Longitudinal Performance of Cochlear Implants in Neurofibromatosis Type 2

Abstract

ObjectiveCochlear implants (CIs) are a well‐established treatment modality for hearing loss due to neurofibromatosis type 2 (NF2). Our aim is to investigate variables that affect longitudinal performance of CIs among patients with NF2.Study DesignRetrospective review at a single academic institution consisting of patients who have received cochlear implants following hearing loss due to NF2.MethodsThe primary outcome examined was CI disuse or explantation. Associated clinical and surgical variables were analyzed using descriptive statistics. These included postoperative pure tone average (PTA) at 500, 1000, and 2000 Hz, tumor size, previous surgery, and comorbid depression.ResultsA total of 12 patients and 14 cochlear implants received at our institution from 2001 to 2022 were included. Notably, 35.7% of CIs (5 out of 14 cases) resulted in disuse or explantation. The average interval until explant was 9.4 years (range 3–14 years). In explanted CI cases, 20% had previous surgery and 80% had a diagnosis of comorbid depression as compared to 22.2% and 22.2%, respectively, in intact CI cases. Maximum tumor diameter was the only variable found to impact CI usage outcome (p = 0.028). Long‐term data showed that on average, patients benefit from 13.85 years of CI utility and a maximum PTA improvement of 45.0 ± 29.0 dB.ConclusionDespite the recurrent nature of NF2, patients continue to receive audiological benefit from cochlear implants. We found that larger tumor size may be associated with longitudinal CI failure.Level of EvidenceLevel 4 Laryngoscope, 2023

Cochlear Implantation in Neurofibromatosis Type 2‐Related Schwannomatosis: Long‐Term Hearing Outcomes

“…55 Explantation of the CI may be considered if it no longer provides auditory benefits, particularly if it causes discomfort, or to prevent MRI artifacts and magnet displacement. 56 ABI should be considered in cases of CI failure and in patients with nonfunctioning cochlear nerves. 57 Indeed, unpublished data from our center showed that (57%) of the 58 NF2 patients with ABI, made use of their implants and that 7 (12%) had a speech intelligibility score with ABI alone ≥40%.…”