Journal of Otolaryngology and Ophthalmology of Shandong University ›› 2024, Vol. 38 ›› Issue (4): 96-196.doi: 10.6040/j.issn.1673-3770.0.2024.219

• Original Article • Previous Articles     Next Articles

Analysis of clinical data form 112 cases of children with congenital single-sided deafness

ZHANG Xiao1, LIU Haihong1, LIU Wei1, LI Ying1, CHEN Min1, LIU Bing1, BAI Jie1, ZHANG Jie1, ZHENG Jun2, YANG Yang1   

  1. 1. Department of Otorhinolaryngology & Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University National Center for Children's Health, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, Beijing 100045, China2. Department of Otorhinolaryngology & Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100005, China
  • Published:2024-07-09

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221

Abstract: Objective To investigate the causes/detection pathways, audiological characteristics, and imaging features of children with congenital single-sided deafness(SSD), and to summarize the clinical characteristics and intervention strategies. Methods We retrospectively analyzed the clinical data of children diagnosed with congenital SSD at the Department of Otorhinolaryngology, Beijing Children's Hospital; from June 2020 to June 2022, including general information of the children, reason for consultation/path of discovery, ABR, pure tone audiometry, CM, OAE, acoustic impedance, temporal bone CT and/or inner auditory canal MRI, summarized the clinical characteristics and followed up the children who received treatment. Results A total of 112 children were enrolled in this study, including 66 males and 46 females; 61 left ears and 51 right ears; 0-13 years old; OAE: none of the deaf ears were elicited; CM: 38 deaf ears could be elicited; the highest proportion of children visited the clinic due to failure of newborn hearing screening(41.1%); 75 cases(66.9%)had structural anomalies shown by imaging, with the proportion the highest percentage was 62 cases(82.7%)with simple cochlear nerve foramen stenosis/cochlear nerve dysplasia; 2 children with SSD underwent surgical interventions, including 1 case of cochlear implantation without cochlear nerve dysplasia and 1 case of bone bridge implantation with cochlear nerve dysplasia. Six months after surgery, both children's RMSE and BIAS improved, and the ability to localize the sound source was improved. Conclusion Children with congenital SSD have a high proportion of structural abnormalies of the inner ear, with stenosis of the cochlear nerve foramen/cochlear nerve hypoplasia being the most common. SSD in children is not easy to detect, and attention should be paid to newborn hearing screening and hearing screening, and vigilance should be exercised to prevent the occurrence of potential traffic accident risks. Given the current situation that the intervention rate of congenital SSD is extremely low, we should improve the understanding of the characteristics of the disease and provide individualised and scientific interventions.

Key words: Children, Congenital single-sided deafness, Inner ear malformation, Hearing screen, Cochlear nerve

CLC Number: 

