la EMAA. Los resultados del AFC fueron satisfactorios: χ2(5) = 57.73, p < .001; CFI = .970; RMSEA = .079, 90% CI [.061, .097], y SRMR = .024. Los análisis de TRI favorecieron al modelo logístico de dos parámetros, indicando que los ítems no fueron igualmente discriminativos. El Modelo de Ecuaciones Estructurales en el que la motivación académica predecía de forma estadísticamente significativa las calificaciones obtuvo un ajuste excelente: χ2(53) = 182.76, p < .001; CFI = .980; RMSEA = .038 [.032, .044], and SRMR = .025. En resumen, este trabajo presenta un exhaustivo análisis psicométrico de la EMAA en una muestra representativa de estudiantes dominicanos de instituto.(AU)

Within the growing body of research that has focused on aca-demic success, academic motivation has gathered considerable attention. The aim of this this research is to present the first validation of the Ado-lescents’ Academic Motivation Scale (AAMS). Total sample was composed by 1712 students of secondary education from two districts in the Domini-can Republic. The main measurement outcome was the AAMS. Results from the CFA were satisfactory: χ2(5) = 57.73, p< .001; CFI = .970; RMSEA = .079 [.061, .097], and SRMR = .024. IRT analyses favored the two-parameter logistic model, indicating that items were not equallydis-criminant. Structural Equation Model with latent variables in which aca-demic motivation was a significant predictor of grades resulted in excellent fit: χ2(53) = 182.76, p< .001; CFI = .980; RMSEA = .038 90% CI [.032, .044], and SRMR = .025. In sum, this work presents an exhaustive psy-chometric analysis of the AAMS in a representative sample of high school Dominican students.(AU)

Satisfacción Laboral en el Docente Dominicano: Antecedentes Laborales / Job Satisfaction of the Dominican Teacher: Labor Background / Satisfação Profissional no Docente Dominicano: Antecedentes Profissionais

Resumen La satisfacción laboral del colectivo docente ha sido estudiada desde diversas perspectivas, debido a los importantes efectos que esta puede tener tanto a nivel económico como a nivel organizacional. La muestra empleada en este trabajo se compone de 978 docentes dominicanos, y fue obtenida mediante un muestreo estratificado, y, por tanto, representativo de la población. En este estudio se examina un modelo de ecuaciones estructurales en el cual el contexto de trabajo y las condiciones laborales afectan el burnout y el engagement de los profesores. A su vez, se plantea que burnout y engagement afectan la satisfacción laboral docente. El modelo ajustó a los datos: x2(223)= 1089.9, p<.001; CFI=.93; RMSEA=.066; SRMR=.107. Los resultados arrojan luz sobre los factores contextuales del plano educativo que pudieran replantearse en el desarrollo de futuras políticas educativas.

Abstract The job satisfaction of the teachers' collective has been studied from various perspectives due to the significant effect it can have at both the economic and organizational levels. The sample used for this study was made up of 978 Dominican teachers, obtained through stratified sampling so that it would be representative of the population. An analysis of a structural equations model shows that work context and labor conditions affect the burnout and engagement of teachers. The study suggests that both burnout and engagement affect the job satisfaction of teachers. The model fits the data: x2(223)=1089.9, p<.001; CFI=.93; RMSEA=.066; SRMR=.107. The results shed light on the contextual factors of the educational field that could be reformulated in the development of future education policies.

Resumo A satisfação profissional do coletivo docente vem sendo estudada de diversas perspectivas, devido aos importantes efeitos que ela pode ter tanto no âmbito econômico quanto no organizacional. A amostra deste trabalho está composta por 978 docentes dominicanos e foi obtida mediante uma amostragem estratificada, e, portanto, representativa da população. Neste estudo, foi examinado um modelo de equações estruturais em que o contexto de trabalho e as condições de trabalho afetam o burnout e o engagement dos professores. Por sua vez, argumenta-se que burnout e engagement afetam a satisfação profissional docente. O modelo ajusta-se aos dados: x2(223)=1089.9, p<.001; CFI=.93; RMSEA=.066; SRMR=.107. Os resultados esclarecem os fatores contextuais do plano educativo que podem ser repensados no desenvolvimento de futuras políticas educativas.

