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, InSeok Moon, Kyu Rin Hwang, Jeon Mi Lee, Mi Young Bang, Jae Young Choi

Research output: Contribution to journalArticle

2 Citations (Scopus)

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. 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
Volume37
Issue number3
DOIs
Publication statusPublished - 2016 May 1

Fingerprint

De Lange Syndrome
Hearing Loss
Ventilation
Conductive Hearing Loss
Pediatrics
Middle Ear
Otitis Media with Effusion
Therapeutics
Hearing
Magnetic Resonance Imaging
Cochlear Implantation
Hearing Aids
Brain Stem Auditory Evoked Potentials
Temporal Bone
Inner Ear
Reoperation
Rehabilitation
Brain

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology

Cite this

Jung, Jinsei ; Park, Sera ; Kim, Sung Huhn ; Moon, InSeok ; Hwang, Kyu Rin ; Lee, Jeon Mi ; Bang, Mi Young ; Choi, Jae Young. / Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome. In: American Journal of Otolaryngology - Head and Neck Medicine and Surgery. 2016 ; Vol. 37, No. 3. pp. 231-235.
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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. Conclusion V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients.",
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Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome. / Jung, Jinsei; Park, Sera; Kim, Sung Huhn; Moon, InSeok; Hwang, Kyu Rin; Lee, Jeon Mi; Bang, Mi Young; Choi, Jae Young.

In: American Journal of Otolaryngology - Head and Neck Medicine and Surgery, Vol. 37, No. 3, 01.05.2016, p. 231-235.

Research output: Contribution to journalArticle

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T1 - Ventilation tube insertion is not effective to the treatment of hearing impairment in pediatric patients with Cornelia de Lange syndrome

AU - Jung, Jinsei

AU - Park, Sera

AU - Kim, Sung Huhn

AU - Moon, InSeok

AU - Hwang, Kyu Rin

AU - Lee, Jeon Mi

AU - Bang, Mi Young

AU - Choi, Jae Young

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N2 - 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.

AB - 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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