内耳畸形人工耳蜗植入进展

doi:10.3969/j.issn.1006-7795.2024.06.009

摘要/Abstract

摘要： 内耳畸形（inner ear malformation，IEM）主要指骨迷路的畸形，目前IEM患者多可通过人工耳蜗植入（cochlear implantation，CI）获益，但手术难度和风险较正常耳蜗更大。本文就IEM患者CI手术的技术难点，如面神经畸形、脑脊液耳漏、电极异位等的防治策略，和电极选择、植入侧别选择等手术策略以及术后效果、术后调试的特点做了总结，并利用文献计量学方法总结了IEM患者CI手术的研究热点、展望了未来研究前景。

关键词: 内耳畸形, 人工耳蜗植入, 面神经畸形, 术后效果

Abstract: Inner ear malformation (IEM) primarily refers to malformations of the bony labyrinth. Currently, patients with IEM can benefit from cochlear implantation (CI), but the surgical difficulty and risks are greater compared to those with normal cochleae. This article summarizes the technical challenges of CI surgery in IEM patients, such as the prevention and management strategies for facial nerve malformations, cerebrospinal fluid otorrhea, and electrode malposition, as well as surgical strategies regarding electrode selection and the choice of implantation side. We also discusses the characteristics of postoperative outcomes and programming. Furthermore, using bibliometrics, this article highlights the research hotspots in CI surgery for IEM patients and forecast the future of research prospects.

Key words: inner ear malformation, cochlear implantation, facial nerve deformity, postoperative outcomes

中图分类号:

R764

魏兴梅, 李永新. 内耳畸形人工耳蜗植入进展[J]. 首都医科大学学报, 2024, 45(6): 1001-1007.

Wei Xingmei, Li Yongxin. Advance of cochlear implantation for inner ear malformation[J]. Journal of Capital Medical University, 2024, 45(6): 1001-1007.