Utrecht Work Engagement Scale in Dominican teachers: dimensionality, reliability, and validity / Escala de Utrecht de implicación en el trabajo en los profesores dominicanos: dimensionalidad, fiabilidad y validez

Work engagement is described by dedication, vigor, and absorption. The most widely used measure of engagement is the Utrecht Work Engagement Scale (UWES), intended to measure engagement for any occupational group. This research aims to study psychometric properties of the UWES for its use in the Dominican Republic and other Caribbean Spanish-speaking countries. The Composite Reliability Index (CRI) as well as alphas were calculated, indicating good internal consistency. Confirmatory factor analyses were carried out to test its dimensionality. Both tested models showed extremely good fit to the data, which called for model comparison. The three-factor solution was retained as the one showing the best relative fit. However, the three dimensions of the scale were largely correlated, providing evidence for some overlapping. Regarding criterion-related validity, the three factors were correlated as expected with the three dimensions of burnout, working climate, and working conditions

La implicación en el trabajo se describe por la dedicación, el vigor y la absorción. La medida más utilizada de implicación es la Escala de Utrecht de Implicación en el Trabajo (UWES), cuyo propósito es medir la implicación en cualquier grupo ocupacional. Esta investigación tiene como objetivo estudiar las propiedades psicométricas de la UWES para su uso en la República Dominicana y otros países del Caribe de habla hispana. Se calculó el Índice de Fiabilidad Compuesto (CRI), así como el alfa, indicando una buena consistencia interna. Se llevaron a cabo análisis factoriales confirmatorios para probar su dimensionalidad. Ambos modelos se ajustaron muy bien a los datos, lo que llevó a la comparación entre estos. La solución de tres factores resultó ser la que mostraba el mejor ajuste relativo. Sin embargo, las tres dimensiones de la escalase correlacionaban en buena medida, lo que prueba evidencia de solapamiento. En relación con la validez de criterio, como se esperaba, los tres factores correlacionaron con las tres dimensiones de burnout, con el clima de trabajo y con las condiciones de trabajo

Seudotumor inflamatorio subglótico en un niño de 3 años / Subglottis inflammatory pseudotumour in a 3-year-old child

INTRODUCCIÓN: El cierre quirúrgico de las perforaciones septales sintomáticas a menudo conduce a resultados no satisfactorios. El conocimiento de la irrigación vascular es de suma importancia para el éxito. OBJETIVO: El manejo de las perforaciones septales constituye un reto para el cirujano. Hay descritas una gran variedad de técnicas quirúrgicas, con distintas vías de abordaje. No existen pruebas científicas que avalen un abordaje en concreto. El objetivo de esta revisión es presentar una guía práctica sobre la técnica de elección para cada caso de perforación septal. DISCUSIÓN: La inspección de la mucosa nasal, el tamaño de la perforación, la localización y, sobre todo, el soporte osteocartilaginoso son los pilares para lograr el éxito de la cirugía. Para los colgajos de deslizamiento o rotación de la mucosa del tabique es fundamental haber estudiado previamente si es posible la elevación del mucopericondrio o mucoperiosteo del septum, de lo contrario, el uso de estos colgajos no estaría indicado. Los colgajos de la pared lateral o del suelo nasal son la alternativa. El colgajo pericraneal podría estar indicado en perforaciones totales o casi totales. CONCLUSIÓN: El remanente del septum nasal y el estado del soporte osteocartilaginoso son los factores determinantes en el manejo de las perforaciones septales. Cada caso debe valorarse individualmente y la elección del abordaje se realiza según el tamaño y la localización de la perforación, la calidad de la mucosa, los antecedentes personales, la cirugía previa y la experiencia del cirujano

OBJECTIVE: The management of septal perforations is a challenge for the surgeon. A wide variety of surgical techniques have been described, with different approaches. There is no scientific evidence to support a particular approach. The objective of this review is to present a practical guide on the technique of choice for each case of septal perforation. DISCUSSION: Inspection of the nasal mucosa, the size of the perforation, the location and especially the osteo-cartilaginous support, are the pillars of a successful surgery. For the sliding or rotating flaps of the mucosa of the septum it is essential to know in advance if the elevation of the mucopericondrio or mucoperiosteo of the septum is possible, otherwise the use of these flaps would not be indicated. The flaps of the lateral wall or nasal floor are the alternative. The pericranial flap may be indicated in total or near total perforations. CONCLUSION: The remnant of the nasal septum and status of osteo-cartilaginous support are the determining factors in the management of septal perforations. Each case should be evaluated individually and the approach chosen according to the size and location of the perforation, mucosal quality, personal history, previous surgery and the experience of the surgeon

Early Diagnosis of CAPOS Syndrome Before Acute-Onset Ataxia-Review of the Literature and a New Family.

