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

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3 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)231-235
Number of pages5
JournalAmerican Journal of Otolaryngology - Head and Neck Medicine and Surgery
Issue number3
Publication statusPublished - 2016 May 1

Bibliographical note

Funding Information:
Funding: This work was supported by a National Research Foundation of Korea (NRF) grant funded by the Korean government ( 2015R1D1A1A02062202 , 2014R1A2A1A11049478 and NRF-2013M3A9D5072551 ).

Publisher Copyright:
© 2016 Elsevier Inc. All rights reserved.

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology


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