Reliability of functional outcome measures in adults with neurofibromatosis 2

OBJECTIVE: To determine inter- and intra-rater reliability of functional performance outcome measures in people with neurofibromatosis 2. To ascertain how closely objective and subjective measures align. METHODS: Twenty-nine people with neurofibromatosis 2 were recorded performing the modified clinical test of sensory integration and balance, four square step test and modified nine-hole peg tests. Three raters scored each measure to determine inter-rater reliability. One rater scored the measures a second time to determine intra-rater reliability. Participants also completed a disease-specific quality of life questionnaire and dynamic visual acuity testing. RESULTS: Inter-rater and intra-rater reliability scores (intra-class correlation coefficient) were excellent for all tests (intra-class correlation coefficient r ⩾ 0.9). The four square step test correlated with perceived walking challenges and modified clinical test of sensory integration and balance correlated with perceived balance challenges in a neurofibromatosis 2 quality of life patient report outcome measure. CONCLUSION: The modified clinical test of sensory integration and balance, four square step test and modified nine-hole peg tests are potentially useful measures for monitoring neurofibromatosis 2.