BACKGROUND: CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare disease that has been reported in 22 patients so far. In all cases, the mutation c.2452G>A (p.Glu818Lys) in the ATP1A3 gene was identified. Patients typically present at an early age with an acute-onset fever-induced episode of ataxia frequently associated with encephalopathy and weakness. They usually present one to three episodes. The acute symptoms improve within days, but most patients show slow progression afterward. METHODS: We describe three new patients, a woman and her two sons diagnosed with CAPOS syndrome. A systematic review of literature on previously reported patients was performed. RESULTS: The first son presented with acute-onset ataxia, encephalopathy, and sensorineural hearing loss, induced by febrile illness. The second one developed generalized areflexia and mild instability without an acute episode. The mother had been previously diagnosed with sensorineural hearing loss and optic nerve atrophy. The c.2452G>A mutation in ATP1A3 was found in all three patients. CONCLUSION: Only 25 Individuals with CAPOS syndrome have been reported, including our family. This is the first time a Spanish family has been described. The fact that both siblings were assessed before the first acute-onset episode contributes to the description of early symptoms and signs of the disease, which could aid early diagnosis and management before the onset of acute episodes.

Validation of the Maslach Burnout Inventory-General Survey on a Representative Sample of Dominican Teachers: Normative Data.

Burnout is characterized by emotional exhaustion, depersonalization and lack of personal accomplishment (Bakke, Demerouti, & Sanz-Vergel, 2014). Several instruments for its measurement exist, but the most widely used scale for measuring its dimensions, by far, is the Maslach Burnout Inventory (MBI) in its different versions. Among the available versions of the scale, the MBI-General Survey was developed to measure three dimensions of burnout (cynicism, personal accomplishment, and emotional exhaustion) regardless of the type of work. The aim of this research is to offer evidence on the psychometric properties of the MBI-GS for its use in the Dominican Republic and other Caribbean Spanish-speaking countries, using representative sample of Dominican teachers. The factorial validity was studied through confirmatory factor analysis. Several competing models were proved in order to test the dimensionality of the scale. The confirmatory analyses shown that the original three-factor structure had a superior fit, but item eleven was removed in order to get an excellent fit χ2(87) = 211.19, p < .001, CFI = .98, RMSEA = .038 90% CI [.032-.045]. Regarding internal consistency, the CRI´s are well above the cut-off criteria of .7 (CRI's ranged from .74 to .86). Concerning criterion-related validity, the three factors were correlated in the expected direction. Professional efficacy, a dimension of burnout measured in the opposite direction, was positively correlated with the three factors of work engagement, also as expected. This version was found to be a psychometrically sound measure of the three core dimensions of burnout.

Validation of the Maslach Burnout Inventory-General Survey on a representative sample of Dominican teachers: normative data

Burnout is characterized by emotional exhaustion, depersonalization and lack of personal accomplishment (Bakke, Demerouti, & Sanz-Vergel, 2014). Several instruments for its measurement exist, but the most widely used scale for measuring its dimensions, by far, is the Maslach Burnout Inventory (MBI) in its different versions. Among the available versions of the scale, the MBI-General Survey was developed to measure three dimensions of burnout (cynicism, personal accomplishment, and emotional exhaustion) regardless of the type of work. The aim of this research is to offer evidence on the psychometric properties of the MBI-GS for its use in the Dominican Republic and other Caribbean Spanish-speaking countries, using representative sample of Dominican teachers. The factorial validity was studied through confirmatory factor analysis. Several competing models were proved in order to test the dimensionality of the scale. The confirmatory analyses shown that the original three-factor structure had a superior fit, but item eleven was removed in order to get an excellent fit χ2(87) = 211.19, p < .001, CFI = .98, RMSEA = .038 90% CI [.032-.045]. Regarding internal consistency, the CRI's are well above the cut-off criteria of .7 (CRI’s ranged from .74 to .86). Concerning criterionrelated validity, the three factors were correlated in the expected direction. Professional efficacy, a dimension of burnout measured in the opposite direction, was positively correlated with the three factors of work engagement, also as expected. This version was found to be a psychometrically sound measure of the three core dimensions of burnout (AU)

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Hipoacusia en niños con conducto auditivo interno agrandado / Hearing loss and enlarged internal auditory canal in children

