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Published on: December 1, 2023
Voice and Swallowing Dysfunction in Neurofibromatosis 2
Simon R Best1, Julie Ahn1, Shannon Langmead2
11 Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
Neurofibromatosis 2 (NF2) patients often experience significant voice and swallowing problems, frequently linked to cerebellopontine angle (CPA) surgery. These impairments substantially impact their quality of life.
Area of Science:
- Neuro-oncology
- Otolaryngology
- Neurology
Background:
- Neurofibromatosis 2 (NF2) is a genetic disorder characterized by bilateral vestibular schwannomas in the cerebellopontine angle (CPA).
- Vagal nerve involvement or surgical resection of CPA tumors can lead to voice and swallowing difficulties.
Purpose of the Study:
- To determine the prevalence of voice and swallowing impairments in NF2 patients.
- To analyze the impact of vagal dysfunction on these impairments and quality of life.
Main Methods:
- Cross-sectional study at an academic tertiary care center.
- Surveys (Voice Handicap Index, Sydney Swallow Questionnaire) and clinical evaluations (stroboscopy, voice, swallowing assessments) were conducted.
- Patients with NF2 were recruited from a specialized neurofibromatosis center.
Main Results:
- High rates of self-reported and objective voice (35%) and swallowing (50%) handicaps were found.
- Vocal fold motion impairment (VFMI) affected 71% of examined patients.
- Significant associations were observed between VFMI, CPA surgery, and reduced quality of life due to voice and swallowing issues.
Conclusions:
- Voice and swallowing impairments are highly prevalent in NF2 patients.
- These impairments significantly affect quality of life.
- CPA surgical interventions are a primary cause of these functional deficits.
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