INTRODUCTION: In our previous report, we described a surgical technique for cochlear implantation in canal wall down (CWD) ears. Specifically, this involved extending the endaural incision superiorly, harvesting a large temporal fascia flap, completely removing the tympanic epithelium, inserting the electrode array, obliterating the middle ear cavity with the temporal fascia flap, and sealing the external auditory canal with a tragal cartilage plug. This report details the safe application of this technique in a patient with severe ear malformation and suspected CHARGE syndrome, supplemented with a brief literature review. CASE PRESENTATION: The patient was a 28-year-old woman with malformations of the auricle, middle ear, and inner ear, as well as patent ductus arteriosus, amblyopia, and mild intellectual disability.Temporal bone CT revealed significant inferior displacement of the emissary vein, precluding standard electrode insertion via the posterior tympanotomy approach to the round window. We therefore applied our previously reported technique to this malformed ear without prior surgical history. Sequential bilateral implantation was performed. METHODS AND RESULTS: Surgical duration was 96 minutes (right) and 167 minutes (left). Wide bony exposure via CWD facilitated uneventful electrode insertion bilaterally, with minimal bleeding. Postoperative sound-field aided thresholds were favorable at 35 dB (right) and 38.3 dB (left). Speech discrimination scores were 90% at 60 dB (right) and 0% (left). CONCLUSION: The cochlear implantation technique for CWD ears was safely adapted to this patient with congenital malformation. This approach represents a viable surgical option for cases with severe malformations such as CHARGE syndrome.