Cochlear Implantation Via Extended Endaural Incision in a Patient With Congenital Ear Malformation.

Area of Science:

• Otolaryngology
• Neurosurgery
• Medical Engineering

Background:

• A previously reported surgical technique for cochlear implantation in canal wall down (CWD) ears is detailed.
• This technique involves specific steps including flap harvesting, tympanic epithelium removal, electrode insertion, middle ear obliteration, and ear canal sealing.

Purpose of the Study:

• To describe the safe application of the CWD cochlear implantation technique in a patient with severe congenital ear malformation and suspected CHARGE syndrome.
• To supplement the case report with a brief literature review.

Main Methods:

• The technique was applied to a 28-year-old female patient with complex auricle, middle ear, and inner ear malformations.
• Temporal bone CT revealed anatomical challenges, necessitating the use of the modified CWD approach for electrode insertion.
• Sequential bilateral cochlear implantation was performed using the described technique.

Main Results:

• Bilateral electrode insertion was successfully achieved via wide bony exposure with minimal bleeding.
• Postoperative sound-field aided thresholds were favorable (35 dB right, 38.3 dB left).
• Speech discrimination scores showed significant variation (90% right, 0% left).

Conclusions:

• The described cochlear implantation technique for CWD ears was safely adapted for a patient with congenital malformations.
• This surgical approach is a viable option for complex cases, including those with CHARGE syndrome.