Establishment of nomograms for the prediction of useful hearing loss in patients with neurofibromatosis type 2.

Introduction: Treatment for vestibular schwannoma (VS) in patients with neurofibromatosis type 2 (NF2) is extremely challenging due to the high risk of hearing loss. The aim of this study was to develop nomograms for the prediction of useful hearing loss in patients with NF2.

Methods: The nomogram was based on a retrospective study of 111 NF2 patients who underwent resection of large VS (> 2 cm) at Beijing Tiantan Hospital between 2011 and 2018. The utility of the proposed nomogram models was evaluated by receiver operating characteristic (ROC) curve, area under ROC curve (AUC), and calibration curve. The results were validated using a prospective cohort study on 33 patients consecutively enrolled at the same institution from 2019 to 2021.

Results: On multivariate analysis of the primary cohort, large tumour size (> 3 cm) and long duration of symptoms (> 24 months) were independent risk factors for preoperative useful hearing loss (AAO-HNS Class D) (P = 0.001 and P = 0.011, respectively), while large tumour size (> 3 cm), poor hearing (Class C), and lobular growth were significantly related to postoperative useful hearing loss (P < 0.001, P = 0.031 and P = 0.033, respectively). Factors derived from multivariable analysis were all assembled into the nomogram. The calibration curve for probability of hearing loss showed good agreement between predictions by nomogram models and actual observation. The ROC curves showed good predictive accuracy of the nomogram models in both cohorts (AUC: 0.708 to 0.951).

Conclusion: The proposed nomograms resulted in accurate predictions of hearing outcomes for patients with NF2.