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    

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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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
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