Medline ® Abstract for Reference 65
of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'
Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease.
Jagannathan J, Lonser RR, Stanger RA, Butman JA, Vortmeyer AO, Zalewski CK, Brewer C, Surowicz C, Kim HJ
Otol Neurotol. 2007;28(7):927.
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.
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.