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.

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.