参考文献

［1］卜行宽. 介绍《世界听力报告》［J］. 听力学及言语疾病杂志, 2021, 29(2): 123－124.
［2］ Huang B Y, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: practical aspects for neuroradiologists［J］. AJNR Am J Neuroradiol, 2012, 33(2): 211－217.
［3］ Sennarolu L. Cochlear implantation in inner ear malformations--a review article［J］. Cochlear Implants Int, 2010, 11(1): 4－41.
［4］ Mondini C. Minor works of Carlo Mondini: the anatomical section of a boy born deaf［J］. Am J Otol, 1997, 18(3): 288－293.
［5］ Sennarolu L, Bajin M D. Classification and current management of inner ear malformations［J］. Balkan Med J, 2017, 34(5): 397－411.
［6］ Jackler R K, Luxford W M, House W F. Congenital malformations of the inner ear: a classification based on embryogenesis［J］. Laryngoscope, 1987, 97(3 Pt 2 suppl 40): 2－14.
［7］ Cosetti M K, Waltzman S B. Cochlear implants: current status and future potential［J］. Expert Rev Med Devices, 2011, 8(3): 389－401.
［8］ Zeng F G. Celebrating the one millionth cochlear implant［J］. JASA Express Lett, 2022, 2(7): 077201.
［9］ Karatas E. Repair of cerebrospinal fluid leak in cochlear implantation［J］. J Craniofac Surg, 2019, 30(2): 535－538.
［10］ Kamogashira T, Iwasaki S, Kashio A, et al. Prediction of intraoperative CSF gusher and postoperative facial nerve stimulation in patients with cochleovestibular malformations undergoing cochlear implantation surgery［J］. Otol Neurotol, 2017, 38(6): e114－e119.
［11］许庆庆, 翟所强, 韩东一, 等. 伴内耳畸形聋病患者人工耳蜗植入效果的Meta分析［J］. 临床耳鼻咽喉头颈外科杂志, 2015, 29(8): 743－747.
［12］ Gheorghe D C, Zamfir-Chiru-Anton A. Complications in cochlear implant surgery［J］. J Med Life, 2015, 8(3): 329－332.
［13］ Wei X M, Li Y X, Fu Q J, et al. Slotted labyrinthotomy approach with customized electrode for patients with common cavity deformity［J］. Laryngoscope, 2018, 128(2): 468－472.
［14］任媛媛, 赵守琴. 正常及先天性外中耳畸形颞骨内面神经管的HRCT表现［J］. 听力学及言语疾病杂志, 2009, 17(2): 187－189.
［15］ Camerin G R, Passos U L, da Costa S S, et al. Cochlear-Facial dehiscence detected after cochlear implant［J］. Otol Neurotol, 2020, 41(2): e293－e294.
［16］ Schart-Morén N, Larsson S, Rask-Andersen H, et al. Anatomical characteristics of facial nerve and cochlea interaction［J］. Audiol Neurootol, 2017, 22(1): 41－49.
［17］ Romo L V, Curtin H D. Anomalous facial nerve canal with cochlear malformations［J］. AJNR Am J Neuroradiol, 2001, 22(5): 838－844.
［18］ Li Y. Cochlear implantation for cochlear nerve deficiency［M］. Singapore: Springer, 2022.
［19］ Lu S M, Wei X M, Kong Y, et al. Cochlear implantation for rudimentary otocysts: two case reports［J］. Otol Neurotol, 2023, 44(5): e295－e299.
［20］ Allen K P, Bartels L J, Isaacson B. Cochlear implantation requiring a retrofacial approach to the round window［J］. Otol Neurotol, 2015, 36(3): e84－e86.
［21］邹馨悦, 薛书锦, 魏兴梅, 等. 先天性小耳畸形合并面神经畸形面后径路人工耳蜗植入术1例［J］. 临床耳鼻咽喉头颈外科杂志, 2024, 38(5): 416－420.
［22］ Kronenberg J, Baumgartner W, Migirov L, et al. The suprameatal approach: an alternative surgical approach to cochlear implantation［J］. Otol Neurotol, 2004, 25(1): 41－44; discussion 44－45.
［23］ Chen B, Li Y X, Lu S M, et al. Cochlear implant surgery through oval window: a novel approach in a child with facial nerve aberration［J］. Int J Pediatr Otorhinolaryngol, 2020, 135: 110110.
［24］ Yuan Y Y, Song Y S, Chai C M, et al. Intraoperative CT-guided cochlear implantation in congenital ear deformity［J］. Acta Otolaryngol, 2012, 132(9): 951－958.
［25］ Bloom J D, Rizzi M D, Germiller J A. Real-time intraoperative computed tomography to assist cochlear implant placement in the malformed inner ear［J］. Otol Neurotol, 2009, 30(1): 23－26.
［26］赵啸天, 韩德民, 李永新, 等. 内耳畸形患者的人工耳蜗植入术［J］. 中华医学杂志, 2003, 83(2): 103－105.
［27］雷雳, 李永新, 赵守琴, 等. 儿童先天性内耳畸形伴再发性脑膜炎26例临床分析［J］. 中华耳科学杂志, 2020, 18(2): 325－328.
［28］ Suk Y, Lee J H, Lee K S. Surgical outcomes after cochlear implantation in children with incomplete partition type Ⅰ: comparison with deaf children with a normal inner ear structure［J］. Otol Neurotol, 2015, 36(1): e11－e17.
［29］ Kontorinis G, Goetz F, Giourgas A, et al. Radiological diagnosis of incomplete partition type Ⅰ versus type Ⅱ: significance for cochlear implantation［J］. Eur Radiol, 2012, 22(3): 525－532.
［30］ Wang B, Dai W J, Cheng X T, et al. Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases［J］. J Zhejiang Univ Sci B, 2019, 20(2): 156－163.
