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Resumen: 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. Abstract: 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.

The biallelic inheritance of an expanded intronic pentamer (AAGGG)A retrospective descriptive study from an ataxia database comprising 500 patients.The study was performed at the Otorhinolaryngology Department of a hospital in the north of Spain.Specific genetic testing for CANVAS was performed in 13 patients with clinical suspicion of complete or incomplete syndrome. The clinical diagnosis was supported by quantitative vestibular hypofunction, cerebellar atrophy, and abnormal sensory nerve conduction testing.Nine of 13 (69%) patients met clinical diagnostic criteria for definite CANVAS disease. The first manifestation of the syndrome was lower limb dysesthesia in 8 of 13 patients and gait imbalance in 5 of 13. Eleven of 13 (85%) patients were carriers of the biallelic (AAGGG)A genetic cause of CANVAS has recently been discovered. We propose genetic screening for biallelic expansions of the AAGGG pentamer of

Resumen Introduccion La hipoacusia neurosensorial (HNS) es el deficit sensorial mas prevalente en nuestro medio. La secuenciacion genomica de nueva generacion (NGS) permite obtener un diagnostico etiologico en un alto porcentaje de pacientes. Nuestro estudio piloto muestra los resultados de la aplicacion sistematica de la NGS en una Unidad de Hipoacusia Infantil, asi como sus implicaciones en el manejo clinico de los pacientes y sus familiares. Material y metodo Se incluyo a 27 pacientes diagnosticados de HNS entre 2014 y 2017 en los que se descarto una causa ambiental. El test genetico consistio en un panel de genes analizados mediante NGS (panel OTOgenicsTM). Este panel ha sido disenado para incluir genes asociados con hipoacusia neurosensorial o mixta, de inicio precoz o tardio, sindromica y no sindromica, independientemente de su patron de herencia. Resultados Se obtuvo un diagnostico genetico en el 56% (15/27) de los pacientes (62% en el caso de las HNS bilaterales); 5/27 (19%) presentaron variantes patogenicas en el gen GJB2 y el resto variantes patogenicas o probablemente patogenicas en otros genes asociados con HNS aislada (PR2X2, TECTA y STRC), con HNS sindromicas (CHD7, GATA3, COL4A5, MITF y SOX10) o con HNS sindromicas y no sindromicas (BSND, ACTG1 y CDH23). Discusion El diagnostico etiologico de la HNS supone un desafio en la practica clinica. Nuestra serie demuestra que es posible implementar el diagnostico genetico en la rutina asistencial y que esta informacion tiene implicaciones pronosticas y terapeuticas.

Introduction Sensorineural hearing loss (SNL) is the most prevalent sensory deficit in our environment. The next generation genomic sequencing (NGS) allows to obtain an etiological diagnosis in a high percentage of patients. Our pilot study shows the results of the systematic application of NGS in a Childhood Hearing Loss Unit, as well as its implications in the clinical management of patients and their families. Material and method We included 27 patients diagnosed with SNH between 2014 and 2017, in which an environmental cause was ruled out. The genetic test consisted of a panel of genes analyzed by NGS (OTOgenics™ panel). This panel has been designed to include genes associated with sensorineural or mixed hearing loss, early onset or late, syndromic and non-syndromic, regardless of their inheritance pattern. Results A genetic diagnosis was obtained in 56% (15/27) of the patients (62% in the case of bilateral HNS). 5/27 (19%) presented pathogenic variants in the GJB2 gene and the rest variants pathogenic and/or probably pathogenic in other genes associated with isolated HNS (PR2 × 2, TECTA and STRC), with syndromic HNS (CHD7, GATA3, COL4A5, MITF and SOX10) or with syndromic and non-syndromic HNS (BSND, ACTG1 and CDH23). Discussion The etiological diagnosis of SNL is a challenge in clinical practice. Our series demonstrates that it is possible to implement genetic diagnosis in the daily routine and that this information has prognostic and therapeutic implications.

