An unusual disease presenting at an unusual age: Susac’s syndrome

Susac's syndrome: Intratympanic therapy for hearing loss and a review of the literature

“…It is rare for all three classical components of Susac's syndrome to be apparent at the time of presentation, delaying diagnosis and likely contributing to underdiagnosis of the disorder. Patients are usually women between the ages of 20 and 40 years, although some are pediatric, such as the first in our study; and at least two have been diagnosed in their sixth decade 2. The clinical course typically fluctuates over months to years, with eventual resolution of the acute phase giving way to permanent neuropsychiatric, visual, and hearing sequelae 1…”

“…Perilymphatic pressure (measured by electrocochleography [ECOG] and tympanic membrane [TM] displacement analysis) is often found to be normal, though transiently elevated pressures have been reported 9. An MRI‐documented central nervous system (CNS) response has been detected subsequent to systemic treatment with steroids and cytotoxic medications but many patients retain serious hearing loss after such treatment, some requiring the placement of cochlear implants due to the development of profound deafness 2…”

Susac Syndrome-A Report of Cochlear Implantation and Review of Otologic Manifestations in Twenty-Three Patients

“…Furthermore, the mean age in the study presented by Papo et al (29) was 30 years, with a range of 18 to 40 years. Barker et al (19) reported the oldest patient with new-onset Susac syndrome at 50 years, and later, Plummer et al (14) reported a 55-year-old patient. The oldest patient in our study was …”

“…Similarly, the vestibulocochlear involvement varies in intensity. It is regarded as a disease of young Caucasian women, but it is also reported in men (6Y13), various races (10), and previously in patients as old as 55 years (14). The disease has a chronic relapsing course with unpredictable exacerbations and remissions but is generally regarded as self-limited.…”

Susac's syndrome: the first case report in a Nordic country, with an 8‐year follow‐up

“…Usually, it is self‐limiting and in some cases it responds well to steroid therapy with corticosteroids. Fewer than 100 cases of Susac's syndrome have been reported worldwide (Plummer et al 2005). To our knowledge this is the first case report of Susac's syndrome presented in a Nordic country.…”