Successful cochlear implantation in a Susac syndrome patient

Otologic manifestations of Susac syndrome

“…Currently, cochlear implantation has been successfully employed in 5 patients who met surgical candidacy within the otolaryngology literature. Postoperatively, there was evidence of functional hearing restoration and notable improvement in communication abilities for all implanted SS patients 21 24 49-51 .…”

A Rare Cause of Hearing Loss: Susac Syndrome

“…Hearing loss can be a cause of serious and dramatic disability. The typical SNHL in SS, generally affecting low and middle frequencies, is mainly caused by microinfarcts in the apical cochlea and may be the first and only clinical feature of the disease [2] . Audiological symptoms are present in 22% of patients during onset.…”

“…Meniere's disease, Cogan's syndrome, idiopathic sudden hearing loss, viral diseases, autoimmune inner ear disease, and acoustic tumors should be considered in the differential diagnosis of the otologic involvement [2] . In our case, the imaging and laboratory findings performed for differential diagnosis were within normal limits.…”

“…Bilateral multiple retinal artery occlusions on fundoscopic examination or fluorescein angiography and SNHL typically involving low and medium frequencies are observed on audiological evaluation. Magnetic resonance imaging (MRI) findings include T2-weighted hyperintense small lesions in the corpus callosum and both grey and white matter of the infratentorial structures [2,3] . We presented a case with SS because of its rarity.…”

“…The classic clinical triad consists of acute or subacute encephalopathy, branch retinal artery obstruction, and hearing loss without prominent systemic manifestations [2] . SS frequently affects young women (20-40 years of age), and its pathogenesis is not yet clear.…”