Volume 20 Issue 10
Oct.  2022
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Article Navigation >  Chinese Journal of General Practice  > 2022  >  20(10): 1645-1649
LU Jin-shan, ABULIKEMU Yiming, WU Mei, WUMAIER Yasen, CHEN Jie, ZHANG Xiao-yu, ZHANG Jin. Inner ear malformation among 255 cases with severe to extremely severe sensorineural hearing loss in Xinjiang and relevant issues[J]. Chinese Journal of General Practice, 2022, 20(10): 1645-1649. doi: 10.16766/j.cnki.issn.1674-4152.002669
Citation: LU Jin-shan, ABULIKEMU Yiming, WU Mei, WUMAIER Yasen, CHEN Jie, ZHANG Xiao-yu, ZHANG Jin. Inner ear malformation among 255 cases with severe to extremely severe sensorineural hearing loss in Xinjiang and relevant issues[J]. Chinese Journal of General Practice, 2022, 20(10): 1645-1649. doi: 10.16766/j.cnki.issn.1674-4152.002669
shu

Inner ear malformation among 255 cases with severe to extremely severe sensorineural hearing loss in Xinjiang and relevant issues

doi: 10.16766/j.cnki.issn.1674-4152.002669
  • 1.

    Department of Otolaryngology, People ' s Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xingjiang 830000, China

Funds:

 81460099

  • Received Date: 2022-04-21
    Available Online: 2022-11-30
    Abstract
  •   Objective  To investigate the image and surgical characteristics of inner ear malformation in children with severe to extremely severe sensorineural hearing loss, so as to provide better clinical guidance for cochlear implantation.   Methods  From January 2020 to December 2021, children admitted to People's Hospital of Xinjiang Uygur Autonomous Region for screening and cochlear implantation were examined by high-resolution CT of temporal bone and the cases of inner ear malformation were screened out. According to Sennaroglu ' s classification method, they were classified as follows: Michel malformation, cochlea aplasia, common cavity, cochlear hypoplasia types Ⅰ, Ⅱ and Ⅲ, incomplete partition types Ⅰ-Ⅲ (IP-Ⅰ, IP-Ⅱ and IP-Ⅲ), vestibular and semicircular canal malformation, large vestibular aqueduct and internal auditory canal stenosis. Cochlear implantation approaches, electrode types and complications were recorded, and the image characteristics and surgical precautions of various malformations were analysed.   Results  Among 255 patients with severe to extremely severe sensorineural hearing loss, 66 cases (125 ears) had inner ear malformation, in which IP-Ⅱ accounted for 30.30% (20 cases/66 cases) of inner ear malformation cases, large vestibular aqueduct accounted for 19.70% (13 cases/66 cases) and other types were relatively low. A total of 55 children (57 ears) with inner ear malformation received cochlear implantation (CI), including two bilateral CI cases. All cases had been implanted with electrodes through a cochleotomy or round window. Short straight electrodes were implanted in patients with cochlear hypoplasia types Ⅱ and Ⅲ, IP-Ⅰ and IP-Ⅲ. Standard electrodes were implanted in patients with IP-Ⅱ, large vestibular aqueduct and enlarged internal auditory canal. Postoperative CT scans suggested one IP-Ⅱ case were implanted into vestibular cavity, so this case received another operation by cochlear implantation successfully. The incidence of cerebrospinal fluid "gusher" was 29.82% (17 ears/57 ears). There was no complications such as cerebrospinal fluid otorrhea and flap necrosis.   Conclusion  IP-Ⅱ and LVA are the predominant types of inner ear malformations. The incidence of cerebrospinal fluid "gusher" in CI is high in children with inner ear malformation. HRCT and MRI are complementary, which can clearly and comprehensively display the inner ear structure and provide guarantee for cochlear implementation successfully.