Introducción: Entre las anomalías del hueso temporal que pueden encontrarse en el estudio etiológico de la hipoacusia neurosensorial (HANS) infantil mediante pruebas de imagen, las relacionadas con el conducto auditivo interno (CAI) se hallan entre las menos frecuentes. De ellas, la más prevalente y relacionada con HANS es el CAI estenótico por su asociación a deficiencias del nervio coclear. Menos frecuente y menos concomitante con HANS es el hallazgo de un CAI agrandado (> 8 mm). Métodos: Estudio retrospectivo y descriptivo de las asociaciones clínicas, estudios de imagen, patrones audiológicos y opciones de tratamiento de 9 niños diagnosticados de hipoacusia en el periodo 1999-2012 con un CAI agrandado. Resultados: Se describen 2 grupos de pacientes. El primero, sin asociación con displasias cocleovestibulares: 2 pacientes con HANS sin otras alteraciones de hueso temporal o sistémicas, una hipoacusia mixta bilateral con cromosomopatía por deleción 18q, una hipoacusia genética DFN 3 ligada a X, una hipoacusia unilateral en neurofibromatosis tipo 2 con neurinoma del acústico bilateral, y una hipoacusia unilateral con déficit de nervio coclear unilateral; y un segundo grupo con asociación a displasias cocleovestibulares: una hipoacusia mixta bilateral moderada en síndrome branquio-oto-renal, una HANS profunda unilateral con meningitis recurrentes, y una HANS bilateral profunda con hipotiroidismo congénito. Conclusiones: La presencia de un CAI agrandado en niños puede encontrarse en diferentes contextos clínicos y audiológicos, con relevancias que pueden variar desde situaciones con riesgo vital como en meningitis recurrentes, hasta hipoacusias aisladas sin otras asociaciones (AU)

Introduction: Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (> 8 mm). Methods: Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Results: Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. Conclusions: The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations (AU)

Tumor de células granulares de localización laríngea: descripción de 2 casos pediátricos / Granular cell tumor of laryngeal location: description of 2 pediatric cases

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Hearing loss and enlarged internal auditory canal in children.

INTRODUCTION: Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). METHODS: Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. RESULTS: Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. CONCLUSIONS: The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations.

Hipoacusia y problemas de vía aérea en niños con mucopolisacaridosis / Hearing loss and airway problems in children with mucopolysaccharidoses

Introducción: Las mucopolisacaridosis (MPS) son un grupo de enfermedades sistémicas caracterizadas por un déficit genético de enzimas lisosomales que ocasiona el acúmulo de glucosaminoglucanos en diferentes tejidos. El inicio de los síntomas suele presentarse en la primera infancia, ocasionando en el área ORL problemas de otitis media, hipoacusia y obstrucción de vía aérea. Objetivo: Descripción de los hallazgos audiológicos y la patología de vía aérea encontrados en 9 niños diagnosticados de MPS. Métodos: Revisión retrospectiva de los hallazgos clínicos, audiológicos y procedimientos exploratorios y terapéuticos ORL realizados a 9 niños diagnosticados de MPS en un centro público pediátrico terciario en el período 2007-2010. Resultados: Los subtipos encontrados fueron 4 MPS I, 3 MPS II, 1 MPS-IV y 1 MPS VI. Todos los pacientes presentaban otitis seromucosa. Un caso desarrolló hipoacusia neurosensorial bilateral leve, otro fue diagnosticado de hipoacusia mixta. El patrón auditivo restante fue hipoacusia conductiva bilateral moderada. Cuatro pacientes presentaban SAHOS (síndrome de apnea/hipopnea del sueño) secundario a hiperplasia del anillo linfático de Waldeyer, en uno de ellos no pudo realizarse cirugía por compresión medular cervical por depósitos de mucopolisacáridos. En dos de los casos el SAHOS recidivó. Conclusiones: Los niños con MPS presentan mayor riesgo para desarrollar hipoacusia neurosensorial. El SAHOS se encuentra en mayor proporción que en la población general infantil, pudiendo recidivar más frecuentemente tras cirugía. Asimismo pueden ser pacientes de riesgo en el manejo de la vía aérea (AU)

Introduction: Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that cause the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. Objective: Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. Methods: A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007-2010. Results: Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. Conclusions: Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management (AU)

[Hearing loss and airway problems in children with mucopolysaccharidoses]. / Hipoacusia y problemas de vía aérea en niños con mucopolisacaridosis.

