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Peer-reviewedCC BY-NC-ND 4.0The Journal of Laryngology and Otology

Sudden hearing loss in enlarged vestibular aqueduct syndrome: a systematic review of treatment and outcomes

  • Ching Yee Chan1,2
  • Joshua Nadler3
  • Sheng Yep Brian Yeo4
  • Xue Li Samantha Sim1
  • Joshua Gurberg2
4 affiliations
  1. 1Department of Otolaryngology, KK Women’s and Children’s Hospital, Singapore
  2. 2Department of Otolaryngology–Head and Neck Surgery and Paediatric Surgery, Montreal Children’s Hospital, McGill University, Montreal, QC, Canada
  3. 3Hamilton College, Clinton, NY, USA
  4. 4Yong Loo Lin School of Medicine, National University of Singapore, Singapore
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Abstract

Objectives: Up to 60 per cent of patients with enlarged vestibular aqueduct syndrome experience sudden hearing loss. This review analyses treatment options and outcomes when sudden hearing loss occurs.

Methods: Embase, Ovid MEDLINE and Web of Science databases were the data sources. This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and was registered on PROSPERO. The number of patients, episodes, treatments, baseline hearing and change in hearing thresholds after intervention and return to baseline were extracted.

Results: The final analysis included 60 episodes of hearing loss from 11 studies, from a search of 1,064 studies. The treatment groups were: 1, no treatment; 2, systemic steroids; 3, salvage hyperbaric oxygen therapy in steroid partial or non-responders. The hearing improvement across the groups was 9.1 per cent, 50 per cent and 89.5 per cent, respectively.

Conclusion: Enlarged vestibular aqueduct patients with sudden hearing loss may first be treated with systemic steroids. Salvage hyperbaric oxygen therapy can be considered in steroid non-responders to improve further chances of recovery.

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Cite

BibTeXRIS
BibTeX
@article{chan2026sudden,
  title = {{Sudden hearing loss in enlarged vestibular aqueduct syndrome: a systematic review of treatment and outcomes}},
  author = {Ching Yee Chan and Joshua Nadler and Sheng Yep Brian Yeo and Xue Li Samantha Sim and Joshua Gurberg},
  journal = {The Journal of Laryngology and Otology},
  year = {2026},
  month = nov,
  doi = {10.1017/s0022215126105088},
  url = {https://doi.org/10.1017/s0022215126105088},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Sudden hearing loss in enlarged vestibular aqueduct syndrome: a systematic review of treatment and outcomes
AU  - Ching Yee Chan
AU  - Joshua Nadler
AU  - Sheng Yep Brian Yeo
AU  - Xue Li Samantha Sim
AU  - Joshua Gurberg
PY  - 2026
DA  - 2026/11/06
JO  - The Journal of Laryngology and Otology
DO  - 10.1017/s0022215126105088
UR  - https://doi.org/10.1017/s0022215126105088
AB  - Objectives: Up to 60 per cent of patients with enlarged vestibular aqueduct syndrome experience sudden hearing loss. This review analyses treatment options and outcomes when sudden hearing loss occurs.  Methods: Embase, Ovid MEDLINE and Web of Science databases were the data sources. This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and was registered on PROSPERO. The number of patients, episodes, treatments, baseline hearing and change in hearing thresholds after intervention and return to baseline were extracted.  Results: The final analysis included 60 episodes of hearing loss from 11 studies, from a search of 1,064 studies. The treatment groups were: 1, no treatment; 2, systemic steroids; 3, salvage hyperbaric oxygen therapy in steroid partial or non-responders. The hearing improvement across the groups was 9.1 per cent, 50 per cent and 89.5 per cent, respectively.  Conclusion: Enlarged vestibular aqueduct patients with sudden hearing loss may first be treated with systemic steroids. Salvage hyperbaric oxygen therapy can be considered in steroid non-responders to improve further chances of recovery.
SN  - 0022-2151
SN  - 1748-5460
ER  -