Otto Steven R, Brackmann Derald E, Hitselberger William E, Shannon Robert V, Kuchta Johannes
House Ear Institute, Los Angeles, California 90057, USA.
J Neurosurg. 2002 Jun;96(6):1063-71. doi: 10.3171/jns.2002.96.6.1063.
Neurofibromatosis Type 2 (NF2) has typically resulted in deafness after surgical removal of bilateral vestibular schwannomas (VSs). Cochlear implants are generally ineffective for this kind of deafness because of the loss of continuity in the auditory nerve after tumor removal. The first auditory brainstem implant (ABI) in such a patient was performed in 1979 at the House Ear Institute, and this individual continues to benefit from electrical stimulation of the cochlear nucleus complex. In 1992, an advanced multichannel ABI was developed and a series of patients with NF2 received this implant to study the safety and efficacy of the device.
At the time of first- or second-side VS removal, patients received an eight-electrode array applied to the surface of the cochlear nucleus within the confines of the lateral recess of the fourth ventricle. The device was activated approximately 6 weeks after implantation. and patients were tested every 3 months for the 1st year after the initial stimulation, and annually thereafter. The protocol included a comprehensive battery of psychophysical and speech perception tests.
The multichannel ABI proved to be effective and safe in providing useful auditory sensations in most patients with NF2. The ABI improved patients' ability to communicate compared with the lipreading-only condition, it allowed the detection and recognition of many environmental sounds, and in some cases it provided significant ability to understand speech by using just the sound from the ABI (with no lipreading cues). Its performance in most patients has continued to improve for up to 8 years after implantation.
2型神经纤维瘤病(NF2)患者在手术切除双侧前庭神经鞘瘤(VS)后通常会导致耳聋。由于肿瘤切除后听神经连续性丧失,人工耳蜗植入通常对此类耳聋无效。1979年,在豪斯耳科研究所为一名此类患者进行了首例听觉脑干植入(ABI),该患者至今仍受益于耳蜗核复合体的电刺激。1992年,研发出了一种先进的多通道ABI,一系列NF2患者接受了该植入装置,以研究其安全性和有效性。
在首次或第二次切除VS时,患者在第四脑室外侧隐窝范围内接受一个八电极阵列,将其应用于耳蜗核表面。植入后约6周激活该装置。在首次刺激后的第1年,每3个月对患者进行一次测试,此后每年进行一次测试。该方案包括一系列全面的心理物理学和言语感知测试。
多通道ABI在大多数NF2患者中被证明能有效且安全地提供有用的听觉感受。与仅靠唇读的情况相比,ABI提高了患者的沟通能力,使他们能够检测和识别许多环境声音,在某些情况下,仅通过ABI发出的声音(无唇读线索)就能显著提高理解言语的能力。在大多数患者中,其性能在植入后长达8年的时间里持续改善。