INTRODUCTION: Mucopolysaccharidoses (MPS) are a group of systemic diseases characterised by a genetic deficiency of lysosomal enzymes that causes the accumulation of glycosaminoglycans in different tissues. The onset of symptoms usually occurs in early childhood, causing problems of otitis media, hearing loss and airway obstruction in the ENT area. OBJECTIVE: Describing the audiological findings and airway pathology found in 9 children diagnosed as having MPS. METHODS: A retrospective review was performed of the clinical and audiological findings, exploratory results and therapeutic ENT procedures for 9 children diagnosed with MPS in an ENT service at a tertiary paediatric public centre in the period 2007-2010. RESULTS: Subtypes found were 4 MPS type I, 2 moderate MPS type II, 1 severe MPS type II, 1 MPS type IV and 1 MPS type VI. All patients presented chronic middle ear effusions. A child developed mild bilateral sensorineural hearing loss; another case was diagnosed as mixed hearing loss. The remaining auditory pattern was moderate bilateral conductive hearing loss. Four patients showed secondary obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to Waldeyer ring hyperplasia; surgery could not be performed on one of them because of cervical spinal cord compression from mucopolysaccharide deposits. In 2 cases, there was OSAHS relapse. CONCLUSIONS: Children with MPS are at increased risk for developing sensorineural hearing loss. The OSAHS syndrome appears in greater proportion than in the general child population, and recurrences may occur more frequently after surgery. Such children can also be risk patients in airway management.

Hipoacusia en niños con acueducto vestibular dilatado. Estudio de 55 casos / Enlarged vestibular aqueduct syndrome. A review of 55 paediatric patients

Introducción: El acueducto vestibular dilatado (AVD) es la anomalía congénita más frecuentemente encontrada en técnicas de imagen en hipoacusia neurosensorial infantil. Nuestro objetivo es describir las características clínicas y audiológicas de los niños hipoacúsicos con hallazgo de AVD. Métodos: Estudio retrospectivo de 55 niños diagnosticados de AVD en el periodo 2000–2009. Se analizaron las pruebas audiológicas objetivas y/o subjetivas estándar realizadas según la edad de desarrollo de los niños. Se describen los hallazgos y concomitancias clínicas y audiológicas. Resultados: Treinta y siete pacientes (67,27%) presentaban AVD bilateral y 18 (32,72%) unilateral. La hipoacusia era bilateral en 46 (83,63%) casos y unilateral en 9 (16,36%). La media de edad resultó 3,78 años. Presentaron hipocausa neurosensorial 53 (96,36%) casos (28 bilaterales y profundas), y 2 (3,63%) casos hipoacusia mixta. Tres casos fueron progresivos, 2 fluctuantes, 2 asimétricas y 2 presentaron síntomas vestibulares. Se evidenciaron otras anomalías radiológicas asociadas (6 hipoplasias cocleares, 2 conductos auditivos internos agrandados, 1 vestíbulo dilatado y 1 conducto semicircular horizontal hipoplásico), y 6 síndromes clínicos concomitantes (2 Down, 1 Jacobsen, 1 Pendred, 1 Waardenburg, 1 branquio-oto-renal). Un caso resultó positivo a la mutación GJB2. Se encontró historia familiar de hipoacusia en 12 (21,8%) casos. Conclusión: La presentación clínica de la hipoacusia infantil en el AVD se caracteriza por su variabilidad. Debe incluirse en el diagnóstico diferencial de la hipoacusia mixta. La asociación familiar y sindrómica del AVD deben considerarse en el estudio diagnóstico. Es necesario conocer la historia natural de la enfermedad con fines de información pronóstica a los padres (AU)

Introduction: Enlarged vestibular aqueduct (EVA) is the commonest congenital anomaly found with imaging techniques in paediatric sensorineural hearing loss (SNHL). Our aim was to describe clinical and audiological findings in paediatric hearing loss associated to EVA. Methods: Retrospective review of 55 children with imaging-technique EVA findings from 2000 to 2009. Subjective and/or objective audiological tests were analysed and audiological findings related to clinical features were described. Results: Thirty-seven patients (67.27%) showed bilateral EVA and 18 (32.72%) were unilateral. Hearing loss was bilateral in 46 (83.63%) patients and unilateral in 9 (16.36%). Mean age at diagnosis was 3.78 years. Fifty-three (96.36%) children showed SNHL (28 bilateral and profound), while 2 (3.63%) patients had mixed hearing loss. There were 3 cases of hearing loss progression, 2 fluctuations, 2 of them were asymmetric and 2 patients suffered from vestibular symptoms. Concomitant image findings were 6 cochlear hypoplasia, 2 enlarged internal auditory canals, 1 enlarged vestibule and 1 hypoplastic lateral semicircular canal. Six clinical syndromes were found (2 cases of Down's, and 1 each of Jacobsen, Pendred, Waardenburg and branchio-oto-renal). One child was positive for GJB2 mutation. Familial hearing loss was demonstrated on 12 (21.8%) cases. Conclusion: The clinical picture of hearing loss associated to EVA is characterised by great variability. It should be included in the differential diagnosis of unexplained mixed hearing loss. Familial and syndromic findings have to be taken into consideration in the diagnostic evaluations of such patients. Knowledge about the natural history of this illness is needed so as to give parents prognostic information (AU)

