Susac syndrome - A report of cochlear implantation and review of otologic manifestations in twenty-three patients

OBJECTIVE: Susac syndrome is a disease condition of unknown cause consisting of vestibulocochlear dysfunction, retinopathy, and multifocal encephalopathy derived from microangiopathy of the ear, retina, and brain, respectively. We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. STUDY DESIGN: Clinical records of 23 patients diagnosed with Susac syndrome were reviewed. Analysis included demographics, clinical course, and audiometric data. An additional review of relevant vestibulocochlear data is undertaken among the approximately 100 previously reported cases. RESULTS: Of the 23 patients with Susac syndrome, 19 (83%) were women. Mean age was 36 years, ranging from 19 to 69 years. Ten patients (43.5%) reported a fluctuating hearing loss, 14 (61%) reported tinnitus, and 13 (56.5%) noted vertigo. Eleven patients (48%) presented with bilateral symptoms, and 12 (52%) were unilateral. In the 34 affected ears, the pure-tone average was 41.5 dB, and the mean percent hearing loss was 26.4%. Forty-seven percent of the affected ears had American Academy of Otolaryngology-Head and Neck Surgery hearing classification type A. Only 26.5% of the affected ears had 100% word recognition. Statistical analysis supported an overall "upsloping" pattern of hearing loss. Bilateral simultaneous cochlear implantation was successful in restoring significant hearing in our patient. CONCLUSION: Susac syndrome is a rare and potentially devastating disease. Hearing loss is quite variable. Low- and mid-range frequencies seem to be most commonly affected. Patients whose hearing loss meets criteria should be considered for cochlear implantation.