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Updated: Feb 12, 2026

Author Spotlight: Optimizing EAS with Long Electrodes for Enhanced Cochlear Coverage and Hearing Preservation
Published on: October 11, 2024
Emberger syndrome: A rare association with hearing loss
Faisal Zawawi1, Meirav Sokolov2, Thomas Mawby1
1Department of Otolaryngology, Head and Neck Surgery, Hospital for Sick Children, Toronto, ON Canada; Department of Otolaryngology, Head and Neck Surgery, University of Toronto, Toronto, ON Canada.
Abstract:
Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES.
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