Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 Apr;73(2):104-11.
doi: 10.1055/s-0032-1301396.

The NFTI-QOL: A Disease-Specific Quality of Life Questionnaire for Neurofibromatosis 2

Rachael E Hornigold  1 , John F Golding, Guy Leschziner, Rupert Obholzer, Michael J Gleeson, Nick Thomas, Daniel Walsh, Shakeel Saeed, Rosalie E Ferner
Affiliations

The NFTI-QOL: A Disease-Specific Quality of Life Questionnaire for Neurofibromatosis 2

Rachael E Hornigold et al. J Neurol Surg B Skull Base. 2012 Apr.

Abstract

The objective of this study was to develop a reliable, validated disease-specific score measuring quality of life (QOL) in clinical practice and treatment trials in Neurofibromatosis 2 (NF2) individuals. In NF2 patients, qualitative interviews (n = 15) and a focus group session (n = 30) generated items for a pilot questionnaire. This was tested and refined (n = 20). The final version (NFTI-QOL) was validated (n = 50) with two generic QOL questionnaires (SF-36 and EuroQOL). The NFTI-QOL was also administered to patients with solitary vestibular schwannoma (SVS) (n = 30) and normal controls (n = 30). The participants were NF2 patients, SVS patients, and normal controls. NFTI-QOL score, SF-36 score, and EuroQOL score were the main outcome measures. Mean NFTI-QOL score was 9.4 (range: 0 to 20, maximum possible score = 24). The NFTI-QOL score correlated strongly with EuroQOL (r = 0.71, p < 0.001) and SF-36 (r = 0.81, p < 0.001). NF2 individuals were significantly worse than the SVS patients, who in turn were worse than the controls on the NIFTI-QOL. The NFTI-QOL showed good internal reliability (Cronbach's α = 0.87). We developed an eight-item disease-specific QOL score for NF2 patients, validated against SF-36 and EuroQOL. It correlated strongly with clinician-rated disease severity in NF2, with better correlation than the SF-36 in this regard.

Keywords: EuroQOL; NFTI-QOL; SF-36; neurofibromatosis 2; quality of life; vestibular schwannoma.

PubMed Disclaimer

References

    1. Evans D GR, Howard E, Giblin C. et al.Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A. 2010;152A(2):327–332. - PubMed
    1. Ferner R E. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 2007;6(4):340–351. - PubMed
    1. Baser M E, Friedman J M, Wallace A J, Ramsden R T, Joe H, Evans D G. Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology. 2002;59(11):1759–1765. - PubMed
    1. Plotkin S R, Stemmer-Rachamimov A O, Barker F G. et al.Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 2009;361(4):358–367. - PMC - PubMed
    1. Mautner V F, Nguyen R, Kutta H. et al.Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro-oncol. 2010;12(1):14–18. - PMC - PubMed