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Clinical Problem Solving: Pathology
December 2001

Diagnosis Pathology Quiz Case 1

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2001;127(12):1504. doi:

Endolymphatic sac tumors are neuroectodermal neoplasms involving the inner ear. Even though they are considered benign, they are characterized by destructive and infiltrative local growth. Ten percent of ELSTs are associated with von Hippel–Lindau disease.1-4 The most prominent clinical symptoms are hearing loss, which is usually unilateral and sensorineural, and tinnitus with episodic vertigo. With progressive enlargement, the tumor may invade the CPA and involve cranial nerves and other local structures. In these instances, tumor growth may clinically manifest as facial nerve paralysis or jugular foramen syndrome.2,3,5