［31］ Yi H J, Guo H, Ch W, et al. Use of the translabyrinthine approach to repair congenital spontaneous cerebrospinal fluid leakage in five Chinese patients with Mondini dysplasia［J］. Int J Pediatr Otorhinolaryngol, 2013, 77(12): 1965－1968.
［32］ Deng W M, Liu J, Pang F, et al. Diagnosis and management of pediatric cerebrospinal fluid leakage secondary to inner ear malformations: a report of 13 cases［J］. Int J Pediatr Otorhinolaryngol, 2020, 135: 110049.
［33］ Sampath, Chandra, Prasad, et al. Subtotal petrosectomy: surgical technique,indications, outcomes,and comprehensive review of literature［J］. Laryngoscope, 2017, 127(12): 2833－2842.
［34］ Magliulo G, Iannella G, Ciniglio Appiani M, et al. Subtotal petrosectomy and cerebrospinal fluid leakage in unilateral anacusis［J］. J Neurol Surg B Skull Base, 2014, 75(6): 391－396.
［35］ Chen B, Shi Y, Gong Y, et al. Simultaneous repair of cerebrospinal fluid otorrhea and cochlear implantation in two patients with recurrent meningitis and severe inner ear malformation［J］. Int J Pediatr Otorhinolaryngol, 2019, 124: 147－151.
［36］ Hong R, Du Q, Pan Y. New imaging findings of incomplete partition type Ⅲ inner ear malformation and literature review［J］. AJNR Am J Neuroradiol, 2020, 41(6): 1076－1080.
［37］ Corvino V, Apisa P, Malesci R, et al. X-Linked sensorineural hearing loss: a literature review［J］. Curr Genomics, 2018, 19(5): 327－338.
［38］ Nance W E, Setleff R, McLeod A, et al. X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher［J］. Birth Defects Orig Artic Ser, 1971, 07(4): 64－69.
［39］ Wootten C T, Backous D D, Haynes D S. Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery［J］. Laryngoscope, 2006, 116(11): 2055－2059.
［40］ Haroon, Saeed, Harry, et al. Cochlear implantation in X-linked deafness-how to manage the surgical challenges［J］. Cochlear Implants Int, 2016, 17(4): 178－183.
［41］ Tekin A M, Matulic M, Wuyts W, et al. A new pathogenic variant in POU3F4 causing deafness due to an incomplete partition of the cochlea paved the way for innovative surgery［J］. Genes, 2021, 12(5): 613.
［42］ Sennaro L L, Atay G, Bajin M D. A new cochlear implant electrode with a“Cork”-type stopper for inner ear malformations［J］. Auris Nasus Larynx, 2014, 41(4): 331－336.
［43］ Sennarolu L, Bajin M D. Incomplete partition type Ⅲ: A rare and difficult cochlear implant surgical indication［J］. Auris Nasus Larynx, 2018, 45(1): 26－32.
［44］ Xia J, Wang W, Zhang D X. Cochlear implantation in 21 patients with common cavity malformation［J］. Acta Otolaryngol, 2015, 135(5): 459－465.
［45］ Aschendorff A, Maier W, Jaekel K, et al. Radiologically assisted navigation in cochlear implantation for X-linked deafness malformation［J］. Cochlear Implants Int, 2009, 10(S1): 14－18.
［46］ Di Maro F, Carner M, Sacchetto A, et al. Frequency reallocation based on cochlear place frequencies in cochlear implants: a pilot study［J］. Eur Arch Otorhinolaryngol, 2022, 279(10): 4719－4725.
［47］ Pearl M S, Roy A, Limb C J. High-resolution secondary reconstructions with the use of flat panel CT in the clinical assessment of patients with cochlear implants［J］. AJNR Am J Neuroradiol, 2014, 35(6): 1202－1208.
［48］ Jiam N T, Jiradejvong P, Pearl M S, et al. The effect of round window vs cochleostomy surgical approaches on cochlear implant electrode position: a Flat-Panel computed tomography study［J］. JAMA Otolaryngol Head Neck Surg, 2016, 142(9): 873－880.
［40］ Briggs R J S, Tykocinski M, Lazsig R, et al. Development and evaluation of the modiolar research array--multi-centre collaborative study in human temporal bones［J］. Cochlear Implants Int, 2011, 12(3): 129－139.
［50］ Wei X M, Li Y X, Chen B, et al. Predicting auditory outcomes from radiological imaging in cochlear implant patients with cochlear nerve deficiency［J］. Otol Neurotol, 2017, 38(5): 685－693.
［51］ Dhondt C, Maes L, Vanaudenaerde S, et al. Changes in vestibular function following pediatric cochlear implantation: a prospective study［J］. Ear Hear, 2022, 43(2): 620－630.
［52］ Shen M Y, Xue S J, Wei X M, et al. Characteristics of vestibular-evoked myogenic potentials in children with vestibular malformation and severe sensorineural hearing loss［J］. Int J Pediatr Otorhinolaryngol, 2024, 176: 111781.
［53］ Jacot E, Van Den Abbeele T, Debre H R, et al. Vestibular impairments pre- and post-cochlear implant in children［J］. Int J Pediatr Otorhinolaryngol, 2009, 73(2): 209－217.
［54］ Thierry B, Blanchard M, Leboulanger N, et al. Cochlear implantation and vestibular function in children［J］. Int J Pediatr Otorhinolaryngol, 2015, 79(2): 101－104.
