Evaluation and management of endolymphatic sac and duct tumors.

The endolymphatic sac tumor is a slow-growing, locally aggressive neoplasm that originates from the epithelium of the endolymphatic sac and duct. Disease progression can lead to profound sensorineural hearing loss, posterior fossa invasion, brainstem compression, drop metastasis, and eventual death. Early diagnosis and surgical attention are the primary objectives in the management of patients who have endolymphatic sac tumor. This article describes the latest rationale and techniques for hearing preservation surgery and a review of the latest developments surrounding this disease entity.