     

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    • References(27)
  • [1]
    DAHLEN R T, HARNSBERGER H R, GRAY S D, et al. Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome[J]. AJNR Am J Neuroradiol, 1997, 18(1): 67-75.
    [2]
    刘军, 李长勤. 微小内耳畸形的感音神经性耳聋患儿与正常儿童内耳HRCT标准化测量的对比研究[J]. 医学影像学杂志, 2016, 26(11): 1962-1965. https://www.cnki.com.cn/Article/CJFDTOTAL-XYXZ201611005.htm

    LIU J, LI C Q. The comparative study of small inner ear malformation of sensorineural hearing loss children and norraul children of the comparison[J]. J Med Imaging, 2016, 26(11): 1962-1965. https://www.cnki.com.cn/Article/CJFDTOTAL-XYXZ201611005.htm
    [3]
    鲁兆毅, 王宇, 潘滔. 听神经发育异常合并中耳内耳畸形及面神经畸形的人工耳蜗植入术1例[J]. 中华耳科学杂志, 2018, 16(6): 861-863. doi: 10.3969/j.issn.1672-2922.2018.06.022

    LU Z Y, WANG Y, PAN T. Cochlear implantation in a patient with auditory nerve hypoplasia, middle and inner ear malformation and facial nerve malformation: A case report[J]. Chinese Journal of Otology, 2018, 16(6): 861-863. doi: 10.3969/j.issn.1672-2922.2018.06.022
    [4]
    马辉, 韩萍, 梁波, 等. 多层螺旋CT对先天性内耳发育畸形的诊断价值[J]. 中华耳鼻咽喉头颈外科杂志, 2005, 40(4): 275-278. doi: 10.3760/j.issn:1673-0860.2005.04.009

    MA H, HAN P, LIANG B, et al. Diagnostic significance of multi-slice computed tomography imaging in congenital inner ear malformations[J]. Chin J Otorhinolaryngol Head Neck Surg, 2005, 40(4): 275-278. doi: 10.3760/j.issn:1673-0860.2005.04.009
    [5]
    鲜军舫, 王振常, 燕飞, 等. MRI快速自旋回波T2WI三维重建技术在内耳病变中的应用[J]. 中华放射学杂志, 1999, 33(7): 473-476. doi: 10.3760/j.issn:1005-1201.1999.07.009

    XIAN J F, WANG Z C, YAN F, et al. Application of MRI fast spin echo T2Wl 3D reconstruction in inner ear lesions[J]. Chinese Journal of Radiology, l999, 33(7): 473-476. doi: 10.3760/j.issn:1005-1201.1999.07.009
    [6]
    李建红, 王振常, 鲜军舫, 等. MRI在先天性内耳畸形儿童人工耳蜗植入术前的评估价值[J]. 磁共振成像, 2012, 3(6): 415-419. https://www.cnki.com.cn/Article/CJFDTOTAL-CGZC201206007.htm

    LI J H, WANG Z C, XIAN J F, et al. Value of MRI in evaluating the children with congenital inner ear malformations before cochlear implantation[J]. Chin J Magn Reson Imaging, 2012, 3(6): 415-419. https://www.cnki.com.cn/Article/CJFDTOTAL-CGZC201206007.htm
    [7]
    丁贺宇, 赵鹏飞, 吕晗, 等. 先天性感音神经性耳聋面神经管迷路段与耳蜗间骨壁的HRCT研究[J]. 临床放射学杂志, 2019, 38(2): 229-232. https://www.cnki.com.cn/Article/CJFDTOTAL-LCFS201902010.htm

    DING H Y, ZHAO P F, LV H, et al. High-resolution CT study on the bony septum between labyrinth segment of facial nerve canal and cochlear in congenital sensorineural hearing loss[J]. Chin J Magn Reson Imaging, 2019, 38(2): 229-232. https://www.cnki.com.cn/Article/CJFDTOTAL-LCFS201902010.htm
    [8]
    周宝, 林少莲, 林有辉, 等. 先天性内耳畸形的影像学及听力学分析[J]. 临床耳鼻咽喉头颈外科杂志, 2015, 29(22): 1950-1954. https://www.cnki.com.cn/Article/CJFDTOTAL-LCEH201522005.htm

