What factors influence implant success in patients with neurofibromatosis type 2 (NF2) who have undergone both cochlear implantation and auditory brainstem implantation (ABI) for hearing rehabilitation?
If the cochlear nerve is intact, cochlear implantation can be effective for hearing rehabilitation in NF2 patients; however, a significant proportion experience a decline in CI performance. ABI remains the standard option for surgical hearing rehabilitation in NF2, but peak performance is generally lower than that achievable with CIs.
Explore this issue:September 2018
Background: NF2 is an autosomal dominant hereditary disorder characterized by bilateral vestibular schwannomas in 90% to 95% of affected patients. Minimizing morbidity and maximizing the duration of useful hearing can be at odds. Unaidable hearing can be rehabilitated through an ABI, cochlear implant (CI), or both. However, device performance is highly variable and affected by patient and surgical factors.
Study design: Retrospective case series of 10 NF2 patients with both cochlear implantations and auditory brainstem implantations.