The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome

Cameron C. Wick, Amy M. Moore, Daniel E. Killeen, Brandon Isaacson

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Objective: CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. Patients: Three children with profound hearing loss secondary to CHARGE syndrome. Intervention: Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. Main Outcome Measure(s): Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. Results: The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. Conclusions: The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Original languageEnglish (US)
Pages (from-to)1268-1272
Number of pages5
JournalOtology and Neurotology
Volume38
Issue number9
DOIs
StatePublished - Oct 1 2017

Fingerprint

CHARGE Syndrome
Cochlear Implantation
Cochlear Implants
Temporal Bone
Facial Nerve
Semicircular Canals
Ear Canal
Mastoid
Cochlea
Intraoperative Complications
Deafness
Middle Ear
Hearing Loss
Population
Ear
Anatomy
Electrodes
Outcome Assessment (Health Care)
Equipment and Supplies

Keywords

  • CHARGE
  • Cochlear implant
  • Inner ear malformation
  • Otology
  • Pediatric

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome. / Wick, Cameron C.; Moore, Amy M.; Killeen, Daniel E.; Isaacson, Brandon.

In: Otology and Neurotology, Vol. 38, No. 9, 01.10.2017, p. 1268-1272.

Research output: Contribution to journalArticle

Wick, Cameron C. ; Moore, Amy M. ; Killeen, Daniel E. ; Isaacson, Brandon. / The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome. In: Otology and Neurotology. 2017 ; Vol. 38, No. 9. pp. 1268-1272.
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