Audiovestibular Characteristics of Small Cochleovestibular Schwannomas in Neurofibromatosis Type 2

Abstract

Objective

Describe the relationship between cochleovestibular schwannoma (CVS) volume, audiovestibular characteristics, and magnetic resonance imaging (MRI) findings in patients with neurofibromatosis type 2 (NF2).

Study Design

Subgroup analysis of NF2 prospective natural history study from 2008 to 2011.

Setting

Quaternary medical research institute.

Subjects and Methods

NF2 patients with small treatment-naive CVSs (volume <1000 mm³) by ear; N = 49 ears (32 patients). Cross-sectional analysis of the following parameters was performed: tumor size, auditory brainstem response (ABR), 4-frequency pure-tone average (4f-PTA; 0.5, 1, 2, and 4KHz), cervical vestibular evoked myogenic potential (cVEMP), caloric testing, 240° velocity step test (VST), and MRI findings.

Results

For all physiologic measures but the 4f-PTA, larger tumors correlated with abnormal responses (P < .05). For abnormal ABR, mean tumor volume was 405 vs 151 mm³ (P = .0007) for normal ABR. Similarly, larger tumors correlated with weak caloric responses (mean 521 vs 165 mm³; P = .0007) and weak cVEMP (mean 357 vs 192 mm³; P = .0262). Tumor volume was not significantly correlated with 4f-PTA. Elevated intralabyrinthine protein on MRI fluid-attenuated inversion recovery sequences was correlated with larger tumor volume (mean 333 vs 55 mm³; P = .001) and abnormal ABR and 4f-PTA (P < .05) but did not correlate with cVEMP, VST, or caloric responses.

Conclusion

In our cohort, ABR, caloric response, cVEMP, and elevated intralabyrinthine protein correlated with tumor volume, but 4f-PTA did not. Abnormal ABR and 4f-PTA correlated with elevated intralabyrinthine protein. These findings may provide insight on the effect of small CVS on the inner ear and cochleovestibular nerves, which may aid in their optimal management.