title: Laryngeal Manifestations of Neurofibromatosis.
abstract: OBJECTIVE
  To describe the range of findings in patients with neurofibromatosis (NF) presenting to a laryngology clinic and to analyze the etiologic factors of vocal fold dysfunction in this cohort.
  
  
  METHODS
  Case series with chart review.
  
  
  METHODS
  Tertiary laryngology practice.
  
  
  METHODS
  All cases of NF presenting to an academic laryngology practice were retrospectively reviewed (August 2005 to May 2014), with a total of 34 cases. Demographic data, symptoms, and endoscopic examination findings were reviewed. Etiologic factors of laryngeal complaints were analyzed with reference to NF-associated pathologies and surgical history.
  
  
  RESULTS
  Thirty-four patients with NF-1 or NF-2 were evaluated, and 28 of these patients (6 NF-1 and 22 NF-2) had laryngeal pathology. The most common presenting symptoms were vocal weakness (n = 21), dysphagia (n = 5), and globus (n = 4). Three patients had NF-related vocal fold masses on examination, including 2 neurofibromas and 1 schwannoma. Unilateral vocal cord paralysis was seen in 17 patients; bilateral paralysis was observed in 5 patients. Of patients with unilateral or bilateral paralysis, 20 had intracranial masses (vestibular schwannoma, meningioma, or skull base tumors), and 16 had previously undergone surgery for these lesions. Of the patients with NF-associated intracranial tumors, 87.0% presented with vocal cord paralysis, whereas only 40.0% of those without intracranial masses had paralysis (P = .0560). Seven patients underwent medialization procedures.
  
  
  CONCLUSIONS
  Neurofibromatosis patients may present to laryngology clinic with primary laryngeal tumors or, more commonly, unilateral or bilateral paralysis. Otolaryngologists should be keenly aware of vocal fold paralysis caused by the NF-associated tumors, with particular attention to bilateral paralysis in NF-2.
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Matthew_Naunheim@meei.harvard.edu.
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Naunheim
authors.affiliation: Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Scott R
authors.initials: SR
authors.lastname: Plotkin
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Ramon A
authors.initials: RA
authors.lastname: Franco
authors.affiliation: Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Phillip C
authors.initials: PC
authors.lastname: Song
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology* / Q000601:surgery*; D007828:Laryngoscopy; D008297:Male; D008875:Middle Aged; D017253:Neurofibromatoses / Q000150:complications*; D012189:Retrospective Studies
keywords: NF-1; NF-2; neurofibroma; neurofibromatosis; vocal cord paralysis