Patient Report of Hearing in Neurofibromatosis Type 2: Recommendations for Clinical Trials.

OBJECTIVE: To systematically evaluate published patient-reported outcome measures for the assessment of hearing function and hearing-related quality of life (QoL) and recommend measures selected by the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration (REiNS) as endpoints for clinical trials in neurofibromatosis type 2 (NF2). METHODS: The REiNS Patient-Reported Outcomes Working Group systematically evaluated published patient-reported outcome measures of (1) hearing function and (2) hearing-related QoL for individuals with hearing loss of various etiologies using previously published REiNS rating procedures. Ten measures of hearing functioning and 11 measures of hearing-related QoL were reviewed. Measures were numerically scored and compared primarily on their participant characteristics (including participant age range and availability of normative data), item content, psychometric properties, and feasibility for use in clinical trials. RESULTS: The Self-Assessment of Communication and the Self-Assessment of Communication-Adolescent were identified as most useful for adult and pediatric populations with NF2, respectively, for the measurement of both hearing function and hearing-related QoL. Measures were selected for their strengths in participant characteristics, item content, psychometric properties, and feasibility for use in clinical trials. CONCLUSIONS: REiNS recommends the Self-Assessment of Communication adult and adolescent forms for the assessment of patient-reported hearing function and hearing-related QoL for NF2 clinical trials. Further work is needed to demonstrate the utility of these measures in evaluating pharmacologic or behavioral interventions.

The differential influence of vowels and palatal covering on nasalance scores.

Objective : To investigate whether nasalance scores would differ when the hard palate and soft palate were covered during speech. Design : Nasalance scores were obtained during production of sentence stimuli loaded with differing vowel content across three conditions: (1) hard and soft palate uncovered (U); (2) hard palate covered by acrylic appliance (H); (3) both hard and soft palate covered by acrylic appliance (B). Sentences were loaded with high front, low front, high back, low back, or mixed vowels. Velopharyngeal closure was verified by nasoendoscopy. Participants : Six female adults with normal speech and resonance provided the speech samples. Main Outcome Measures : Nasalance scores for each of five sentences in each of three palatal conditions. Results : Mixed and high front sentences had significantly higher mean nasalance scores in the U (P < .01) and H (P < .05) conditions but not in the B condition. For all sentence stimuli combined across palatal conditions, there was a significant difference in mean nasalance scores between H and B conditions (P < .05) but not between the U condition and either the H or B conditions (P > .05). Conclusions : Data suggest that the primary place of transpalatal transfer of acoustic energy was the soft palate but only sentences with high-front vowels were affected by this phenomenon.

Comparison of short-term and long-term variability in nasalance scores.

OBJECTIVE: To evaluate long-term nasalance score variability while accounting for short-term variation associated with subject performance and headgear change variability. STIMULI: Turtle and Mouse Passages. DESIGN: Short-term immediate test-retest nasalance score variability was assessed with no headgear change (NCHG) and with change of headgear (CHG). Long-term variability was assessed with scores obtained in the morning and afternoon of the same day, 1 day apart, and 1 week apart. Scores from the long-term conditions necessarily reflect variability associated with headgear change plus variability, which may be attributed to time. PARTICIPANTS: Twenty-six adults (19 to 70 years of age) with normal speech and resonance. MAIN OUTCOME MEASURES: Forty-six nasalance scores per subject. RESULTS: Mean nasalance difference scores across conditions were compared. Three contrasts were significantly different, each involving comparison of nasalance difference scores in the NCHG condition to difference scores from a CHG condition. Overall, long-term variability was slightly greater than short-term variability. For the Turtle Passage, in the short-term CHG condition, 92% of repeated scores were within five points. In the long-term conditions, 83% to 89% of scores were within five points. For the Mouse Passage, 88% of repeated scores in the CHG condition were within five points. In the long-term conditions, 81% to 83% of scores were within five points. CONCLUSIONS: Nasalance scores obtained over time showed slightly greater variability than scores obtained in immediate test-retest conditions; however, variability did not increase as the length of time between measures increased.