Transcutaneous Auricular Vagus Nerve Stimulation in a Pacemaker-Dependent Child with Syndromic Drug-Resistant Epilepsy: A Case Report with Prospective 9-Month Follow-Up.

Abstract

Introduction: Transcutaneous auricular vagus nerve stimulation (taVNS) is an emerging noninvasive neuromodulation therapy for drug-resistant epilepsy (DRE). However, evidence regarding its use in patients with cardiac implantable electronic devices, particularly pacemaker-dependent children, remains extremely limited.

Case presentation: We report a 6-year-old boy with genetically confirmed TAF6-related Alazami-Yuan syndrome, severe developmental impairment, complete atrioventricular block requiring permanent epicardial VVIR pacing, and syndromic DRE refractory to multiple antiseizure medications (ASMs). Auricular taVNS was initiated at the cymba conchae under multidisciplinary supervision using stimulation parameters of 25 Hz, pulse width 250 μs, intensity titrated to tolerance up to 1.7 mA, for 4 h/day. Treatment initiation was performed in the pediatric intensive care unit with continuous electrocardiographic monitoring for 5 days. The patient underwent prospective follow-up over 9 months using caregiver-maintained seizure diaries, serial electroencephalography (EEG), serial electrocardiography, repeated pacemaker interrogation, adverse event surveillance, and Pediatric Quality of Life Family Impact Module (PedsQL-FIM) assessments. No arrhythmias, pacing abnormalities, device malfunction, or clinically significant pacemaker parameter changes were observed during follow-up. A mild transient local skin irritation resolved with conservative care. Seizure burden decreased from 2 to 4 prolonged seizures daily at baseline to approximately two brief generalized tonic seizures weekly, with sustained improvement through month 9. Reduction of ASM burden began only after clinical improvement had already been observed. Follow-up EEGs remained markedly abnormal with only limited interval electrographic change. PedsQL-FIM improved progressively from 10.4 at baseline to 54.9 at month 3, 80.6 at month 7, and 84.0 at month 9.

Conclusion: Auricular taVNS was successfully administered in a pacemaker-dependent child under structured multidisciplinary monitoring without identifiable cardiac or pacemaker-related adverse events during 9 months of follow-up. Although efficacy and safety conclusions cannot be inferred from a single case, this report supports the clinical feasibility of combining noninvasive vagal neuromodulation with permanent cardiac pacing in carefully selected pediatric patients.