de Oliveira Adriana Kosma Pires, Hamerschmidt Rogerio, Mocelin Marcos, Rezende Rodrigo K
Otolaryngological Doctor at Hospital of Parana of Otolaryngology.
Professor of Otolaryngology, Federal University of Parana. Otolaryngologist Doctor at, Hospital of Parana of Otolaryngology.
Int Arch Otorhinolaryngol. 2012 Jul;16(3):406-9. doi: 10.7162/S1809-97772012000300018.
Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment.
To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation.
CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.
丹迪-沃克综合征是一种中枢神经系统先天性异常疾病，其特征为小脑中部结构发育不全、后颅窝囊肿性扩张并与第四脑室相通，以及横窦、小脑幕和小脑蚓部上移。临床体征包括枕部突出、头颅逐渐增大、囟门前凸、视乳头水肿、共济失调、步态障碍、眼球震颤和智力障碍。
描述一名13岁女性患者的病例，该患者诊断为此综合征且伴有双侧听力损失，在局部麻醉和镇静下接受了人工耳蜗植入手术。
CGS，13岁女性，因“丹迪-沃克综合征”被转诊至巴拉那州耳鼻喉研究所耳鼻喉科，以评估双侧听力损失，使用助听器无效。最终评论：人工耳蜗植入领域正在迅速发展。我们认为，丹迪-沃克综合征的存在不能被视为人工耳蜗植入手术的禁忌症，并且没有因神经系统疾病导致的手术并发症，对该患者的效果非常好，其辨别能力出色。该患者对唇读的需求减少，语音质量有所改善。
