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Unilateral Sensorineural Hearing Loss Presenting With Bilateral Temporal Bone Lesions.

A 15 year-old male presented with several months of left-sided hearing loss and tinnitus. He reported no loss of balance, vertigo, headaches, otalgia, or neurological impairment. Physical examination was unremarkable. Audiometry revealed left-sided profound sensorineural hearing loss and normal hearing on the right. Computed tomography (CT) of the temporal bones without contrast demonstrated expansion of the vestibular aqueduct without erosion on the right (Fig. 1A). On the left there was an 11 14 19 mm expansile lesion at the posterior petrous ridge with extension into the mastoid cavity and effacement of the mastoid segment of the facial nerve (Fig. 1B). T1-weighted magnetic resonance imaging (MRI) with gadolinium contrast showed patchy opacification of the left mastoid with a hyperintense lesion along the left posterior petrous ridge and a smaller hyperintense lesion along the right petrous ridge (Fig. 1C). Several punctate areas of enhancement in the cerebellum were also noted. T2-weighted MRI revealed the petrous ridge lesions to have heterogeneous hyperintense signals. Diffusion-weighted sequences showed no restriction. These radiographic findings suggested bilateral endolymphatic sac tumors (ELST) and cerebellar hemangiomas consistent with von-Hippel-Lindau (VHL) disease. Subsequent abdominal imaging was negative. Endolymphatic sac tumors are low-grade, locally invasive tumors that arise from the epithelium of the endolymphatic duct and sac. They are commonly sporadic, although there is a well-established association with VHL disease (1). Sporadic ELST are often diagnosed in the fifth decade of life as compared with the third decade of life for tumors associated with VHL disease.