ENDOLYMPHATIC SAC TUMOURS (ELSTS)
ELSTs have only been recognized as a specific component of VHL disease relatively recently. In a large survey of VHL patients using MRI and CT scans, Manski et al. (1997) found that 11 % of patients with VHL disease had an ELST. Hearing loss is the most common symptom of ELST, but tinnitus and vertigo also occur in many cases. Hence an ELST should be considered in all VHL patients who complain of hearing loss. However, ELSTs are frequently asymptomatic and surgical intervention is not always indicated.