  • R764.43
[1] 郗昕. 成人单侧聋和不对称性听力损失干预方式的评估框架[J]. 中国听力语言康复科学杂志, 2018, 16(6): 401-406. doi:10.3969/j.issn.1672-4933.2018.06.001 XI Xin. Framework of various treatments for adults with single-sided deafness or asymmetric hearing loss[J]. Chinese Scientific Journal of Hearing and Speech Rehabilitation, 2018, 16(6): 401-406. doi:10.3969/j.issn.1672-4933.2018.06.001
[2] Mahomva C, Kim A, Lieu JEC, et al. Speech and language outcomes in mild-moderate unilateral sensorineural hearing loss[J]. Int J Pediatr Otorhinolaryngol, 2021, 141: 110558. doi:10.1016/j.ijporl.2020.110558
[3] Yan F, Li JH, Xian JF, et al. The cochlear nerve canal and internal auditory canal in children with normal cochlea but cochlear nerve deficiency[J]. Acta Radiol, 2013, 54(3): 292-298. doi:10.1258/ar.2012.110596
[4] Stjernholm C, Muren C. Dimensions of the cochlear nerve canal: a radioanatomic investigation[J]. Acta Otolaryngol, 2002, 122(1): 43-48. doi:10.1080/00016480252775724
[5] Glastonbury CM, Davidson HC, Harnsberger HR, et al. Imaging findings of cochlear nerve deficiency[J]. AJNR Am J Neuroradiol, 2002, 23(4): 635-643
[6] Young NM, Kim FM, Ryan ME, et al. Pediatric cochlear implantation of children with eighth nerve deficiency[J]. Int J Pediatr Otorhinolaryngol, 2012, 76(10): 1442-1448. doi:10.1016/j.ijporl.2012.06.019
[7] 刘佳星, 王宁宇, 李欢, 等. 言语空间听觉质量量表-父母版的信效度分析[J]. 中华耳科学杂志, 2021, 19(2): 265-272. doi:10.3969/j.issn.1672-2922.2021.02.017 LIU Jiaxing, WANG Ningyu, LI Huan, et al. Validation of Chinese version of the speech, spatial and other qualities of hearing scale for parents[J]. Chinese Journal of Otology, 2021, 19(2): 265-272. doi:10.3969/j.issn.1672-2922.2021.02.017
[8] 李颖, 林智涵,王雪瑶,等.儿童单侧听神经病的识别[J].中华耳鼻咽喉头颈外科杂志, 2024,59(5): 18-25.doi:10.3760/cma.j.cn115330-20231129-00248 LI Ying, LIN Zhihan, WANG Xueyao, et al. Recognition of unilateral auditory neuropathy in children[J]. Chinese Journal of Otorhinolaryngology Head and Neck Surgery, 2024,59(5): 18-25.doi:10.3760/cma.j.cn115330-20231129-00248
[9] 盘琳琳, 孔令漪, 翟丰, 等. 新生儿听力障碍常见危险因素及听力筛查方法研究进展[J]. 山东大学耳鼻喉眼学报, 2022, 36(1): 131-137. doi:10.6040/j.issn.1673-3770.0.2021.080 PAN Linlin, KONG Lingyi, ZHAI Feng, et al. Research progress on auditory risk factors and hearing screening methods among neonates[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2022, 36(1): 131-137. doi:10.6040/j.issn.1673-3770.0.2021.080
[10] Vincent C, Arndt S, Firszt JB, et al. Identification and evaluation of cochlear implant candidates with asymmetrical hearing loss[J]. Audiol Neurootol, 2015, 20(Suppl 1): 87-89. doi:10.1159/000380754
[11] Usami SI, Kitoh R, Moteki H, et al. Etiology of single-sided deafness and asymmetrical hearing loss[J]. Acta Otolaryngol, 2017, 137(sup565): S2-S7. doi:10.1080/00016489.2017.1300321
[12] Clemmens CS, Guidi J, Caroff A, et al. Unilateral cochlear nerve deficiency in children[J]. Otolaryngol Head Neck Surg, 2013, 149(2): 318-325. doi:10.1177/0194599813487681
[13] van Beeck Calkoen EA, Engel MSD, van de Kamp JM, et al. The etiological evaluation of sensorineural hearing loss in children[J]. Eur J Pediatr, 2019, 178(8): 1195-1205. doi:10.1007/s00431-019-03379-8
[14] Paul A, Marlin S, Parodi M, et al. Unilateral sensorineural hearing loss: medical context and etiology[J]. Audiol Neurootol, 2017, 22(2): 83-88. doi:10.1159/000474928
[15] Sunwoo W, Lee WW, Choi BY. Extremely common radiographic finding of cochlear nerve deficiency among infants with prelingual single-sided deafness and its clinical implications[J]. Int J Pediatr Otorhinolaryngol, 2018, 112: 176-181. doi:10.1016/j.ijporl.2018.07.013
[16] Boudewyns A, Declau F, van den Ende J, et al. Auditory neuropathy spectrum disorder(ANSD)in referrals from neonatal hearing screening at a well-baby clinic[J]. Eur J Pediatr, 2016, 175(7): 993-1000. doi:10.1007/s00431-016-2735-5
[17] Ward KM, Coughran AJ, Lee M, et al. Prevalence of cochlear nerve deficiency and hearing device use in children with single-sided deafness[J]. Otolaryngol Head Neck Surg, 2023, 169(2): 390-396. doi:10.1002/ohn.255
[18] 陈敏, 高军, 刘薇, 等. 儿童内耳畸形相关脑脊液耳漏的影像学特点[J]. 中华耳鼻咽喉头颈外科杂志, 2023, 58(5): 452-459. doi:10.3760/cma.j.cn115330-20220527-00310 CHEN Min, GAO Jun, LIU Wei, et al. Imaging evaluation of cerebrospinal fluid otorrhea associated with inner ear malformation in children[J]. Chinese Journal of Otorhinolaryngology Head and Neck Surgery, 2023, 58(5): 452-459. doi:10.3760/cma.j.cn115330-20220527-00310
[19] Zwierz A, Masna K, Burduk P. Recurrent meningitis in congenital inner ear malformation[J]. Ear Nose Throat J, 2021, 100(1_suppl): 38S-41S. doi:10.1177/0145561320920399
[20] 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. doi:10.1016/j.ijporl.2019.05.045
[21] 陈敏, 郑军, 刘薇, 等. 儿童脑脊液耳漏修补与人工耳蜗植入同期手术的探讨[J]. 中华耳科学杂志, 2020, 18(4): 663-667. doi:10.3969/j.issn.1672-2922.2020.04.006 CHEN Min, ZHENG Jun, LIU Wei, et al. Simultaneous cerebrospinal fluid(CSF)otorrhea repair and cochlear implantation in children with bilateral inner ear malformation[J]. Chinese Journal of Otology, 2020, 18(4): 663-667. doi:10.3969/j.issn.1672-2922.2020.04.006
[22] 胡春燕, 党攀红, 张睿, 等. 儿童单侧感音性听力损失的听力学及影像学特征149例分析[J]. 山东大学耳鼻喉眼学报, 2022, 36(1): 31-36. doi:10.6040/j.issn.1673-3770.0.2021.418 HU Chunyan, DANG Panhong, ZHANG Rui, et al. Analysis of audiological and imaging characteristics of 149 children with unilateral sensorineural hearing loss[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2022, 36(1): 31-36. doi:10.6040/j.issn.1673-3770.0.2021.418
[23] 王梦, 龚树生. 单侧聋的干预进展[J]. 听力学及言语疾病杂志, 2021, 29(6): 695-700. doi:10.3969/j.issn.1006-7299.06.024 WANG Meng, GONG Shusheng. Intervention progress of unilateral deafness[J]. Journal of Audiology and Speech Pathology, 2021, 29(6): 695-700. doi:10.3969/j.issn.1006-7299.06.024
[24] 银力, 高珊仙, 屠文河, 等. 单侧聋患者人工耳蜗植入的进展[J]. 听力学及言语疾病杂志, 2017, 25(2): 210-215. doi:10.3969/j.issn.1006-7299.2017.02.021 YIN Li, GAO Shanxian, TU Wenhe, et al. Progress of cochlear implant in patients with unilateral deafness[J]. Journal of Audiology and Speech Pathology, 2017, 25(2): 210-215. doi:10.3969/j.issn.1006-7299.2017.02.021
[25] Park LR, Griffin AM, Sladen DP, et al. American cochlear implant alliance task force guidelines for clinical assessment and management of cochlear implantation in children with single-sided deafness[J]. Ear Hear, 2022, 43(2): 255-267. doi:10.1097/AUD.0000000000001204
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