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Otologic manifestation of the Susac syndrome – Disease-specific diagnosis
18 April 2018 (online)
Creating an otological profile of the Susac syndrome. The Susac syndrome has to become more present in the daily work routine of an ENT-specialist.
A cohort of 25 patients was evaluated in this study. The otologic symptoms were measured based on pure tone audiogram, German Freiburg speech test, video head impulse test, caloric test and vestibular evoked myogenic potentials (VEMPS). The patients were interviewed about suffering from tinnitus, sudden hearing loss and vertigo.
Mean age at otologic manifestation was 32.6 years. Pure tone average showed an overall hearing loss of 37.6 dB. In the Freiburger speech test the mean hearing loss of numbers was 38 dB and the mean speech perception at 65 dB was 57%. The amplitude ratio in the VEMPS was in half of the cases out of range. 79% of the patients suffered from tinnitus. Vertigo was reported in 70%, sudden hearing loss in 67% of the cases.
In contrast to other studies a hearing loss was measured in the higher audio frequencies in 52% of the tone audiograms. A characteristic hearing curve in pure tone audiograms was found related to the amount of hearing impairment. In moderate hearing loss (PTA-4 = 40 – 59 dB) it shows a boat form, in severe hearing loss (PTA-4 ≥60 dB) a high frequency attenuation. At least a tinnitus and/or a sudden hearing loss was found in patients with Susac syndrome.
Tinnitus, vertigo in combination with (sudden) hearing loss, in young female should lead to further neurological and ophthalmological examination. In ENT diagnostics especially the pure tone audiogram and the VEMPS are effective to characterize the Susac syndrome. To determine the diagnosis a MRI-Scan performed by a center specialized in Susac syndrome is necessary.