［55］ Dhooge, Ingeborg, Van, et al. Examining the impact of cochlear implantation on the early gross motor development of children with a hearing loss［J］. Ear Hear, 2015, 36(3): E113－E121.
［56］ Coudert A, Van H T, Ayari-Khalfallah S, et al. Vestibular assessment in cochlear implanted children: how to do? When to do? A review of literature［J］. Curr Otorhinolaryngol Rep, 2017, 5(4): 259－267.
［57］ Potdukhe K, Vishwakarma R, Rao S, et al. Audiological and speech outcomes of cochlear implantation in inner ear malformation compared to the normal inner ear［J］. Indian J Otolaryngol Head Neck Surg, 2023, 75(3): 1870－1875.
［58］ Shi Y, Li Y X, Gong Y, et al. Cochlear implants for patients with inner ear malformation: Experience in a cohort of 877 surgeries［J］. Clin Otolaryngol, 2019, 44(4): 702－706.
［59］ Buchman C A, Copeland B J, Yu K K, et al. Cochlear implantation in children with congenital inner ear malformations［J］. Laryngoscope, 2004, 114(2): 309－316.
［60］ Benchetrit L, Jabbour N, Appachi S, et al. Cochlear implantation in pediatric patients with enlarged vestibular aqueduct: a systematic review［J］. Laryngoscope, 2022, 132(7): 1459－1472.
［61］ Farhood Z, Nguyen S A, Miller S C, et al. Cochlear implantation in inner ear malformations: systematic review of speech perception outcomes and intraoperative findings［J］. Otolaryngol Head Neck Surg (1979), 2017, 156(5): 783－793.
［62］ Lu S M, Xie J, Wei X M, et al. Machine Learning-Based prediction of the outcomes of cochlear implantation in patients with cochlear nerve deficiency and normal cochlea: a 2-Year Follow-Up of 70 children［J］. Front Neurosci, 2022, 16: 895560.
［63］ Lu S M, Wei X M, Kong Y, et al. A 3-Year Follow-Up and radiological analysis of cochlear implantation patients with cochlear nerve deficiency and modiolar Deficiency-Type malformations［J］. Otol Neurotol, 2023, 44(1): 26－33.
［64］ Braun K, Walker K, Sürth W, et al. Triphasic pulses in cochlear implant patients with facial nerve stimulation［J］. Otol Neurotol, 2019, 40(10): 1268－1277.
［65］ Wei X M, Zhang H Y, Lu S M, et al. Application of multiplanar volume reconstruction technique for the assessment of electrode location and analysis of the correlation to cochlear programming and performance in common cavity deformity［J］. Front Neurol, 2021, 12: 783225.
［66］ Pelosi S, Rivas A, Haynes D S, et al. Stimulation rate reduction and auditory development in poorly performing cochlear implant users with auditory neuropathy［J］. Otol Neurotol, 2012, 33(9): 1502－1506.
［67］ He S M, Shahsavarani B S, McFayden T C, et al. Responsiveness of the electrically stimulated cochlear nerve in children with cochlear nerve deficiency［J］. Ear Hear, 2018, 39(2): 238－250.
［68］ Young N M, Kim F M, Ryan M E, et al. Pediatric cochlear implantation of children with eighth nerve deficiency［J］. Int J Pediatr Otorhinolaryngol, 2012, 76(10): 1442－1448.
［69］ Wei X M, Lu S M, Chen B, et al. Cochlear implantation programming characteristics and outcomes of cochlear nerve deficiency［J］. Eur Arch Otorhinolaryngol, 2023, 280(10): 4409－4418.
［70］ He S M, Chao X H, Wang R J, et al. Recommendations for measuring the electrically evoked compound action potential in children with cochlear nerve deficiency［J］. Ear Hear, 2020, 41(3): 465－475.
［71］ Xue S J, Wei X M, Kong Y, et al. Trends in research on cochlear implantation with inner ear malformation: a bibliometric and visualization analysis from 1986 to 2024［J］. Eur Arch Otorhinolaryngol, 2024, 281(11): 5657－5667.
［72］ Sennarolu L, Yarali M, Sennarolu G, et al. Simultaneous cochlear and auditory brainstem implantation in children with severe inner ear malformations: initial surgical and audiological results［J］. Otol Neurotol, 2020, 41(5): 625－630.
［73］ Minami S B, Yamamoto N, Hosoya M, et al. Cochlear implantation in cases of inner ear malformation: a novel and simple grading, intracochlear EABR, and outcomes of hearing［J］. Otol Neurotol, 2021, 42(2): e117－e123.
［74］ Weng J L, Xue S J, Wei X M, et al. Machine learning-based prediction of the outcomes of cochlear implantation in patients with inner ear malformation［J］. Eur Arch Otorhinolaryngol, 2024, 281(7): 3535－3545.
［75］ Crowson M G, Lin V, Chen J M, et al. Machine learning and cochlear Implantation-A structured review of opportunities and challenges［J］. Otol Neurotol, 2020, 41(1): e36－e45.