Resumen La paralisis de Bell es la forma mas comun de paresia o paralisis facial. Sin embargo, no todos los pacientes con paralisis facial tienen una paralisis de Bell. Otras causas frecuentes incluyen las secuelas del tratamiento del neurinoma del viii par, el cancer de cabeza y cuello, la iatrogenia, el zoster otico y los traumatismos. El abordaje de cada una de estas situaciones es totalmente diferente. El objetivo de esta guia es servir de consejo para el tratamiento y el seguimiento de los pacientes con paralisis facial. Nuestra idea es que la guia sea practica, haciendo hincapie en recomendaciones efectivas y utiles en el manejo diario de los pacientes. Esta guia ha sido promovida por la Sociedad Espanola de ORL y escrita por medicos con experiencia en la enfermedad del nervio facial, incluyendo al menos un especialista de cada comunidad autonoma. Redactada en un formato de preguntas y respuestas, incluye 56 cuestiones relevantes relacionadas con el nervio facial.

Bell's palsy is the most common diagnosis associated with facial nerve weakness or paralysis. However, not all patients with facial paresis/paralysis have Bell's palsy. Other common causes include treatment of vestibular schwannoma, head and neck tumours, iatrogenic injuries, Herpes zoster, or trauma. The approach to each of these conditions varies widely. The purpose of this guideline is to provide clinicians with guidance on the treatment and monitoring of patients with different causes of facial paralysis. We intend to draft a practical guideline, focusing on operationalised recommendations deemed to be useful in the daily management of patients. This guideline was promoted by the Spanish Society of Otolaryngology and developed by a group of physicians with an interest in facial nerve disorders, including at least one physician from each Autonomous Community. In a question and answer format, it includes 56 relevant topics related to the facial nerve.

Resumen Antecedentes y objetivo El riesgo de meningitis bacteriana aumenta en los pacientes con implante coclear. Por ello, se indica la vacunacion antineumococica, 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 metodos 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 polisacarida de 23 serotipos. Se dividio la muestra en 2 grupos por edad ( Resultados De los 153 pacientes estudiados (28,01% 0-13 anos y 71,9% ≥ 14), solo 2 (5,71%) tuvieron un 100% de adherencia al calendario vacunal, mientras que el 65,71% registro un cumplimiento del 50% o menor. Globalmente, la cobertura de vacunacion frente a la pauta secuencial de neumococo fue del 48,57%. La poblacion pediatrica supero 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 anos apenas supero el 50%. La cobertura frente a gripe estacional fue inferior al 40%. Se obtuvo una correlacion inversa entre la edad y el cumplimiento, aunque no estadisticamente significativa. Conclusiones Las coberturas de vacunacion en los pacientes con implante coclear evaluados son mas bajas de lo esperado. Se propone la colaboracion estrecha entre los servicios de Otorrinolaringologia y las Unidades de Vacunas como principal estrategia para la mejora.

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.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.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.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.

Resumen Introduccion y objetivos La inadecuada retroalimentacion auditiva en los ninos con hipoacusia prelocutiva altera la articulacion de consonantes y vocales. El proposito de esta investigacion es comparar la produccion de vocales en ninos hipoacusicos hispanohablantes con implante coclear o adaptacion audioprotesica con ninos normooyentes por medio del analisis acustico de las frecuencias formanticas y el espacio vocal. Metodo Un total de 56 ninos con hipoacusia prelocutiva (25 con implante coclear y 31 con audifonos) y 47 ninos normooyentes participaron en el estudio. Los 2 primeros formantes (F1 y F2) de las 5 vocales del idioma espanol se midieron utilizando el programa Praat. Para analizar las diferencias entre los 3 grupos se aplico el metodo ANOVA de analisis de la varianza y el test de Scheffe. Tambien se calculo el area del espacio vocal. Resultados El valor medio del F1 en todas las vocales no mostro diferencias significativas en los 3 grupos de ninos. Para las vocales /i/, /o/ y /u/ el valor medio del F2 fue significativamente diferente entre los 2 grupos de ninos hipoacusicos y el grupo de ninos normooyentes. Conclusion Ambos grupos de ninos con hipoacusia prelocutiva tienden a presentar sutiles desviaciones en la articulacion de las vocales que pueden ser analizadas mediante un programa de analisis acustico.

