https://scholars.lib.ntu.edu.tw/handle/123456789/474455
標題: | Etiologic and audiologic characteristics of patients with pediatric-onset unilateral and asymmetric sensorineural hearing loss | 作者: | PEI-HSUAN LIN Hsu C.-J. Lin Y.-H. Lin Y.-H. Lee H.-Y. Chen-Chi Wu TIEN-CHEN LIU |
公開日期: | 2017 | 出版社: | American Medical Association | 卷: | 143 | 期: | 9 | 起(迄)頁: | 912-919 | 來源出版物: | JAMA Otolaryngology - Head and Neck Surgery | 摘要: | IMPORTANCE: Pediatric-onset unilateral and asymmetric sensorineural hearing loss (SNHL) is a common condition, but in most patients, the cause remains unclear; thus, determination of the hearing outlook is difficult. OBJECTIVE: To analyze the etiologic and audiologic characteristics of pediatric-onset unilateral and asymmetric SNHL. DESIGN, SETTING, AND PARTICIPANTS: In this retrospective cohort study performed from January 1, 2008, through December 31, 2016, patients at a tertiary referral center who were diagnosed with pediatric-onset unilateral or asymmetric SNHL were divided into 3 groups according to their hearing levels: unilateral hearing loss with scaled-out levels (UHL-SO), unilateral hearing loss with residual hearing (UHL-RH), and asymmetric hearing loss (AHL). MAIN OUTCOMES AND MEASURES: Basic demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and compared among patients of the 3 groups. RESULTS: A total of 133 patients (mean [SD] age, 9.1 [10.9] years; 63 [47.4%] male and 70 [52.6%] female), including 50 with UHL-SO, 42 with UHL-RH, and 41 with AHL, were enrolled for analyses. Of 50 patients with UHL-SO, 49 (98.0%) had stable hearing levels with time, whereas 10 of 42 patients with UHL-RH (23.8%) and 18 of 41 patients with AHL (43.9%) revealed progressive or fluctuating hearing loss. Inner ear malformations detected with temporal bone high-resolution computed tomography, particularly cochlear aperture stenosis, were detected at higher rates in patients with UHL-SO (9 of 31 [29.0%]) and UHL-RH (6 of 24 [25.0%]) than in those with AHL (1 of 30 [3.3%]). In contrast, screening for mutations in 3 common deafness genes—GJB2, SLC26A4, and MTRNR1—achieved definite diagnosis in a higher percentage of patients with AHL (10 of 37 [27.0%]) than patients with UHL-SO (0 of 33) and UHL-RH (1 of 25 [4.0%]). CONCLUSIONS AND RELEVANCE: The UHL-SO and UHL-RH conditions share a common or similar etiopathogenesis different from that of AHL. Imaging studies and genetic testing might be prioritized during the respective general etiologic workups for patients with UHL and AHL. Regular hearing checkups are warranted for patients with UHL and AHL because a certain proportion of patients might sustain progression in SNHL. ? 2017 American Medical Association. All rights reserved. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-85029832842&doi=10.1001%2fjamaoto.2017.0945&partnerID=40&md5=979bfe0b887b13bce973567da6a34ed6 https://scholars.lib.ntu.edu.tw/handle/123456789/474455 |
ISSN: | 2168-6181 | DOI: | 10.1001/jamaoto.2017.0945 | SDG/關鍵字: | connexin 26; pendrin; protein derivative; protein MTRNR1; unclassified drug; carrier protein; DFNA3 protein, human; gap junction protein; SLC26A4 protein, human; Article; asymmetric hearing loss; audiology; child; cochlea; cohort analysis; female; gene mutation; genetic analysis; genetic screening; high resolution computer tomography; human; inner ear malformation; major clinical study; male; onset age; pathogenesis; perception deafness; retrospective study; temporal bone; unilateral hearing loss; abnormalities; diagnostic imaging; genetics; Hearing Loss, Sensorineural; Hearing Loss, Unilateral; inner ear; inner ear disease; mutation; stenosis, occlusion and obstruction; vestibulocochlear nerve disease; Child; Cochlear Diseases; Cohort Studies; Connexins; Constriction, Pathologic; Ear, Inner; Female; Genetic Testing; Hearing Loss, Sensorineural; Hearing Loss, Unilateral; Humans; Male; Membrane Transport Proteins; Mutation; Retrospective Studies; Temporal Bone; Vestibulocochlear Nerve Diseases |
顯示於: | 醫學系 |
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