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Hearing changes after diagnosis in neurofibromatosis type 2
Ann Masuda1, Laurel M Fisher, Mark L Oppenheimer
1Clinical Studies Department, House Ear Institute, Los Angeles, California 90057, USA. amasuda@hei.org
Summary
Patients with neurofibromatosis Type 2 (NF2) often maintain stable hearing in untreated ears for 1-2 years post-diagnosis. This study tracked hearing changes in NF2 patients with vestibular schwannomas.
Area of Science:
- Otolaryngology
- Neurology
- Genetics
Background:
- Neurofibromatosis Type 2 (NF2) is a genetic disorder characterized by the development of tumors, primarily vestibular schwannomas, which can impact hearing.
- Understanding the natural history of hearing loss in NF2 is crucial for patient management and treatment planning.
Purpose of the Study:
- To describe hearing ability changes and disease progression over time in individuals with NF2.
- To analyze audiological data from a multicenter natural history study of vestibular schwannomas in NF2 patients.
Main Methods:
- A retrospective clinical study was conducted across international NF2 tertiary care centers.
- Sixty-three subjects (108 ears) with NF2, at least one untreated vestibular schwannoma, and audiology data at short-term (7 mo-2 yr) or long-term (3-5 yr) follow-up were analyzed.
- Changes in four-frequency pure-tone average and speech discrimination scores were assessed before any treatment intervention.
Main Results:
- Within two years of NF2 diagnosis, 27% of ears showed a significant decline in pure-tone average.
- Conversely, 73% of ears demonstrated no significant change in hearing during the same period.
Conclusions:
- Newly diagnosed NF2 patients without immediate need for bilateral vestibular schwannoma treatment are likely to experience stable hearing in their unoperated ear(s) for approximately 1 to 2 years.
- This suggests a window of observation for hearing preservation in select NF2 patients.