    ZHOU B, LIN S L, LING Y H, et al. Imaging and audiology analysis of the congenital inner ear malformations[J]. J Clin Otorhinolaryngol Head and Neck Surg, 2015, 29(22): 1950-1954. https://www.cnki.com.cn/Article/CJFDTOTAL-LCEH201522005.htm
    [9]
    KUMAR S, MAJHI B N, YADAV K K, et al. Radiological anatomy of inner ear malformation in hearing impaired children and it ' s correlation with hearing loss: A hospital based observational study[J]. Indian J Otolaryngol Head Neck Surg, 2018, 70(2): 278-283. doi: 10.1007/s12070-017-1238-7
    [10]
    SENNAROGLU L. A new classification for cochleovestibular malformations[J]. Laryngoscope, 2002, 112(12): 2230-2241. doi: 10.1097/00005537-200212000-00019
    [11]
    姜泗长. 耳科学[M]. 上海: 上海科学技术出版社, 2002: 24-33.

    JIANG S C. Otology[M]. Shanghai: Shanghai Science and Technology Press, 2002: 24-33.
    [12]
    SNOW J B, WACKYM P A. Ballenger耳鼻咽喉头颈外科学[M]. 北京: 人民卫生出版社, 2012: 25-28.

    SNOW J B, WACKYM P A. Ballenger otorhinolaryngology head neck surgery[M]. Beijing: People ' s Medical Publishing House, 2012: 25-28.
    [13]
    LIM R, BRICHTA A M. Anatomical and physiological development of the human inner ear[J]. Hear Res, 2016, 338(8): 9-21.
    [14]
    孙宝春. 内耳畸形影像学诊断及分类的研究进展[J]. 医学信息(中旬刊), 2011, 24(5): 1958-1959. https://www.cnki.com.cn/Article/CJFDTOTAL-YXSS201105328.htm

    SUN B C. Advances in imaging diagnosis and classification of inner ear malformations[J]. Medical Information, 2011, 24(5): 1958-1959. https://www.cnki.com.cn/Article/CJFDTOTAL-YXSS201105328.htm
    [15]
    JACKLER R K, CRUZ A D L. The large vestibular aqueduct syndrome[J]. Laryngoscope, 1989, 99(12): 1238-1242.
    [16]
    GOLDFELD M, GLASER B, NASSIR E, et al. CT of the ear in Pendred syndrome[J]. Radiology, 2005, 235(2): 537-540.
    [17]
    耿国兴, 林彩娟, 黄小桃, 等. 42 708例新生儿耳聋基因筛查结果分析[J]. 中华全科医学, 2020, 18(10): 1688-1690. doi: 10.16766/j.cnki.issn.1674-4152.001594

    GENG G X, LING C J, HUANG X T, et al. An analysis of deafness genes screening in 42 708 newborns[J]. Chinese Journal of General Practice, 2020, 18(10): 1688-1690. doi: 10.16766/j.cnki.issn.1674-4152.001594
    [18]
    刘兰, 费静, 陶美慧, 等. 内耳发育畸形患者人工耳蜗植入术后的疗效分析[J]. 中国耳鼻咽喉颅底外科杂志, 2019, 25(2): 152-156. https://www.cnki.com.cn/Article/CJFDTOTAL-ZEBY201902013.htm

    LIU L, FEI J, TAO M H, et al. Efficacy of cochlear implantation in patients with inner ear malformations[J]. Chinese Journal of Otorhinolaryngology-skull Base Surgery, 2019, 25(2): 152-156. https://www.cnki.com.cn/Article/CJFDTOTAL-ZEBY201902013.htm
    [19]
    陈艳丹, 陈柳, 华清泉. 内耳发育畸形的人工耳蜗植入术[J]. 中国医药导报, 2017, 14(12): 113-116. https://www.cnki.com.cn/Article/CJFDTOTAL-YYCY201712028.htm

