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Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome

 Jinsei Jung  ;  Sera Park  ;  Sung Huhn Kim  ;  In Seok Moon  ;  Kyu Rin Hwang  ;  Jeon Mi Lee  ;  Mi Young Bang  ;  Jae Young Choi 
 American Journal of Otolaryngology, Vol.37(3) : 231-235, 2016 
Journal Title
 American Journal of Otolaryngology 
Issue Date
Audiometry ; Child ; Child, Preschool ; De Lange Syndrome/complications* ; De Lange Syndrome/diagnostic imaging ; De Lange Syndrome/surgery* ; Female ; Hearing Loss, Conductive/diagnosis ; Hearing Loss, Conductive/etiology* ; Hearing Loss, Conductive/surgery* ; Humans ; Male ; Middle Ear Ventilation* ; Patient Selection ; Retrospective Studies ; Treatment Outcome
OBJECTIVE: Cornelia de Lange syndrome (CdLS) is a multiple developmental disorder including hearing loss. The hearing impairment in CdLS patients is not only sensorineural but also conductive hearing loss (CHL). The aim of this study was to elucidate hearing loss causes in CdLS patients and evaluate the effect of ventilation tube (v-tube) insertion in the cases of CHL. METHODS: Thirty-two patients clinically diagnosed with CdLS were enrolled and analyzed with retrospective case review. Audiologic evaluations and imaging studies such as a temporal bone computed tomogram or brain magnetic resonance imaging (MRI) were performed for all patients. Hearing rehabilitation such as ventilation tube insertion, hearing aid fitting, or cochlear implantation was chosen depending on the audiological condition. RESULTS: Among 32 CdLS patients who underwent auditory brainstem response test, 81.2% presented hearing loss. Imaging studies showed that only middle ear lesions without inner ear anomalies were identified in 56.3%. Notably, the soft tissue lesion in middle ear was identified even in the neonatal MRI. When 7 patients were thought to have CHL due to otitis media with effusion, v-tube insertion was applied first. However, v-tube insertion rarely improved CHL postoperatively. Moreover, middle ear lesion was not fluid effusion but soft tissue lesion according to the intraoperative finding. These lesions were not eradicated even after revision surgery of v-tube insertion. CONCLUSION: V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients.
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1. College of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Sung Huhn(김성헌)
Moon, In Seok(문인석) ORCID logo https://orcid.org/0000-0002-3951-5074
Bang, Mi Young(방미영)
Lee, Jeon Mi(이전미)
Jung, Jinsei(정진세) ORCID logo https://orcid.org/0000-0003-1906-6969
Choi, Jae Young(최재영) ORCID logo https://orcid.org/0000-0001-9493-3458
Hwang, Kyu Rin(황규린)
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