Cochlear Implantation for Hearing Loss Associated With Bilateral Endolymphatic Sac Tumors in von Hippel-Lindau Disease
Posted by Callier Library on October 5, 2007
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.
