Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease

Jay Jagannathan, Russell R. Lonser, Richard A. Stanger, John A. Butman, Alexander O. Vortmeyer, Christopher K. Zalewski, Carmen Brewer, Christal Surowicz, H. Jeffrey Kim

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8 Scopus citations

Abstract

OBJECTIVE: Bilateral endolymphatic sac tumors (ELSTs) are associated with von Hippel-Lindau disease and often underlie significant audiovestibular morbidity, including hearing loss. PATIENT: 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. INTERVENTION: 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. MAIN OUTCOME MEASURES: Clinical, audiometric, and imaging data. RESULTS: Postoperatively, the patient had resolution of tinnitus and vertigo with a significant implant-aided improvement in hearing. CONCLUSION: 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.

Original languageEnglish (US)
Pages (from-to)927-930
Number of pages4
JournalOtology and Neurotology
Volume28
Issue number7
DOIs
StatePublished - Oct 1 2007

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Keywords

  • Bilateral
  • Cochlear implant
  • Endolymphatic sac tumor
  • Hearing loss
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

Jagannathan, J., Lonser, R. R., Stanger, R. A., Butman, J. A., Vortmeyer, A. O., Zalewski, C. K., Brewer, C., Surowicz, C., & Kim, H. J. (2007). Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease. Otology and Neurotology, 28(7), 927-930. https://doi.org/10.1097/MAO.0b013e31805c7506