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.
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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Callier Library is a satellite facility of The University of Texas at Dallas, McDermott Library. It is located at the Dallas, Texas campus of the Callier Center for Communication Disorders. The library supports the graduate-level programs and faculty in communications sciences which are located at the center. It also supports the work of clinicians in hearing and speech disorders who work at both campuses of the Callier Center. One of the missions of Callier Library is to be a useful source of information to the international community of researchers and clinicians in communication disorders. To that end, this web log of citations and news in the field has been built and maintained by Allen Clayton, the Callier Center Librarian.
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