Related Experiment Video
Updated: Jul 16, 2025
06:04
Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
419
Longitudinal Performance of Cochlear Implants in Neurofibromatosis Type 2
Stefanie Seo1, Sarek Shen2, Andy S Ding2
1Johns Hopkins School of Medicine, Baltimore, Maryland, U.S.A.
The Laryngoscope
|September 12, 2023
Summary
Cochlear implants (CIs) offer hearing benefits for neurofibromatosis type 2 (NF2) patients. Larger tumor size may increase the risk of CI failure over time.
Area of Science:
- Otolaryngology
- Neurosurgery
- Genetics
Background:
- Neurofibromatosis type 2 (NF2) is a genetic disorder leading to tumors, often causing hearing loss.
- Cochlear implants (CIs) are a standard treatment for hearing loss in NF2 patients.
Purpose of the Study:
- To investigate factors influencing the long-term performance of cochlear implants (CIs) in patients with neurofibromatosis type 2 (NF2).
Main Methods:
- Retrospective review of patients with NF2 who received CIs.
- Analysis of clinical and surgical variables, including postoperative pure tone average (PTA), tumor size, prior surgery, and depression.
- Primary outcome: CI disuse or explantation.
Main Results:
- Of 14 CIs in 12 patients, 35.7% were disused or explanted, with a mean explantation interval of 9.4 years.
- Larger tumor diameter was significantly associated with CI usage outcomes (p=0.028).
- Patients experienced an average of 13.85 years of CI utility and significant PTA improvement.
Conclusions:
- Cochlear implants provide lasting audiological benefits for NF2 patients despite the condition's recurrent nature.
- Increased tumor size appears to be a risk factor for longitudinal CI failure in NF2 patients.
