Idiopathic vocal fold paralysis in two adolescent patients with neurofibromatosis type 2

摘要

ObjectivesNeurofibromatosis 2 (NF2) is an autosomal dominant condition presenting with bilateral vestibular schwannomas and other neural tumors. Vocal fold paralysis (VFP) in NF2 patients is typically due to compression of the vagus nerve at the cerebellopontine angle (CPA) or vagal injury during tumor surgery. Idiopathic VFP in these patients has not been reported.MethodsCase series.ResultsA 15 year old female with NF2 presenting with dyspnea and dysphonia was found to have left VFP and right vocal fold paresis narrowing her glottic airway. She had no history of surgery for CPA tumors, and bilateral vestibular schwannomas were stable on imaging and not compressing the vagus nerves. Symptoms improved with voice and respiratory retraining therapy. She remains under close observation and may require surgical intervention to relieve glottic airway obstruction should her right vocal fold become immobile.A 14-year-old male with NF2 presenting with new onset dysphonia was found to have left VFP. He had undergone surgery for a right facial neuroma and right eye enucleation 7 years prior. His left CPA tumors were stable on imaging and not compressing the vagus nerve. He has undergone two in-office injection laryngoplasties and voice therapy with improvement in dysphonia. He will be a candidate for medialization thyroplasty as an adult if his right vocal fold remains mobile.ConclusionsCurrently there are no reports of idiopathic VFP in patients with NF2. Given these patients’ young age and the progressive nature of NF2, they are managed with temporary interventions and observation.