Division of Otolaryngology, Chiba Children's Hospital, Chiba, Japan.
Otol Neurotol. 2013 Apr;34(3):554-8. doi: 10.1097/MAO.0b013e3182804b31.
To clarify the prevalence and clinical characteristics of cochlear nerve deficiency (CND) in patients with congenital bilateral and unilateral hearing loss.
Retrospective case review.
Tertiary referral center.
One hundred fourteen children with bilateral and 56 children with congenital unilateral sensoneural hearing loss.
Review of medical records, audiologic tests, and imaging studies. Imaging studies were evaluated for the presence or absence of abnormalities in the bony cochlear nerve canal (BCNC), internal auditory canal (IAC), and inner ear.
The prevalence of CND, whether unilateral or bilateral, was much higher in the unilateral than in the bilateral hearing loss group: 50% (28/56) versus 5.3% (6/114). Among the 6 children with bilateral hearing loss and CND, 2 had bilateral BCNC stenosis alone, 2 had bilateral BCNC stenosis and unilateral IAC stenosis, 1 had unilateral BCNC stenosis alone, and 1 had unilateral IAC stenosis alone. All 28 children with unilateral hearing loss and CND had BCNC stenosis, whereas 9 (32.1%) also had concurrent IAC stenosis. Three of the 6 children with CND and bilateral hearing loss and 5 of the 28 children with CND and unilateral hearing loss also had other inner ear abnormalities.
Our results suggest differences in the causes and mechanisms of CND in children with bilateral versus unilateral hearing loss.
阐明先天性双侧和单侧听力损失患者中耳蜗神经缺失（CND）的患病率和临床特征。
回顾性病例研究。
三级转诊中心。
114 例双侧和 56 例先天性单侧感音神经性听力损失儿童。
回顾病历、听力测试和影像学研究。评估影像学研究中骨耳蜗神经管（BCNC）、内听道（IAC）和内耳是否存在异常。
单侧听力损失组的 CND 患病率（无论是单侧还是双侧）明显高于双侧听力损失组：50%（28/56）比 5.3%（6/114）。在 6 例双侧听力损失合并 CND 的患儿中，2 例仅双侧 BCNC 狭窄，2 例双侧 BCNC 狭窄合并单侧 IAC 狭窄，1 例单侧 BCNC 狭窄，1 例单侧 IAC 狭窄。28 例单侧听力损失合并 CND 的患儿均有 BCNC 狭窄，而 9 例（32.1%）同时存在 IAC 狭窄。6 例双侧 CND 患儿中有 3 例和 28 例单侧 CND 患儿中有 5 例还存在其他内耳异常。
我们的结果表明双侧与单侧听力损失患儿的 CND 病因和发病机制存在差异。
