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Otol Neurotol. 2007 Oct;28(7):927-30.

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

Author information

1
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.

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.

PMID:
17468670
DOI:
10.1097/MAO.0b013e31805c7506
[Indexed for MEDLINE]

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