title: Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome.
abstract: 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.
  
  
  CONCLUSIONS
  V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients.
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Jinsei
authors.initials: J
authors.lastname: Jung
authors.affiliation: Department of Otorhinolaryngology, Bundang Jesaeng Hospital, Seongnam-si, Republic of Korea.
authors.forename: Sera
authors.initials: S
authors.lastname: Park
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Sung Huhn
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: In Seok
authors.initials: IS
authors.lastname: Moon
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Kyu Rin
authors.initials: KR
authors.lastname: Hwang
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Jeon Mi
authors.initials: JM
authors.lastname: Lee
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Mi Young
authors.initials: MY
authors.lastname: Bang
authors.affiliation: Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address: jychoi@yuhs.ac.
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Choi
mesh_terms: D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D003635:De Lange Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006314:Hearing Loss, Conductive / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008297:Male; D008876:Middle Ear Ventilation; D018579:Patient Selection; D012189:Retrospective Studies; D016896:Treatment Outcome