Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease.
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.
Jagannathan
Jay
J
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
Lonser
Russell R
RR
Stanger
Richard A
RA
Butman
John A
JA
Vortmeyer
Alexander O
AO
Zalewski
Christopher K
CK
Brewer
Carmen
C
Surowicz
Christal
C
Kim
H Jeffrey
HJ
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*