[1]	张李芳, 魏兴梅, 孔颖, 杨梦戈, 高振橙, 薛书锦, 陆思萌, 陈彪, 陈婧媛, 石颖, 李永新. 共同腔畸形患者经乳突缝状迷路切开入路联合定制电极人工耳蜗植入术后长期效果研究[J]. 首都医科大学学报, 2024, 45(6): 946-955.
[2]	薛书锦, 魏兴梅, 高振橙, 孔颖, 陆思萌, 陈彪, 石颖, 崔丹默, 李永新. 耳蜗发育不全解剖学特点及人工耳蜗植入术后效果研究[J]. 首都医科大学学报, 2024, 45(6): 956-965.
[3]	沈梦雅, 薛书锦, 魏兴梅, 孔颖, 孙家强, 李永新. 人工耳蜗植入对内耳畸形患儿前庭功能影响的纵向研究：基于前庭诱发肌源性电位测试的比较分析[J]. 首都医科大学学报, 2024, 45(6): 980-988.
[4]	薛书锦, 魏兴梅, 陈彪, 高振橙, 崔丹默, 石颖, 陈婧媛, 孔颖, 李永新. 不全分隔Ⅰ型患者合并脑脊液漏人工耳蜗植入手术技术与术后效果研究[J]. 首都医科大学学报, 2024, 45(6): 995-1000.