gms | German Medical Science

Introduction: Susac’s syndrome is an "auto-immune" micro vascular disease of the smallest blood vessels of the brain, the retina and the relevant endothelium of the inner ear, that causes an ischemic reaction through the decrease of the blood flow. The temporally delayed occurring symptoms are hearing loss, encephalopathy and retinal vascular occlusion. There are two different processes that have been described: the monophase, encephalopathic processes are mostly self limiting and the polyphase pathway, which leads to irreparably vascular occlusions of retinal branch arteries and acute hearing loss, which are difficult to treat. By September 2010, 208 cases had been documented in 134 articles. The dark figure is potentially higher. The objective of this article is to draw more attention on the Susac’s syndrome with the existing symptoms.

Material and Methods: Nine patients (six women, three men) have been seen in the ENT Department, who were treated interdisciplinary in the Susac center of Muenster of the Neurological clinic together with the ophthalmological department and the ENT clinic of the University Clinic of Muenster. All patients were examined in the ENT clinic to raise a neurootological status.

Results: The average age at initial diagnosis was 28,5 years. The time frame between initial diagnosis and first complaints was between 1 week–7 years – in average 1.9 years. With eight patients there was a cochlear involvement. The revealed hearing loss through audio and speech audiometric examinations ranged from normal hearing to high graded hearing loss, even deafness . None of the nine patients indicated a middle ear related hearing loss. Six patients had Tinnitus on both sides with tonal character or similar character. A patient had occasional tinnitus on both sides in both ears in the form of a beeping sound. Two patients denied tinnitus. Three patients complained of balance problems, whereby walking difficulties and disturbance in coordination were named (in the case of a patient these complaints were accompanied by falls). By means of videoocculography a nystagmus could be recorded with one patient. The other six patients reported no balance disturbances.

Conclusion: The hearing loss with Susac’s syndrome can occur on one side or even on both and relates to deep and middle frequences. The cause is the affection of the apicale cochlea blood vessel resulting from the occlusion of the cochlear arteries. Often a hearing aid is necessary, and with deafness there is a need for a cochlear implant. A vestibular caused vertigo or tinnitus with Susac’s syndrome is not obligatory. From the occurance of the first symptom to the discovery of the diagnosis of Susac’s syndrome there is often a great deal of time. The otological symtoms evokes the Meniere’s Disease and so the patients are often treated accordingly. Especially with the treatment of relatively younger patients with symptoms are similar to the Meniere’s Symptom Complex and additional visual field loss or neurological deficits, Susac’s syndrome should be considered. The pathognomonic changes in the brain can be found generally in the MRI in Corpus callosum. Due to the complexity of this illness the diagnosis requires an interdisciplinary cooperation between neurology, opthalmology, radiology, and ENT medicine.