Colletti V, Fiorino F, Sacchetto L, Miorelli V, Carner M
ENT Department, Clinica ORL, University of Verona, Ospedale Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, I-37134 Verona, Italy.
Int J Pediatr Otorhinolaryngol. 2001 Aug 20;60(2):99-111. doi: 10.1016/s0165-5876(01)00465-7.
Patients with aplasia and hypoplasia of the cochlear nerve have no chance of having their hearing restored by stimulating the periphery of the auditory system using the traditional cochlear implant. A possible approach to auditory rehabilitation may be direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant (ABI). Recently, two children, aged 4 and 3 years, respectively, with bilateral severe cochlear malformations and cochlear nerve aplasia received an ABI. The present paper reports the technique and the preliminary results of this experience. The classic retrosigmoid approach was used. The correct position of the electrodes was estimated with the aid of EABRs and neural response telemetry (NRT). No postoperative complications were observed. High-resolution CT scans with a bone algorithm reconstruction technique were taken postoperatively to evaluate electrode placement before discharge. The ABI was activated 30 days after implantation in both patients. To date 16 and 13 electrodes, respectively, have been activated in the two children. Three months after activation the first patient had achieved good environmental sound awareness, good speech detection and some speech discrimination. The second child, 1 month after activation, had achieved good environmental sound awareness and moderate speech detection. To the best of our knowledge this is the first report of patients with hypoplasia of the cochlea and aplasia of the cochlear nerve, aged below 5 years and treated with an ABI.
耳蜗神经发育不全和发育不良的患者，通过使用传统人工耳蜗刺激听觉系统外周来恢复听力的可能性不大。一种可能的听觉康复方法是使用听觉脑干植入物（ABI）直接电刺激耳蜗核。最近，两名分别为4岁和3岁的儿童，患有双侧严重耳蜗畸形和耳蜗神经发育不全，接受了ABI植入。本文报告了该手术技术及初步结果。采用经典乙状窦后入路。借助电刺激听性脑干反应（EABR）和神经反应遥测（NRT）来评估电极的正确位置。术后未观察到并发症。术后采用骨算法重建技术进行高分辨率CT扫描，以在出院前评估电极位置。两名患者均在植入后30天开启ABI。迄今为止，两名儿童分别激活了16个和13个电极。开机三个月后，第一名患者已具备良好的环境声音感知、良好的言语察觉能力以及一定的言语辨别能力。第二名儿童在开机1个月后，已具备良好的环境声音感知和中等程度的言语察觉能力。据我们所知，这是关于年龄小于5岁、患有耳蜗发育不全和耳蜗神经发育不全且接受ABI治疗的患者的首例报告。
