Hearing changes after diagnosis in neurofibromatosis type 2.
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.


METHODS
Retrospective clinical study.


METHODS
International neurofibromatosis Type 2 tertiary care centers.


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Masuda
Ann
A
Clinical Studies Department, House Ear Institute, Los Angeles, California 90057, USA. amasuda@hei.org
Fisher
Laurel M
LM
Oppenheimer
Mark L
ML
Iqbal
Zarina
Z
Slattery
William H
WH
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone / Q000379:methods; D001309:Auditory Threshold; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D034381:Hearing Loss / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications / Q000503:physiopathology*; D009464:Neuroma, Acoustic / Q000150:complications / Q000503:physiopathology*; D012044:Regression Analysis; D012189:Retrospective Studies