Department of OtolaryngologyYHead and Neck Surgery, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA.
Otol Neurotol. 2012 Dec;33(9):1502-6. doi: 10.1097/MAO.0b013e31826bec1e.
Patients with auditory neuropathy spectrum disorder (ANSD) exhibit altered neural synchrony in response to auditory stimuli. It has been hypothesized that a slower rate of electrical stimulation in programming strategies for cochlear implant (CI) users with ANSD may enhance development of neural synchrony and speech perception abilities.
Retrospective case series.
Tertiary otologic practice.
Twenty-two patients with ANSD underwent CI. Patients with complete postoperative audiometric data and at least 2 years of follow-up were included in further analysis.
Thirteen patients patients met inclusion criteria. Five "poorly performing" CI recipients with ANSD who had not developed closed-set speech perception abilities despite at least 2 years of implant use underwent implant programming to lower the neural stimulation rate.
Speech perception abilities over time using parent questionnaire, closed-set testing, and open-set measures.
A high incidence of comorbid conditions was present in the poor performers, including cognitive delay (n = 2), motor delay (n = 3), and autism spectrum disorder (n = 1). The median time to rate slowing in 5 poor performers was 29 months after implant activation. Three of 5 patients achieved closed-set speech perception scores higher than 60% after 6 to 16 months of implant use at the slower rates. At last follow-up (median, 42 mo), no poor performer had yet achieved open-set speech perception abilities. Of all CI recipients with ANSD included in analysis, open-set speech perception abilities developed in 46% (6/13).
In CI recipients with ANSD who demonstrate limited auditory skills development despite prolonged implant use, lowering the stimulation rate may facilitate acquisition of closed-set speech perception abilities. Further efforts on the study of programming parameters in ANSD patients with CIs are necessary to maximize auditory development in this patient population.
听觉神经病变谱系障碍（ANSD）患者对听觉刺激表现出神经同步性改变。有人假设，对 ANSD 患者的人工耳蜗（CI）编程策略采用较慢的电刺激率，可能会增强神经同步性的发展和言语感知能力。
回顾性病例系列研究。
三级耳科诊所。
22 名 ANSD 患者接受了 CI。在进一步分析中，仅纳入具有完整术后听力数据且随访时间至少 2 年的患者。
13 名患者符合纳入标准。5 名“表现不佳”的 ANSD CI 接受者尽管至少使用了 2 年植入物，但仍未发展出闭合集言语感知能力，对他们进行了植入物编程以降低神经刺激率。
使用家长问卷、闭合集测试和开放集测量，随时间推移的言语感知能力。
表现不佳者存在较高的合并症发生率，包括认知延迟（n=2）、运动延迟（n=3）和自闭症谱系障碍（n=1）。在 5 名表现不佳者中，有 29 个月的中位时间从植入物激活后开始降低刺激率。在以较慢的速度使用植入物 6 至 16 个月后，有 3 名患者中的 3 名患者达到了闭合集言语感知分数高于 60%。在最后一次随访（中位数，42 mo）时，仍未有表现不佳者获得开放集言语感知能力。在所有纳入分析的 ANSD CI 接受者中，46%（6/13）的人发展出了开放集言语感知能力。
在经过长时间植入物使用后，表现出有限的听觉技能发展的 ANSD CI 接受者中，降低刺激率可能有助于获得闭合集言语感知能力。需要进一步研究 ANSD 患者的 CI 编程参数，以最大限度地促进该患者群体的听觉发育。
