title: Clinical presentation of intralabyrinthine schwannomas: a multicenter study of 110 cases.
abstract: OBJECTIVE
  To describe the presentation of intralabyrinthine schwannomas (ILSs).
  
  
  METHODS
  Retrospective multicenter study involving 12 European skull base surgery tertiary referral centers.
  
  
  METHODS
  One hundred ten patients with the diagnosis of ILS, either labyrinth confined or extending into the internal auditory meatus for less than 50% of their volume.
  
  
  METHODS
  Data collected were age, sex, nature and timing of presenting symptoms, hearing (according to the AAO-HNS grading system), results of vestibular tests (caloric tests and cervical vestibular-evoked myogenic potentials [c-VEMPs]), and tumor localization. Presenting symptoms and laboratory test results were studied according to the extension of the lesion into the cochlea (C) and vestibule (V), on one hand, and according to unifocal (L1) or plurifocal (L2) extension into the labyrinth, on the other.
  
  
  RESULTS
  Intracochlear type was more common (50%) than vestibular (19.1%) and more diffuse forms (30.9%). The mean delay for diagnosis was long (72.5 mo; SD, 76.6). Mean age was 53.9 years (SD, 13.2). Deafness was the most common symptom (77.8 dB HL [SD, 33.6], with only 24.6% of patients keeping viable hearing. Caloric tests (65.5% of patients) were abnormal in 77.8% of cases. c-VEMPs were abnormal in 65.7% of the 36 cases analyzed. In V forms, hearing was significantly better (class A + B in 21.1% in C and 45.8% in V forms) (p = 0.03), and vestibular function was more altered (C: 57%, V: 100%, p = 0.0009*). L2 forms were diagnosed later (L1: 59.1 mo, L2: 104.5 mo; p = 0.004*) and were associated more frequently with a dead ear (L1: 13.1%, L2: 41.2%, p = 0.002*) than L1 forms.
  
  
  CONCLUSIONS
  This series, which is the largest in the literature, demonstrates that even very small and localized ILSs heavily compromise labyrinthine functions.
authors.affiliation: *Department of Otolaryngology, Reims University Hospital, Reims University, Reims, France; †Department of Otolaryngology, St Augustinus Hospital, Wilrijk, Antwerp, Belgium; ‡Department of Otorhinolaryngology-Head and Neck Surgery, ENT Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Greece; §Department of Otolaryngology, Lille University Hospital, Lille University, Lille; ∥Department of Otolaryngology and Skull Base Surgery, Pellegrin University Hospital, Bordeaux Segalen University, Bordeaux; ¶Department of Otolaryngology and Skull Base Surgery, Toulouse University Hospital, Toulouse University, Toulouse; #Department of Otolaryngology, Nantes University Hospital, Nantes University, Nantes, France; **Department of Otorhinolaryngology-Head and Neck Surgery, Kantonsspital Luzern, Luzern, Switzerland; ††Department of Otolaryngology, Tours University Hospital, Tours University, Tours; Department of Otolaryngology-Head and Neck Surgery, Fondation A. de Rothschild, Paris; §§Department of Otolaryngology, Montpellier University Hospital, Montpellier University, Montpellier; ∥∥Department of Otolaryngology, Grenoble University Hospital, Grenoble University, Grenoble; and ¶¶Department of Otolaryngology, Lyon University Hospital, Lyon University, Lyon, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Dubernard
authors.forename: Thomas
authors.initials: T
authors.lastname: Somers
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Veros
authors.forename: Christophe
authors.initials: C
authors.lastname: Vincent
authors.forename: Valérie
authors.initials: V
authors.lastname: Franco-Vidal
authors.forename: Olivier
authors.initials: O
authors.lastname: Deguine
authors.forename: Philippe
authors.initials: P
authors.lastname: Bordure
authors.forename: Thomas
authors.initials: T
authors.lastname: Linder
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Lescanne
authors.forename: Denis
authors.initials: D
authors.lastname: Ayache
authors.forename: Michel
authors.initials: M
authors.lastname: Mondain
authors.forename: Sébastien
authors.initials: S
authors.lastname: Schmerber
authors.forename: Myriam
authors.initials: M
authors.lastname: Dahmani-Causse
authors.forename: Eric
authors.initials: E
authors.lastname: Truy
authors.forename: Vincent
authors.initials: V
authors.lastname: Darrouzet
mesh_terms: D000328:Adult; D000368:Aged; D004428:Ear Neoplasms / Q000150:complications / Q000473:pathology*; D007758:Ear, Inner / Q000473:pathology; D005260:Female; D006309:Hearing; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006320:Hearing Tests; D006801:Humans; D007759:Labyrinth Diseases / Q000150:complications / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009442:Neurilemmoma / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies; D058585:Vestibular Evoked Myogenic Potentials / Q000502:physiology; D014724:Vestibular Function Tests