Absceso mastoideo como presentación de colesteatoma congénito en preescolar ¿cuál es el origen? / Mastoid abscess as congenital cholesteatoma presentation in a child what is the origin?

Resumen El colesteatoma congénito es una entidad que puede manifestarse con una amplia variedad de síntomas o ser silente durante largo tiempo y constituir un hallazgo incidental. Una vez diagnosticada es importante valorar su extensión y el compromiso de estructuras adyacentes, para lograr una adecuada planificación quirúrgica, eliminando la enfermedad y manteniendo la mejor funcionalidad posible. Se presenta un caso de colesteatoma congénito infantil.

Abstract Congenital cholesteatoma is an entity that can manifest with a wide variety of symptoms or be silent for a long time and constitute an incidental finding. Once diagnosed, it is important to assess the extension to apply the most efficient treatment, eliminating the disease and providing functionality if possible. A case of congenital cholesteatoma in a child is presented.

[Prevalence of auditory neuropathy: prospective study in a tertiary-care center]. / Prevalencia de la neuropatía auditiva: estudio prospectivo en un hospital de tercer nivel.

OBJECTIVE: The primary goal of this study is to determine the frequency of onset of this disorder in our infant population. Its clinical importance is due, among other reasons, to the fact that idiopathic cases constitute false negatives in the auditory screening programmes based on the performance of otoacoustic emissions to all newborn children and brainstem auditory evoked response only to those presenting these risk factors, for which reason another goal is to establish the prevalence of these pure cases and the diagnostic delay they cause. PATIENTS AND METHOD: Prospective study of all newborn children at the Virgen de la Arrixaca Mother and Child Hospital (Murcia, Spain) in the period between June 1, 2000 and June 30, 2006. RESULTS: Our screening programme, with a coverage of 95.68 %, detected 114 patients with unilateral or bilateral sensorineural hearing loss, with 6 presenting hearing loss attributable to auditory neuropathy. CONCLUSIONS: The estimated prevalence of auditory neuropathy in our infant population turned out to be 1406 for every 10 000 children, ie 5.26 % of all sensorineural hearing loss diagnosed. Another significant finding was the delay in diagnosis, since the mean age at the moment of diagnosis was of 11.5 months, mainly due to idiopathic cases not detected during neonatal screening. Two clear conclusions can be inferred from these data: a) auditory neuropathy does not constitute an extraordinarily rare disorder, and b) idiopathic cases constitute false negatives in the universal auditory screening programmes based on the performance of otoacoustic emissions, which habitually leads to a delay in diagnosis and treatment.

Prevalencia de la neuropatía auditiva: estudio prospectivo en un hospital de tercer nivel / Prevalence of auditory neuropathy: prospective study in a tertiary-care center

Objetivo: El objetivo principal de este estudio es determinar la frecuencia de aparición de este trastorno en nuestra población infantil. Su importancia clínica se debe, entre otros motivos, a que sus casos idiopáticos son falsos negativos de los programas de cribado auditivo basados en la realización de otoemisiones acústicas a todos los recién nacidos y potenciales evocados auditivos de tronco cerebral sólo de los que presentan dichos factores de riesgo, por lo que otro objetivo es la prevalencia de estos casos puros y el retraso diagnóstico que ocasionan. Pacientes y método: Estudio prospectivo de todos los recién nacidos del Hospital Materno-Infantil Virgen de la Arrixaca (Murcia) en el período comprendido entre el 1 de junio del 2000 y el 30 de junio del 2006. Resultados: Nuestro programa de cribado, con un índice de cobertura del 95,68 %, detectó a 114 niños con hipoacusia neurosensorial unilateral o bilateral, de los que 6 presentaron una hipoacusia neurosensorial atribuible a la neuropatía auditiva. Conclusiones: La prevalencia estimada de la neuropatía auditiva en nuestra población infantil resultó ser de 1,406 cada 10.000 niños, es decir, un 5,26 % de todas las hipoacusias neurosensoriales diagnosticadas. Otro dato relevante es el retraso diagnóstico, ya que la media de edad al momento del diagnóstico fue de 11,5 meses, fundamentalmente por los casos idiopáticos que pasaron el cribado neonatal. De estos datos podemos deducir 2 claras conclusiones: a) la neuropatía auditiva no es un trastorno extraordinariamente raro, y b) los casos idiopáticos son falsos negativos de los programas de cribado auditivo universal basados en la realización de otoemisiones acústicas, lo que habitualmente conduce a un retraso diagnóstico y, por lo tanto, terapéutico

Objective: The primary goal of this study is to determine the frequency of onset of this disorder in our infant population. Its clinical importance is due, among other reasons, to the fact that idiopathic cases constitute false negatives in the auditory screening programmes based on the performance of otoacoustic emissions to all newborn children and brainstem auditory evoked response only to those presenting these risk factors, for which reason another goal is to establish the prevalence of these pure cases and the diagnostic delay they cause. Patients and method: Prospective study of all newborn children at the Virgen de la Arrixaca Mother and Child Hospital (Murcia, Spain) in the period between June 1, 2000 and June 30, 2006. Results: Our screening programme, with a coverage of 95.68 %, detected 114 patients with unilateral or bilateral sensorineural hearing loss, with 6 presenting hearing loss attributable to auditory neuropathy. Conclusions: The estimated prevalence of auditory neuropathy in our infant population turned out to be 1406 for every 10 000 children, ie 5.26 % of all sensorineural hearing loss diagnosed. Another significant finding was the delay in diagnosis, since the mean age at the moment of diagnosis was of 11.5 months, mainly due to idiopathic cases not detected during neonatal screening. Two clear conclusions can be inferred from these data: a) auditory neuropathy does not constitute an extraordinarily rare disorder, and b) idiopathic cases constitute false negatives in the universal auditory screening programmes based on the performance of otoacoustic emissions, which habitually leads to a delay in diagnosis and treatment