Introduction Contralateral sensorineural hearing loss (CSNHL) after vestibular schwannoma (VS) is a severe complication, especially in those cases in which hearing preservation in the operated side was not possible. There are several theories that attempt to explain this issue, but there is no established guideline of treatment. Material and methods We report 4 patients treated in our institution who developed a severe CSNHL after surgery. Results Of the 185 cases of VS treated with surgery, 4 patients (2.2%) developed a CSNHL after VS surgery. After medical treatment, partial recovery of hearing occurred in one patient the other 3 patients presented a well-established severe SNHL. Conclusions Established treatment guidelines do not exist, but the use of high doses of corticosteroids has been recommended and cochlear implant in cases with no recovery and complete hearing loss may be useful.

Cuba implemented policies mandating social distancing on March 11, 2020, which were still in place at the time of this study. During such periods of isolation, people with psychoactive substance-related disorders and other addictions may be tempted to reduce tension, stress, uncertainty and possible distress by increasing the use of substances or practices they have abused. This can mean relapses and setbacks for patients undergoing treatment. A multidisciplinary team of health professionals specializing in addiction at the Center for Academic Development in Drug Addiction, in Havana, Cuba, cares for people with these disorders and followed their evolution during the initial period of COVID-19 social isolation. With the aim of characterizing strategies employed by patients undergoing treatment for substance abuse and addictions, we conducted a qualitative study from April 2020 through May 2020, using a convenience sample of 37 patients (all students) who had been progressing towards recovery from addictive behaviors when face-to-face encounters were suspended due to COVID-19 restrictions. Contact was maintained through information and communication technologies. The research used telepsychology and focused on understanding patient life experiences. Patients were interviewed using a semi-structured survey, which was then transcribed and coded thematically using a grounded-theory approach. We found that patients' ability to cope successfully with challenges presented by COVID-19 were influenced by: 1) the individual's own methods for maintaining self-control (commitment to studies, projects, and work with therapists) that aided them in their goals concerning abstinence; 2) difficulties faced in addressing specific events and situations (doubts, uncertainties, disagreements, isolation and time use); 3) perpetuation and revivification of myths related to substances and addictive activities (exacerbation of supposed benefits of tobacco, alcohol, marijuana, overuse of social networks); and 4) tendencies toward irrationality and lack of emotional control (fear, sadness, anger, constant worry and self-imposed demands). Our findings suggest that despite the potential negative psychological impact of preventive social isolation during the COVID-19 pandemic, individual coping mechanisms developed by these patients, aiming at improved self-control, allowed most to avoid setbacks that could have affected their recovery. Nevertheless, patients faced challenges to their recovery that were compounded by difficulties in specific situations, myths related to substances and addictive activities, and tendencies toward irrationality or lack of emotional control.

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 our 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. Resumen: 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

Objective: To evaluate the efficacy of mandatory cigarette sanitary warnings to persuade smokers. Method: The Tobacco Consumption Questionnaire (TCQ) was applied to a sample of 205 smokers from La Habana and Santiago de Cuba. Results: A 100 % of the sample classifies as high risk smokers according to the TCQ. Most of them do not read the cigarette sanitary warnings. These warnings have never prevented them from smoking. Conclusion: Mandatory cigarette sanitary warnings have no effect whatsoever on smokers. Some possible reasons might be historical and socio-cultural factors, insufficient contributions of Cuban laws, and other inconsistent public policies.