Abstract
To explore health-related quality of life (HRQoL) reported by individuals with neurofibromatosis type 2 (NF2) and to assess for correlations between HRQoL and objective measures of disease manifestations. Prospective observational study. Seven international NF2 centers. Eighty-one individuals with NF2, 73 adults (>18 years) and 8 children/adolescents (10-17 years). Quality of life was measured by Short Form-36 (SF-36) norm-based scores. Objective clinical measures were hearing (categorized by word-recognition scores), facial function (categorized by the House-Brackmann scale) and the volume of subjects' larger vestibular schwannoma (VS). At baseline, adults showed significant deficits in all but two subscales of the SF-36 compared with age- and gender-adjusted United States population norms. In linear regression models including age, gender, inheritance status, hearing, facial weakness and VS volume, demographic and functional measures showed no relationship to any SF-36 subscale. Larger baseline VS volume was significantly related to reduced physical role performance, reduced mental health, and increased pain (p < 0.05). In bivariate analysis, previous VS surgery was not significantly associated with baseline HRQoL; receipt of VS surgery or tumor growth during the observation period was not significantly associated with changes in HRQoL. NF2 patients report reduced HRQoL in physical, social, and mental domains, but this was not significantly related to objective measures of hearing or facial functioning. Larger baseline VS volume negatively impacted patient-reported HRQoL whereas VS surgery or tumor growth did not. Future studies should explore the relationship between tumor volume and HRQoL and psychosocial factors that may moderate this relationship.
Highlights
Neurofibromatosis type 2 (NF2) is a neurogenetic tumor suppressor syndrome, with an estimated birth incidence of 1:25,000.(1) neurofibromatosis type 2 (NF2) is characterized by the development of bilateral vestibular schwannomas (VS), as well as schwannomas of other cranial nerves, meningiomas, and spinal ependymomas.[2]
While prior studies have documented an association between patient reported disease characteristics and health-related quality of life (HRQoL) in patients with NF2, it is unclear whether HRQoL correlates with clinically observed disease manifestations in this population.[3,4,5,6] far, demographic and clinical factors that include age, gender, total peripheral tumor volume, and presence of nonvestibular schwannomas and meningiomas have not been found to be associated with HRQoL.[6;7] the relationship between VS volume and HRQoL has not been assessed in patients with NF2
Radiographic measurements, audiometry, and House-Brackmann ratings of facial function are used by clinicians to objectively assess disease severity in patients with NF2
Summary
“Health-Related Quality of Life of Individuals With Neurofibromatosis Type 2.”. Health related quality of life of individuals with neurofibromatosis type 2: Results from the NF2 Natural History Study Running head: Quality of life in NF2 Vanessa L. MS CGC2, Ana-Maria Vranceanu, PhD3, Alona Muzikansky, MA4, William Slattery, III, MD5, Scott R. 1. Department of Neurology and Cancer Center, Massachusetts General Hospital, Boston, MA. 2. Department of Neurology, Johns Hopkins Hospital, Baltimore, MD, USA 3.
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