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Case Reports: Learning from unexpected outcome (positive or negative)
Outcome of cochlear implantation in a case of cochlear aplasia with cochlear nerve deficiency
  1. Bibhu Prasad Hota,
  2. Sanjay Kumar Behera,
  3. Vinusree Karakkandy and
  4. Preetam Chappity
  1. ENT, AIIMS, Bhubaneswar, Odisha, India
  1. Correspondence to Dr Preetam Chappity; preetam82{at}gmail.com

Abstract

Cochlear nerve deficiency in cochlear aplasia is a contraindication for cochlear implantation (CI) anticipating poor auditory response. Few authors have reported auditory outcomes even without nerves following CI. This study outlines the audiological outcomes of a patient in early childhood with cochlear aplasia and cochlear nerve deficiency who underwent CI. Auditory and speech-language development were assessed using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale and Ling-6 sound test; at the time of switch-on, after 6 and 12 months of auditory verbal therapy. Significant differences across CAP, SIR and Ling-6 sound detection scores were noted, with the highest mean scores at the 12th-month postimplantation, indicating substantial improvement in auditory and speech-language skills. In cochlear aplasia cases, residual cochlear elements and nerve fibres cannot be ruled out. Our report emphasises the need for research, as this has the potential to impact the existing guidelines for CI candidacy.

  • Ear, nose and throat
  • Ear, nose and throat/otolaryngology

https://doi.org/10.1136/bcr-2022-253079

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    Footnotes

    • Contributors BPH is responsible for drafting the text, investigation and results, and collecting data. SKB is responsible for drafting the text, investigation and results, and collecting data. VK is responsible for drafting the text, results, collecting data, editing, sourcing and editing clinical images. PC is responsible for drafting the text, investigation and results, and critical revision for important intellectual content. The following author gave final approval of the manuscript: PC.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.