Characterization of children with auditory neuropathy spectrum disorder and their rehabilitation

Authors

  • Marta Mariano Serviço de Otorrinolaringologia, Centro Hospitalar Universitário de Lisboa Central, Portugal http://orcid.org/0000-0002-6898-9631
  • Isabel Correia Serviço de Otorrinolaringologia, Centro Hospitalar Universitário de Lisboa Central, Portugal
  • Sofia Nunes Serviço de Genética Médica, Centro Hospitalar Universitário de Lisboa Central, Portugal
  • Inês Cunha Serviço de Otorrinolaringologia, Centro Hospitalar Universitário de Lisboa Central, Portugal
  • Herédio Sousa Serviço de Otorrinolaringologia, Centro Hospitalar Universitário de Lisboa Central, Portugal
  • Teresa Kay Serviço de Genética Médica, Centro Hospitalar Universitário de Lisboa Central, Portugal
  • Ezequiel Barros Serviço de Otorrinolaringologia, Centro Hospitalar Universitário de Lisboa Central, Portugal

DOI:

https://doi.org/10.34631/sporl.875

Keywords:

Auditory Neuropathy Spectrum Disorder, Otoacoustic emissions, Brainstem auditory evoked responses, Newborn hearing screening, Sensorineural hearing loss

Abstract

Objectives: To identify and characterise the paediatric cases of Auditory Neuropathy Spectrum Disorder (ANSD) and to analyse its rehabilitation. 

Study Design: Descriptive observational study. 

Material e Methods: Analysis of clinical processes from 671 patients evaluated in a first auditory rehabilitation appointment between 2012 and 2019. From 467 cases with sensorineural hearing loss, those with absent or profoundly altered brainstem auditory evoked responses and present otoacoustic emissions and/or pure tone thresholds disproportional to electrophysiological results were included. Twelve cases of ANSD were obtained. 

Results: The prevalence of ANSD was 2.6%. Most cases had risk factors for hearing loss. Audiometric results were heterogeneous and fluctuating. Four children were rehabilitated with hearing aids and three with cochlear implants. 

Conclusion: ANSD is an infrequent condition. It is associated with several risk factors and its diagnosis and management are challenging. Customised and guided by the child’s functional development auditory rehabilitation is mandatory. 

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Published

2021-03-07

How to Cite

Mariano, M., Correia, I., Nunes, S., Cunha, I., Sousa, H., Kay, T., & Barros, E. (2021). Characterization of children with auditory neuropathy spectrum disorder and their rehabilitation. Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery, 59(1), 55–61. https://doi.org/10.34631/sporl.875

Issue

Vol. 59 No. 1 (2021): March

Section

Original Article

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