Observaciones clínicas sobre el uso de propranolol en hemangiomas de vía aérea infantil / Clinical observations on propranolol use for paediatric airway hemangiomas

El tratamiento del hemangioma subglótico infantil tradicionalmente se ha caracterizado por presentar multitud de técnicas terapéuticas, tanto médicas como quirúrgicas, sin existir consenso sobre cuál es la mejor opción y con la desventaja de potenciales efectos adversos muy severos. Trabajos recientes informan de mejorías sintomáticas significativas con el uso de propranolol en estos pacientes concluyendo que puede ser una alternativa eficaz a los tratamientos clásicos sin el inconveniente de tan graves efectos secundarios. Se presenta la experiencia de 6 casos de niños con hemangiomas sintomáticos de vía aérea tratados con propranolol. Todos ellos permanecieron asintomáticos respiratoriamente sin necesidad de tratamientos adicionales (AU)

Treatment of subglottic hemangiomas in children has traditionally been characterized as a challenging situation with multiple therapeutic options without consensus as to which one is the best and with risks of severe side effects. Recent reports on the experience of propranolol use in the treatment of paediatric airway hemangiomas suggest favourable reasons for this use due to the rapid improvement and its lack of severe side effects. In this paper we report the experience with 6 children having symptomatic airway hemangiomas treated with propranolol. All children improved their respiratory symptoms dramatically and did not need additional interventions (AU)

[Enlarged vestibular aqueduct syndrome. A review of 55 paediatric patients]. / Hipoacusia en niños con acueducto vestibular dilatado. Estudio de 55 casos.

INTRODUCTION: Enlarged vestibular aqueduct (EVA) is the commonest congenital anomaly found with imaging techniques in paediatric sensorineural hearing loss (SNHL). Our aim was to describe clinical and audiological findings in paediatric hearing loss associated to EVA. METHODS: Retrospective review of 55 children with imaging-technique EVA findings from 2000 to 2009. Subjective and/or objective audiological tests were analysed and audiological findings related to clinical features were described. RESULTS: Thirty-seven patients (67.27%) showed bilateral EVA and 18 (32.72%) were unilateral. Hearing loss was bilateral in 46 (83.63%) patients and unilateral in 9 (16.36%). Mean age at diagnosis was 3.78 years. Fifty-three (96.36%) children showed SNHL (28 bilateral and profound), while 2 (3.63%) patients had mixed hearing loss. There were 3 cases of hearing loss progression, 2 fluctuations, 2 of them were asymmetric and 2 patients suffered from vestibular symptoms. Concomitant image findings were 6 cochlear hypoplasia, 2 enlarged internal auditory canals, 1 enlarged vestibule and 1 hypoplastic lateral semicircular canal. Six clinical syndromes were found (2 cases of Down's, and 1 each of Jacobsen, Pendred, Waardenburg and branchio-oto-renal). One child was positive for GJB2 mutation. Familial hearing loss was demonstrated on 12 (21.8%) cases. CONCLUSION: The clinical picture of hearing loss associated to EVA is characterised by great variability. It should be included in the differential diagnosis of unexplained mixed hearing loss. Familial and syndromic findings have to be taken into consideration in the diagnostic evaluations of such patients. Knowledge about the natural history of this illness is needed so as to give parents prognostic information.

[Clinical observations on propranolol use for paediatric airway hemangiomas]. / Observaciones clínicas sobre el uso de propranolol en hemangiomas de vía aérea infantil.

Treatment of subglottic hemangiomas in children has traditionally been characterized as a challenging situation with multiple therapeutic options without consensus as to which one is the best and with risks of severe side effects. Recent reports on the experience of propranolol use in the treatment of paediatric airway hemangiomas suggest favourable reasons for this use due to the rapid improvement and its lack of severe side effects. In this paper we report the experience with 6 children having symptomatic airway hemangiomas treated with propranolol. All children improved their respiratory symptoms dramatically and did not need additional interventions.