[Otorhinolaryngo logical manifestations in patients with Down syndrome]. / Manifestaciones otorrinolaringológicas en el síndrome de Down.

OBJECTIVE: The objective [corrected] of our study was to assess the most frequent otorhinolaryngological manifestations in patients with Down syndrome, and to propose diagnostic and management guidelines to improve their quality of life. METHODS: Patients with Down's syndrome referred to the ENT Department of two Spanish Hospitals during a 4-year period were retrospectively reviewed. Data of the following variables were collected: main symptoms, diagnosis, comorbidities, surgical procedures, and complications. RESULTS: Thirty patients with Down's syndrome were included in our study. The most frequent reasons for referral were hearing loss and newborns from the Hearing Impairment Screening Program. Otitis media with effusion, adenoid hypertrophy and obstructive sleep apnea were the most common diagnosis. Five patients underwent head and neck surgical procedures without complications. CONCLUSIONS: Hearing loss secondary to chronic otitis media with effusion and upper airway obstruction are frequent pathologies in patients with Down syndrome.

Manifestaciones otorrinolaringológicas en el síndrome de Down / Otorhinolaryngological manifestations in patients with Down syndrome

Introducción: El objetivo de nuestro estudio fue conocer las manifestaciones otorrinolaringológicas más frecuentes en pacientes con síndrome de Down y proponer un protocolo de manejo de las mismas para que tengan el menor impacto en su calidad de vida. Métodos: Se realizó un estudio retrospectivo de los pacientes con síndrome de Down vistos en los Servicios de ORL de dos centros hospitalarios durante 4 años. Las siguientes variables fueron recogidas de la historia clínica: motivo de consulta, diagnóstico, comorbilidad, intervenciones quirúrgicas realizadas y sus complicaciones. Resultados: Treinta pacientes con síndrome de Down fueron incluidos en nuestro estudio. La mayor parte consultó por problemas auditivos o procedente del Programa de Detección de Hipoacusia Neonatal de Cantabria. El diagnóstico más frecuente fueron la otitis serosa, la hipertrofia adenoidea y el síndrome de apnea obstructiva del sueño. Cinco pacientes fueron sometidos a procedimientos quirúrgicos de cabeza y cuello sin presentar complicaciones. Conclusiones: Los pacientes con síndrome de Down presentan con frecuencia manifestaciones otorrinolaringológicas, sobre todo hipoacusia secundaria a patología de oído medio y obstrucción de vías aéreas superiores

Objective: The objetive of our study was to assess the most frequent otorhinolaryngological manifestations in patients with Down syndrome, and to propose diagnostic and management guidelines to improve their quality of life. Methods: Patients with Down’s syndrome referred to the ENT Department of two spanish Hospitals during a 4-year period were retrospectively reviewed. Data of the following variables were collected: main symptoms, diagnosis, comorbidities, surgical procedures, and complications. Results: Thirty patients with Down’s syndrome were included in our study. The most frequent reasons for referral were hearing loss and newborns from the Hearing Impairment Screening Program. Otitis media with effusion, adenoid hypertrophy and obstructive sleep apnea were the most common diagnosis. Five patients underwent head and neck surgical procedures without complications. Conclusions: Hearing loss secondary to chronic otitis media with effusion and upper airway obstruction are frequent pathologies in patients with Down syndrome

[Etiology of severe/profound, pre/perilingual bilateral hearing loss in Cantabria (Spain)]. / Etiología de la hipoacusia severa/profunda bilateral pre/perilocutiva en Cantabria.

OBJECTIVE: To know the etiology of preiperlingual bilateral hearing loss in children. MATERIALS AND METHODS: All the patients diagnosed with bilateral severe/profound, pre or perilingual hearing loss at Sierrallana and Marqués de Valdecilla Hospitals (Cantabria, Spain) during the last 20 years were included in this study. RESULTS: A hundred patients were diagnosed with bilateral severe/profound pre/perilingual hearing loss. The most frequent etiology was hereditary (49%), followed by severe perinatal hypoxia (11%), ototoxicity (5%), meningitis (3%), hyperbilirubinemia (3%) and rubella (2%). In 21% of cases was not known. CONCLUSIONS: The two most frequent etiologies found in severe/profound hearing loss in children in our area were hereditary and non infectious perinatal problems. Infectious disease were scarce. Will decrease when genetic test were used as clinical basis.

Etiología de la hipoacusia severa/profunda bilateral pre/perilocutiva en Cantabria / Etiology of severe/profound, pre/perilingual bilateral hearing loss in Cantabria (Spain)

Objetivo: Conocer las causas implicadas en el desarrollo de hipoacusia bilateral severa/profunda de inicio en la infancia. Métodos: Se realizó un estudio retrospectivo de todos los pacientes diagnosticados de hipoacusia bilateral severa/profunda pre/perilocutiva en los Servicios de ORL del Hospital Sierrallana (Torrelavega) y Marqués de Valdecilla (Santander) en la Comunidad de Cantabria durante los últimos 20 años. Resultados: Cien pacientes fueron diagnosticados de hipoacusia severa/profunda bilateral pre/perilocutiva durante dicho periodo. La etiología más frecuentemente encontrada fue hereditaria (49%),seguida de la hipoxia severa perinatal (11%), ototoxicidad (5%),meningitis (3%), hiperbilirrubinemia (3%) y rubéola (2%). En un21% de los casos no se llegó a determinar la etiología de la misma. Conclusiones: Actualmente las causas más frecuente de hipoacusia severa/profunda en la infancia en nuestro medio son la hereditaria y los trastornos perinatales. Es infrecuente la secundaria a problemas infecciosos. Es probable que con la generalización en la realización de estudios genéticos el número de casos de etiología desconocida disminuya de forma importante en el futuro (AU)

OBJECTIVE: To know the etiology of preiperlingual bilateral hearing loss in children. MATERIALS AND METHODS: All the patients diagnosed with bilateral severe/profound, pre or perilingual hearing loss at Sierrallana and Marqués de Valdecilla Hospitals (Cantabria, Spain) during the last 20 years were included in this study. RESULTS: A hundred patients were diagnosed with bilateral severe/profound pre/perilingual hearing loss. The most frequent etiology was hereditary (49%), followed by severe perinatal hypoxia (11%), ototoxicity (5%), meningitis (3%), hyperbilirubinemia (3%) and rubella (2%). In 21% of cases was not known. CONCLUSIONS: The two most frequent etiologies found in severe/profound hearing loss in children in our area were hereditary and non infectious perinatal problems. Infectious disease were scarce. Will decrease when genetic test were used as clinical basis (AU)