title: Histopathologic markers of hearing loss in intralabyrinthine schwannomas:  implications for management.
abstract: OBJECTIVE
  This study aimed to review the clinical history and management of patients with intralabyrinthine schwannomas and evaluate the histopathologic features of these tumors.
  
  
  METHODS
  A retrospective case review of 24 patients and 7 temporal bone specimens.
  
  
  METHODS
  An otologic tertiary referral private practice.
  
  
  METHODS
  Patients with magnetic resonance imaging-confirmed intralabyrinthine schwannoma diagnosed after 1995 and non-Neurofibromatosis type 2 temporal bone specimens with schwannomas confined to the bony labyrinth.
  
  
  METHODS
  Clinical data including history, imaging and audiometry were evaluated. The histopathologic features of temporal bone specimens including tumor location and degenerative changes were analyzed.
  
  
  RESULTS
  The intralabyrinthine location of the tumor does not predict the degree or time to progression of hearing loss. Both sensorineural and conductive hearing loss can occur. Mechanisms of hearing loss include hair cell, spiral ganglion, and stria vascularis degeneration in addition to mechanical obstruction.
  
  
  CONCLUSIONS
  Hearing loss is the most common symptom with intralabyrinthine schwannomas. Histopathology reveals that multiple mechanisms of degeneration may occur within the labyrinth to induce hearing loss. Observation provides the most reliable hearing preservation strategy.
authors.affiliation: House Clinic, House Ear Institute, Los Angeles, California 90057, USA. santos.neurotology@gmail.com
authors.forename: Felipe
authors.initials: F
authors.lastname: Santos
authors.forename: Fred H
authors.initials: FH
authors.lastname: Linthicum
authors.forename: John W
authors.initials: JW
authors.lastname: House
authors.forename: Eric P
authors.initials: EP
authors.lastname: Wilkinson
mesh_terms: D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D004428:Ear Neoplasms / Q000150:complications / Q000473:pathology*; D005260:Female; D034381:Hearing Loss / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007759:Labyrinth Diseases / Q000150:complications / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009442:Neurilemmoma / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies; D013701:Temporal Bone / Q000473:pathology