Publication:

Multicranial neuropathy secondary to endolymphatic sac tumor

Endolymphatic sac tumors (ELST) are rare neuroectodermal neoplasms arising from the epithelium of the endolymphatic sac or duct. Despite their benign histopathological features, ELSTs frequently demonstrate a locally destructive growth pattern with involvement of the skull base and cranial nerves. While ELSTs may arise sporadically, the majority of cases occur in association with Von Hippel-Lindau (VHL) syndrome. ELSTs are commonly diagnosed late due to their slow growing nature and non-specific symptomatology. Surgical resection is the treatment of choice. However, due to the location of these tumors in the lateral skull base surgical intervention carries a high risk of injury to critical neurovascular structures in close proximity. We presented the case of a 51 years old gentleman with a three-months history of hearing loss and otalgia. He subsequently developed multiple cranial neuropathies and was diagnosed with a sporadic ELST. He underwent a complete surgical resection and received adjuvant radiotherapy.