Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease

Objective: To describe the clinical presentation of patients with isolated saccular endolymphathic hydrops (EH) detected. Study Design: Clinical case series. Setting: University-based tertiary referral center. Patients: All subjects presenting with vertigo or hearing loss who had isolated saccular EH detected. Intervention: High-resolution delayed-contrast magnetic resonance imaging (MRI) conducted between November 2015 and November 2016. Main Outcome Measures: Audiovestibular testing results and analysis of clinical histories. Results: Isolated saccular EH was detected in 18 subjects. Sixteen met criteria for definite Menière's disease (MD, n = 12) or delayed endolymphatic hydrops (DEH, n = 4). One had a history of sudden sensorineural hearing loss (SSNHL) and 3 years after MRI developed recurrent vertigo characteristic of DEH. One patient had a history of atypical DEH (Tumarkin falls without vertigo following SSNHL). Four patients had Tumarkin falls. Most (83%) demonstrated mild-to-severe low-frequency fluctuating loss, and six (33.3%) had a history of ipsilateral sudden profound SNHL. Nine of the 17 (53%) patients tested had an ipsilateral caloric paresis ranging from 26 to 67%. Ipsilateral vestibular-evoked myogenic potentials showed reduced or absent responses in 5 of the 17 tested (29%). Conclusions: The full spectrum of MD may be associated with saccular hydrops. We propose that MD and DEH often begin in the saccule, and MRI may provide clues to the pathophysiology of MD. Saccular hydrops was present in one patient with SSNHL who did not develop vertigo spells until 3 years after MRI, indicating that saccular hydrops may be the first manifestation of MD or DEH.