Auditory Skills following Cochlear Implantation in Children with the Charge Syndrome.

OBJECTIVES: To assess the auditory outcomes and skills of pediatric cochlear implant (CI) users with the CHARGE syndrome. To determine the influence of inner ear malformations on the surgical procedure and speech understanding outcomes in this population. STUDY DESIGN: Observational, retrospective study. MATERIALS AND METHODS: Imaging, auditory testing, intraoperative findings, complications, and postoperative auditory skills and outcomes of pediatric CI users with CHARGE syndrome were recorded. RESULTS: 6 children (8 ears) were included, 5 of whom had prelingual deafness. Their mean age at implantation was 37 months. Six of the 8 ears presented cochlear malformation; the most frequent was hypoplasia type III. Intraoperatively, the transmastoid facial recess approach was used in 5 ears, and abnormalities of facial nerve anatomy were found in 5 ears. All electrode insertions were complete. All children were, to a varying degree, able to detect and identify sound. Verbalization skills were developed by 2 children, 1 of whom used oral language as his primary mode of communication. CONCLUSIONS: Cochlear implantation performed by an experienced surgeon in patients with the CHARGE syndrome is a safe procedure with adequate treatment planning. All children had improved auditory skills although the improvement was variable.

Experiencia clínica en pacientes con síndrome de Meige primario y disfonía espasmódica / Clinical experience with patients with spasmodic dysphonia and primary Meige síndrome

Introducción: El síndrome de Meige (SM) es considerado una distonía craneal segmentaria que afecta a dos o más músculos craneales. Específicamente, asocia blefaroespasmo a otra distonía craneal (oromandibular, cervical o laríngea). El objetivo de este artículo es presentar nuestra experiencia clínica en pacientes con disfonía espasmódica (DE) asociada a SM primario. Material y métodos: Estudio retrospectivo realizado entre mayo de 2010 y junio de 2015. Incluyó 8 pacientes. Las variables recogidas fueron: sexo, edad, distonías asociadas, electromiografía laríngea y tratamiento. Los resultados clínicos objetivados fueron realizados, siempre por el mismo investigador, con la escala GRBAS(i) y el cuestionario VHI-30. Resultados: Cincuenta y seis pacientes fueron tratados de SM por el servicio de Neurología. Ocho pacientes asociaron DE (prevalencia 14%). El 100% de nuestros pacientes tenían DE aductora. La mediana de edad para la aparición de DE fue de 71años. Todos los pacientes fueron tratados con infiltración intralaríngea de toxina botulínica bajo control electromiográfico. Se objetivó mejoría clínica tanto en la escala GRBAS(i) como en los cuestionarios VHI-30. Conclusión: En el estudio de la DE siempre debe tenerse presente su posible asociación con el SM. Desde el punto de vista otorrinolaringológico, el uso conjunto de la escala GRBAS(i) y los cuestionarios VHI-30 es útil, fiable y eficiente como método para evaluar evolución y respuesta a tratamiento. La infiltración laríngea de toxina botulínica bajo control electromiográfico es la alternativa terapéutica que provee mejores resultados clínicos. El manejo de la DE asociada al SM no difiere del de la DE aislada

Introduction: Meige syndrome (MS) is part of the group of segmental cranial dystonias, which affect more than two cranial muscle groups. Specifically, blepharospasm is associated with another cranial dystonia (oromandibular, cervical or laryngeal). The aim of this paper was to report our experience in patients with spasmodic dysphonia (SD) associated with primary MS. Material and methods: A retrospective study involving 8 patients between May 2010 and June 2015. Variables recorded were: age, sex, associated dystonia, electromyographic pattern in laryngeal muscles and treatment given. Outcomes after treatment were assessed using GRBAS(i) scale and VHI-30 questionnaire, always provided by the same examiner. Results: Fifty-six patients with MS were treated in the Neurology Department. Eight patients of 56 were diagnosed with SD (prevalence of 14%). All of our patients had adductor SD. The median age was 71years. All the patients were treated with intralaryngeal botulinum toxin under electromyographic control. Clinically relevant improvements were found after treatment on both the GRBAS(i) scale and the VHI-30 questionnaire. Conclusion: In the study of SD, we should always rule out an association with MS. From the point of view of otorhinolaryngology, the joint use of the GRBAS(i) scale and the VHI-30 questionnaire are useful, reliable and efficient methods for assessing progress and response to treatment. Laryngeal infiltration under electromyographic control with botulinum toxin is the therapeutic alternative that provides better results. The management of SD associated with MS does not differ from isolated SD

Clinical experience with patients with spasmodic dysphonia and primary Meige syndrome. / Experiencia clínica en pacientes con síndrome de Meige primario y disfonía espasmódica.

INTRODUCTION: Meige syndrome (MS) is part of the group of segmental cranial dystonias, which affect more than two cranial muscle groups. Specifically, blepharospasm is associated with another cranial dystonia (oromandibular, cervical or laryngeal). The aim of this paper was to report our experience in patients with spasmodic dysphonia (SD) associated with primary MS. MATERIAL AND METHODS: A retrospective study involving 8 patients between May 2010 and June 2015. Variables recorded were: age, sex, associated dystonia, electromyographic pattern in laryngeal muscles and treatment given. Outcomes after treatment were assessed using GRBAS(i) scale and VHI-30 questionnaire, always provided by the same examiner. RESULTS: Fifty-six patients with MS were treated in the Neurology Department. Eight patients of 56 were diagnosed with SD (prevalence of 14%). All of our patients had adductor SD. The median age was 71years. All the patients were treated with intralaryngeal botulinum toxin under electromyographic control. Clinically relevant improvements were found after treatment on both the GRBAS(i) scale and the VHI-30 questionnaire. CONCLUSION: In the study of SD, we should always rule out an association with MS. From the point of view of otorhinolaryngology, the joint use of the GRBAS(i) scale and the VHI-30 questionnaire are useful, reliable and efficient methods for assessing progress and response to treatment. Laryngeal infiltration under electromyographic control with botulinum toxin is the therapeutic alternative that provides better results. The management of SD associated with MS does not differ from isolated SD.