    CHEN Y D, CHEN L, HUA Q Q. Cochlear implantation of the inner ear malformations[J]. China Medical Hearld, 2017, 14(12): 113-116. https://www.cnki.com.cn/Article/CJFDTOTAL-YYCY201712028.htm
    [20]
    MATHIS J M, SIMMONS D M, HE X, et al. Brain 4: A novel mammalian POU domain transcription factor exhibiting restricted brain-specific expression[J]. EMBO J, 1992, 11(7): 2551-2561.
    [21]
    李庆忠, 王秋菊, 赵亚丽, 等. 先天性内耳畸形家系致病基因的定位克隆[J]. 中国耳鼻咽喉头颈外科, 2006, 13(10): 678-682. https://www.cnki.com.cn/Article/CJFDTOTAL-EBYT200610006.htm

    LI Q Z, WANG Q J, ZHAO Y L, et al. PositinaI cloning in Chinese X-linked congenital inner ear malformation family[J]. Chin Arch Otolaryngol Head Neck Surg, 2006, 13(10): 678-682. https://www.cnki.com.cn/Article/CJFDTOTAL-EBYT200610006.htm
    [22]
    WILKINS A, PRABHU S P, HUANG L, et al. Frequent association of cochlear nerve canal stenosis with pediatric sensorineural hearing loss[J]. Arch Otolaryngol Head Neck Surg, 2012, 138(4): 383-388.
    [23]
    刘贝贝, 王建朝, 徐百成, 等. 内耳畸形在人工耳蜗植入患者中的构成及临床分析[J]. 中华耳科学杂志, 2020, 18(2): 295-300. https://www.cnki.com.cn/Article/CJFDTOTAL-ZHER202002014.htm

    LIU B B, WANG J C, XU B C, et al. Inner ear malformation among cochlear implant recipients and relevant issues[J]. Chinese Journal of Otology, 2020, 18(2): 295-300. https://www.cnki.com.cn/Article/CJFDTOTAL-ZHER202002014.htm
    [24]
    李幼瑾, 杨军, 李蕴. 儿童感音神经性耳聋中先天性内耳畸形的临床特征[J]. 中国临床医学, 2011, 18(2): 215-217. https://www.cnki.com.cn/Article/CJFDTOTAL-LCYX201102030.htm

    LI Y J, YANG J, LI Y. Clinical features of children with congenital malformation of inner ear in sensorineural hearing loss[J]. Chinese Journal of Clinical Medicine, 2011, 18(2): 215-217. https://www.cnki.com.cn/Article/CJFDTOTAL-LCYX201102030.htm
    [25]
    YUAN Y Y, GUO W W, TANG J, et al. Molecular epidemiology and functional assessment of novel allelic variants of SLC26A4 in non-syndromic hearing loss patients with enlarged vestibular aqueduct in China[J]. PLoS One, 2012, 7(11): e49984. DOI: 10.1371/journal.pone.0049984.
    [26]
    MEY K, BILLE M, CAYÉ-THOMASEN P. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct-clinical challenges, surgical results, and complications[J]. Acta Otolaryngol, 2016, 136(10): 1064-1068.
    [27]
    王全, 周航, 王鹏, 等. 内耳磁共振水成像在内耳病变诊断中的应用价值[J]. 中国基层医药, 2017, 24(19): 2897-2901. https://www.cnki.com.cn/Article/CJFDTOTAL-SYYJ202004014.htm

    WANG Q, ZHOU H, WANG P, et al. Clinical value of magnetic resonance imaging in the diagnosis of inner ear lesion[J]. Chin J Prim Med Pharm, 2017, 24(19): 2897-2901. https://www.cnki.com.cn/Article/CJFDTOTAL-SYYJ202004014.htm
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