Endolymphatic Sac Tumor: A Case Report

Introduction: Endolymphatic sac tumors are low-grade adenocarcinoma of the temporal bone and are locally invasive. Material and Methods: The clinical records of a patient diagnostic with endolymphatic sac tumor managed surgically in our department were reviewed retrospectively. Results: A 34-year-old man, with a history of hearing reduction in the right ear since he was 24 years old; was referred to our department. He had a 6-month history of vertigo and tinnitus with progressive worsening in hearing. His bilateral tympanic membranes were found normal; his vestibular and neurological examinations were also normal. Audiometric evaluation showed a severe sensorineural hearing loss on one side. Results of the bilateral caloric test were normal. The speech recognition threshold on the left ear was 80 dB as compared with 35 dB on the right. MRI showed, on T1 gadolinium-enhanced, a heterogeneous enhancement of the tumor. Axial T2-weighted MRI scan of an endolymphatic sac tumor demonstrate heterogeneous signal. Axial and coronal CT scans showed a destructive mass epicentered on the right anterior petrous face of the temporal bone and erodes the internal auditory canal with central speculated calcifications. He underwent surgical resection by a retrolabyrinthine transdural approach. The patient has been well since the postoperative time but his hearing got worse. Conclusion: Endolymphatic sac tumors are known to occur more frequently in patients with von Hippel-Lindau (VHL) disease, but this is not a prerequisite for diagnosis.