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  • Title: Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel-Lindau disease.
    Author: Jagannathan J, Lonser RR, Stanger RA, Butman JA, Vortmeyer AO, Zalewski CK, Brewer C, Surowicz C, Kim HJ.
    Journal: Otol Neurotol; 2007 Oct; 28(7):927-30. PubMed ID: 17468670.
    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.
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