title: Langerhans' cell histiocytosis of the labyrinth in adults.
abstract: OBJECTIVE
  To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  A tertiary referral center.
  
  
  METHODS
  Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone.
  
  
  METHODS
  Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy.
  
  
  RESULTS
  The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred.
  
  
  CONCLUSIONS
  Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.
authors.affiliation: Department of Otolaryngology, Hôpital Louis Mourier, AP-HP, Faculté Xavier Bichat, Université Parist, Colombes, France. isabelle.mosnier@lmr.ap-hop-paris.fr
authors.forename: Isabelle
authors.initials: I
authors.lastname: Mosnier
authors.forename: E
authors.initials: E
authors.lastname: Rondini-Gilli
authors.forename: Paulo Tormin Borges
authors.initials: PT
authors.lastname: Crosara
authors.forename: N
authors.initials: N
authors.lastname: Belmatoug
authors.forename: Françoise
authors.initials: F
authors.lastname: Cyna-Gorse
authors.forename: D
authors.initials: D
authors.lastname: Cazals-Hatem
authors.forename: A
authors.initials: A
authors.lastname: Abbey-Toby
authors.forename: A
authors.initials: A
authors.lastname: Bozorg-Grayeli
authors.forename: Olivier
authors.initials: O
authors.lastname: Sterkers
mesh_terms: D000328:Adult; D007758:Ear, Inner / Q000473:pathology*; D004712:Endolymphatic Sac / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D007801:Langerhans Cells / Q000276:immunology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D015837:Vestibular Diseases / Q000209:etiology*