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Hearing Changes after Diagnosis in Neurofibromatosis Type 2

Masuda, Ann*; Fisher, Laurel M.*; Oppenheimer, Mark L.; Iqbal, Zarina*; Slattery, William H.*‡for the Natural History Consortium

Tumors Of The Ear & Cranial Base

Objective: Describe the changes in hearing ability and progression of disease over time in subjects with neurofibromatosis Type 2 enrolled in a multicenter natural history study of vestibular schwannomas in neurofibromatosis Type 2.

Study Design: Retrospective clinical study.

Setting: International neurofibromatosis Type 2 tertiary care centers.

Patients: Study participants had a clinical diagnosis of neurofibromatosis Type 2, at least one untreated vestibular schwannoma, and were at least 5 years old. Sixty-three subjects (108 ears) with audiology data at either short-term follow-up (7 mo–2 yr) or long-term follow-up (3–5 yr) after diagnosis were examined in this study.

Main Outcome Measures: Changes in four-frequency pure-tone average and speech discrimination score before any treatment intervention for both follow-up intervals.

Results: Within 2 years of the diagnosis of neurofibromatosis Type 2, 27% of the ears experienced a significant loss in pure-tone average relative to diagnosis, and 73% of the ears experienced no significant change in hearing.

Conclusion: Newly diagnosed neurofibromatosis Type 2 patients who do not require immediate treatment of both vestibular schwannomas are likely to have stable hearing in the unoperated ear(s) for approximately 1 to 2 years.

*Clinical Studies Department, House Ear Institute, Los Angeles, California, U.S.A., †Oregon Health Sciences University, Portland, Oregon, U.S.A., and ‡House Ear Clinic, Los Angeles, California, U.S.A.

This research was funded by Grant DAMD 17-98-8612 from the Department of Defense. The data were collected under the auspices of the NF2 Natural History Consortium.

Address correspondence and reprint requests to Ann Masuda, M.S., Clinical Studies Department, House Ear Institute, 2100 West Third Street, 5th Floor, Los Angeles, CA 90057, U.S.A.; Email:

© 2004 Otology & Neurotology, Inc.