Voice-related quality of life in adults with neurofibromatosis type 1

Abstract

Objective: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder, which occurs in about one in 3000-4000 people. Its hallmark features include multiple café-au-lait spots and neurofibromas. Voice characteristics of NF1 patients have been documented using both subjective and objective evaluations. However, the relative impact of these voice characteristics on daily activities has, as far as we know, not been examined yet.

Methods: Thirty adults with NF1 were asked to complete the Flemish Dutch version of the Voice Handicap Index (VHI). Additionally, the level of severity of NF1 was rated and Dysphonia Severity Indices (DSIs) were collected. The results of the patient group were compared with those of a healthy control group frequency matched for age, gender, smoking behavior, and vocal usage.

Results: NF1 patients obtained significantly higher VHI scores compared with healthy controls. Differences between the patient and control group were especially marked for daily functioning and affective responses. Men and women with NF1 had similar VHI scores, but total VHI score did increase with age. An apparent association between VHI, DSI, and severity of NF1 could not be demonstrated.

Conclusion: It is likely that the elevated VHI scores observed in the patient group were not merely caused by the voice characteristics associated with NF1. A flow over from other psychophysical issues relating to the disease might have played a role.