Endolymphatic sac tumors.

Endolymphatic sac tumors (ELST) are slow-growing, locally aggressive, low-grade malignancies that originate from the epithelium of the endolymphatic duct and sac. ELST often present with sensorineural hearing loss, tinnitus, and vertigo, which may mimic Meniere disease. Large tumors may present with additional cranial neuropathies. Management is primarily via microsurgical excision. Radiation therapy has a limited role for residual or unresectable disease. Early detection may enable hearing preservation techniques. ELST have an association with von Hippel-Lindau disease.