title: Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease.
abstract: OBJECTIVE
  Bilateral endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and often underlie significant audiovestibular morbidity, including hearing loss.
  
  
  METHODS
  This 44-year-old female von Hippel-Lindau disease patient presented with tinnitus, vertigo, and binaural hearing loss. Magnetic resonance and computed tomography imaging demonstrated bilateral ELSTs, and audiometry confirmed bilateral hearing loss.
  
  
  METHODS
  The patient underwent staged resection of the ELSTs (left then right). After resection of the left ELST and during the same operation, a cochlear implant was placed.
  
  
  METHODS
  Clinical, audiometric, and imaging data.
  
  
  RESULTS
  Postoperatively, the patient had resolution of tinnitus and vertigo with a significant implant-aided improvement in hearing.
  
  
  CONCLUSIONS
  Because of their unique anatomic and biologic features, resection of bilateral tumors and cochlear implantation in deaf ELST patients is a potential option to improve hearing and quality of life.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
authors.forename: Jay
authors.initials: J
authors.lastname: Jagannathan
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
authors.forename: Richard A
authors.initials: RA
authors.lastname: Stanger
authors.forename: John A
authors.initials: JA
authors.lastname: Butman
authors.forename: Alexander O
authors.initials: AO
authors.lastname: Vortmeyer
authors.forename: Christopher K
authors.initials: CK
authors.lastname: Zalewski
authors.forename: Carmen
authors.initials: C
authors.lastname: Brewer
authors.forename: Christal
authors.initials: C
authors.lastname: Surowicz
authors.forename: H Jeffrey
authors.initials: HJ
authors.lastname: Kim
mesh_terms: D000328:Adult; D001299:Audiometry; D019929:Cochlear Implantation; D004428:Ear Neoplasms / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D004712:Endolymphatic Sac / Q000473:pathology* / Q000601:surgery; D005260:Female; D034381:Hearing Loss / Q000209:etiology* / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D013506:Otologic Surgical Procedures; D014012:Tinnitus / Q000150:complications; D014057:Tomography, X-Ray Computed; D014717:Vertigo / Q000150:complications; D006623:von Hippel-Lindau Disease / Q000150:complications*