Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome

- The ear is prepped and draped in a standard fashion that includes utilization of facial nerve monitoring and pre-incision antibiotics. 1. Modified Rambo meatoplasty for closure of the external auditory canal. 2. Postauricular incision for access to the external auditory canal. Remove all canal skin and tympanic membrane to prevent future cholesteatoma formation. 3. Canaloplasty to ensure canal skin removal and increase exposure to the middle ear. 4. Identify the facial nerve course in the middle ear. The nerve stimulating probe can help to map it out. 5. Cochleostomy is preferably at the round window if it can be identified. In the case of round window atresia, its expected location or a safe location away from the mapped course of the facial nerve is selected as the cochleostomy site. 6. The internal device is secured in a standard fashion. The electrode is implanted and the electrode array can be positioned in a shallow suprameatal trough. Any excess electrode is coiled and secured within the expanded external auditory canal.

Standard age and device appropriate cochlear implant candidacy applies. This technique is consider in cases of severe middle or inner ear malformation, such as those associated with CHARGE syndrome.

Absolute: Absence of a cochlear nerve. This is particularly relevant in patients with CHARGE syndrome as they have a higher rate of cranial nerve eight aplasia. Relative: This is not the preferred implantation technique if normal mastoid, middle ear, and inner ear anatomy is present.

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Standard operative room setup for cochlear implantation including facial nerve monitoring, betadine skin prep, pre-incision antibiotics, and antibiotic irrigation.

Preoperative evaluation consisted of computed tomography (CT), magnetic resonance imaging (MRI), audiometry, cochlear implant evaluation, and cognitive assessment. All patients underwent unilateral implantation after MRI confirmed an intact cochlear nerve on that side.

The typical anatomical landmarks for ear surgery are often absent or severely distorted. For more information on CHARGE syndrome and its anatomical variations, we suggest reviewing the references listed below.

- Bleeding, infection, facial nerve injury, CSF leak/gusher, meningitis, improper electrode placement, electrode extrusion. - Cochlear implant outcomes are still multifactorial and vary widely in this patient population based on their other medical co-morbidities, developmental delay, supportive environment, and other factors known to influence cochlear implant performance.

- Bleeding, infection, facial nerve injury, CSF leak/gusher, meningitis, improper electrode placement, electrode extrusion. - Cochlear implant outcomes are still multifactorial and vary widely in this patient population based on their other medical co-morbidities, developmental delay, supportive environment, and other factors known to influence cochlear implant performance.

1. Vesseur AC, Verbist BM, Westerlaan HE, et al. CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery. Eur Arch Otorhinolaryngol 2016;273:4225-4240.
2. Morimoto AK, Wiggins RH 3rd, Hudgins PA, et al. Absent semicircular canals in CHARGE syndrome: radiologic spectrum of findings. ANJR Am J Neuroradiol 2006;27:1663-1671.
3. Zentner GE, Layman WS, Martin DM, Scacheri PC. Molecular and phenotypic aspects of CHD7 mutation in CHARGE syndrome. Am J Med Genet A 2010;152A:674-686.
4. Buchman CA, Copeland BJ, Yu KK, Brown CJ, Carrasco VN, Pillsbury HC. Cochlear implantation in children with congenital inner ear malformations. Laryngoscope 2004;114:309-316.