Hearing loss in children with Down syndrome

DOI
  • Yoshitomi Ai
    Department of Otorhinolaryngology, Tokyo Metropolitan Children’s Medical Center
  • Baba Shintaro
    Department of Otorhinolaryngology, Tokyo Metropolitan Children’s Medical Center
  • Kanemaru Asako
    Department of Otorhinolaryngology, Tokyo Metropolitan Children’s Medical Center

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  • ダウン症児の難聴の検討

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<p>Hearing loss is highly prevalent among children with Down syndrome (DS), but their hearing acuity sometimes improves as they mature. We retrospectively analyzed data on hearing loss collected from 298 children with DS who visited the Department of Genetics at Tokyo Metropolitan Children’s Medical Center between March 2010 and November 2017. Forty-six children with DS who failed their newborn hearing screening or were pointed out of hearing loss were referred to the Department of Otorhinolaryngology. ABR, ASSR, BOA, COR and play audiometry were performed to assess their hearing ability.</p><p>At the first assessment, 43 patients had hearing loss in at least one ear, 31 patients had bilateral hearing loss and 12 had unilateral hearing loss. We repeatedly performed the assessment, and 55% of ears with hearing loss eventually showed improvement. At the final assessment, 11 patients had bilateral hearing loss and 24 had unilateral hearing loss. Temporal bone CT was performed to examine the inner and middle ear structures in 14 patients. All eight ears in seven DS children with severe hearing loss showed the cochlear nerve canal stenosis while three of the eight ears also showed narrowing of the internal auditory canal, suggesting that this was the cause of the hearing loss. We detected otitis media with effusion (OME) in 64.9% of ears with hearing loss and performed ventilation tube insertion for 27.1% of these ears. Eleven children with DS started using hearing aids; of these, five showed an improvement in their hearing level and stopped using hearing aids. Only three patients continued using hearing aids. Our findings demonstrated that long-term follow up is important for assessing hearing in children with DS because of the high likelihood of improvement in hearing ability in this population.</p>

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