Labyrinthine lesions in presumed inner ear hemorrhage-related sudden deafness

Inner ear hemorrhage (IEH) is a rare cause of sudden sensorineural hearing loss (SSNHL). This study aimed to evaluate the lesional patterns in patients with presumed IEH from morphological and functional aspects.

Seventeen patients with SSNHL and presumed IEH who completed audio-vestibular tests were included. The main outcome measures included clinical characteristics, radiology, and functional test results.

The morphological findings and functional tests revealed differences in locations and lesional spectrums. The magnetic resonance imaging (MRI) hyperintensity was likely to involve the vestibule (88.2%), the cochlea (76.5%), and the posterior and lateral semicircular canals (76.5% and 70.6%, respectively). Furthermore, 70.6% of cases showed abnormality in the entire labyrinth, and abnormalities in the vestibule/semicircular canals were observed in 17.6% of cases. Meanwhile, dysfunction was sequentially detected in the cochlea (100%), semicircular canals (94.1%), and vestibule (70.6%); 64.7% of cases showed combined deficit in the entire labyrinth, and 29.4% of cases showed combined deficit in the cochlea/semicircular canals. Although lesions in the labyrinth were frequently detected, the results of the radiological and functional tests did not always match and significantly differed in either cochlear or superior semicircular canal damage detection (p < 0.05 each).

In this cohort, IEH preferentially caused sudden audio-vestibular impairment, which was well demonstrated by a combination of MRI and functional tests. The specific lesional configurations revealed in this study may suggest a possible pathomechanism that could be further explored as a therapeutic target.