Sensorineural hearing loss is a common disorder that results from damage to the inner ear in over 95% of all cases; therefore, retrocochlear hearing disorders are rare and cannot be differentiated from sensory losses by clinical symptoms alone. Associated vestibular and other neurotological symptoms must lead to appropriate diagnostic procedures. Besides audiological and neurotological tests, e.g. auditory-evoked potentials, otoacoustic emissions or electronystagmography, modern imaging methods are the most valuable tools to detect tumorous, vascular and inflammatory processes. Acoustic neuromas, which are the most frequent causes, require otoneurosurgery with the attempt to preserve hearing and facial nerve function. Multiple sclerosis and vascular lesions of the vertebral arteries or their branches require an interdisciplinary approach. Due to recent advances in diagnostics, imaging and management, retrocochlear disorders have gained much interest from otologists in the last few years. They provide new insight into the physiology and pathophysiology of hearing.