ENT, AIIMS, Bhubaneswar, Odisha, India.
ENT, AIIMS, Bhubaneswar, Odisha, India
BMJ Case Rep. 2023 Aug 17;16(8):e253079. doi: 10.1136/bcr-2022-253079.
Cochlear nerve deficiency in cochlear aplasia is a contraindication for cochlear implantation (CI) anticipating poor auditory response. Few authors have reported auditory outcomes even without nerves following CI. This study outlines the audiological outcomes of a patient in early childhood with cochlear aplasia and cochlear nerve deficiency who underwent CI. Auditory and speech-language development were assessed using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale and Ling-6 sound test; at the time of switch-on, after 6 and 12 months of auditory verbal therapy. Significant differences across CAP, SIR and Ling-6 sound detection scores were noted, with the highest mean scores at the 12th-month postimplantation, indicating substantial improvement in auditory and speech-language skills. In cochlear aplasia cases, residual cochlear elements and nerve fibres cannot be ruled out. Our report emphasises the need for research, as this has the potential to impact the existing guidelines for CI candidacy.
耳蜗发育不全是人工耳蜗植入术（CI）的禁忌症，因为这预示着听觉反应不佳。尽管没有神经，也有少数作者报告了听觉结果。本研究概述了一名早期患有耳蜗发育不全和耳蜗神经发育不全的患者接受 CI 后的听力学结果。使用听觉绩效分类（CAP）量表、言语可懂度分级（SIR）量表和 Ling-6 声音测试评估听觉和言语语言发育；在开机时、听觉言语治疗后 6 个月和 12 个月时进行评估。在 CAP、SIR 和 Ling-6 声音检测评分方面均有显著差异，植入后 12 个月的平均得分最高，表明听觉和言语语言技能有了实质性的提高。在耳蜗发育不全的情况下，不能排除残余的耳蜗结构和神经纤维。我们的报告强调了需要进行研究，因为这有可能影响现有的 CI 候选资格指南。
