Outcomes of Cochlear Implantation in Patients With Jervell and Lange-Nielsen Syndrome: A Systematic Review and Narrative Synthesis
Journal of International Advanced Otology
Metcalfe, C., Muzaffar, J., et al. (2020).
Journal of International Advanced Otology, 16(3), 456-462.
This systematic review investigates the outcomes of cochlear implants in children with Jervell and Lange-Nielsen syndrome (JLNS).
No funding received
Inception to June 18, 2020
Experimental studies; observational studies
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For children with Jervell and Lange-Nielsen syndrome (JLNS), cochlear implantation provided audiological, speech perception, and speech intelligibility benefits in the majority of patients. Because JLNS is characterized by cardiac dysfunction in addition to bilateral sensorineural hearing loss, the authors also reported intraoperative cardiac observations. 12.2% of patients demonstrated a potentially fatal cardiac arrhythmia intraoperatively; however, there was no associated mortality. The authors of the included studies highlighted the necessity of preoperative counseling on risks and of the need for multidisciplinary intraoperative cardiac monitoring. Additional research with larger sample sizes and standardized pre- and post-operative audiological assessment is needed.
