Objective: The goals of this study are to report the clinical presentation, radiographic findings,
operative strategy, and outcomes in two patients (three ears) who underwent cochlear
implantation post temporal bone encephalocele repair and to identify surgical challenges
and report strategies associated with the final outcome.
Patients: Two patients (one patient with bilateral encephaloceles and second patient with unilateral
encephalocele) underwent a middle fossa craniotomy and a combined mastoid-middle cranial
fossa repair of encephalocele respectively at 2 tertiary academic referral centers.
The first patient underwent bilateral cochlear implantation while the second patient
underwent unilateral cochlear implantation on the side with encephalocele repair.
Results: The initial presenting symptom in both patients was bilateral severe to profound
hearing loss. In one case the hearing loss was a result of an explosion while in the
other case, it followed chronic ear disease without prior surgeries. Both patients
did not report a history of intracranial infections or seizures. One patient (JD)
underwent a middle fossa craniotomy for encephalocele repair, while the other patient
(JW) underwent a combined postaural mastoid-middle fossa repair. Four months after
successful encephalocele repair, both patients underwent cochlear implantation with
Cochlear 522 electrodes via a round window approach. The patient with bilateral encephaloceles
underwent second side cochlear implant surgery later that same year. There were no
intraoperative or postoperative complications. Activation of cochlear implants in
both patients was done 6 weeks after cochlear implant surgery and was uneventful.
Conclusion: Cochlear implantation post encephalocele repair can be challenging due to the increased
risk of bleeding due to scarring from previous surgery, alteration in anatomical landmarks,
contracted mastoid cavity following the dural repair and the presence of titanium
mesh at the craniotomy site. Advance planning for management of these patients and
intraoperative strategies can help overcome the surgical challenges and improve outcomes.
This is the first such report of successful cochlear implantation in patients post
encephalocele repair.