1例以听力下降为主要表现的Chiari畸形Ⅰ型及其人工耳蜗植入效果分析

Outcome of cochlear implantation in a patient with Chiari malformation typeⅠ who presented with hearing loss

Chiari畸形是以后颅窝解剖结构发育畸形为特点的一组疾病,尤其是涉及大脑与脊髓交界区域,因其导致小脑扁桃体下部疝入枕骨大孔,又称为小脑扁桃体下疝畸形。脑干及后组脑神经相关症状为该病的典型表现,而国内外陆续报道部分患者以感音神经性听力下降(SNHL)为主要症状。

Chiari malformation typeⅠ(CMI)is a disorder characterized by tonsilla cerebelli herniating into an underdeveloped posterior cranial fossa,hearing loss is often covered by more striking neurological symptoms.Hearing loss in this syndrome is not specific in terms of gender side,degree,age of onset,and progression.The hearing improvement after posterior fossa decompression is controversial on the basis of literature,while satisfactory result was obtained after cochlear implantation in the patient reported here,who was diagnosed as CMI with hearing loss as the main symptom.Therefore,after ensuring the integrity of the auditory pathway,cochlear implantation may be considered in CMI patients with bilateral severe or profound without other severe neurological symptoms.