Cochlear Implantation in Patients with Neurofibromatosis Type 2 and Patients with Vestibular Schwannoma in the Only Hearing Ear

Abstract

Cochlear implants are a new surgical option in the hearing rehabilitation of patients with neurofibromatosis type 2 (NF2) and patients with vestibular schwannoma (VS) in the only hearing ear. Auditory brainstem implant (ABI) has been the standard surgical treatment for these patients. We performed a literature review of patients with NF2 and patients with VS in the only hearing ear. Cochlear implantation (CI) provided some auditory benefit in all patients. Preservation of cochlear nerve integrity is crucial after VS resection. Results ranged from environmental sound awareness to excellent benefit with telephone use. Promontory stimulation is recommended although not crucial. MRI can be performed safely in cochlear implanted patients.

Face Validity Study of an Artificial Temporal Bone for Simulation Surgery

“…ABI surgery carries higher surgical risks and morbidity (20), and in comparison, CI in many centers involves day case surgery (5). Moreover, having a CI does not preclude the opportunity for future ABI placement if required.…”

Vestibular Schwannoma Resection with Ipsilateral Simultaneous Cochlear Implantation in Patients with Normal Contralateral Hearing

“…Even with hearing preservation surgery, the chances of maintaining social serviceable hearing in the short term range from 0 to 37% in the most experienced hands [Rabelo de Freitas et al, 2012]. Patients with neurofibromatosis type 2 (NF-2) or VS in the only or better hearing ear deserve separate consideration, as they are at special risk of developing bilateral anacusia, either as a consequence of treatment or from the natural course of the disease [Vincenti et al, 2008;Celis-Aguilar et al, 2012;Lassaletta et al, 2016].…”

Cochlear implantation in patients with vestibular schwannoma

“…In a study by Vincenti et al, patients with NF2 performed better with CI than ABI, although the outcome was variable in both groups. Two recently published review articles on the subject reached a similar conclusion . With CI becoming the favored option, there is increasing emphasis placed on preservation of the cochlear nerve, where possible, during surgical resection.…”

“…Of late, there has been a growing interest in cochlear implantation (CI) as an alternative option for auditory rehabilitation in patients with VS who have residual cochlear nerve function. Although reported audiologic outcomes are variable, a recent review by Celis‐Aguilar et al suggests that CI is a reasonable option for these patients …”