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| Authors | Age | Sex | Race | Antibody | HL before (negative) or after (positive) onset (years) | Previous neurological symptoms | Immunosuppressive treatment prior to HL | HL type | Simultaneous neurological symptoms with HL | Brain MRI at HL | Treatment | Prognosis |
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| Jarius et al. [16] | 51 | M | NA | AQP4+ | 2 | ON and LETM | MMF | Unilateral (left) | None | No lesions | Oral prednisone | Fully recovered after 4 weeks |
| Kremer et al. [9] | NA | NA | One Caucasian
Two non-Caucasians | One, AQP4+
Two, AQP4- | NA | NA | NA | NA | NA | NA | NA | One, not recovered
Two, completely recovered |
| Gratton et al. [17] | 54 | F | NA | AQP4+ | NA | NA | NA | Bilateral, central SNHL | | T2 lesions near the cochlear nuclei, more prominent on the right | IV mPD, PE | Resolved |
| Takanashi et al. [20] | 40 | F | Japanese | AQP4+ | 0 | None | | Unilateral (right) retrocochlear-type SNHL | Diplopia, upper hemianopsia, numbness in the hands and feet, and dysuria | T2 lesions in the optic chiasma, optic tract, hypothalamus, and left cerebral fornix | IV mPD | Improved |
| Tanaka and Tanaka [8] | 26 | F | Japanese | AQP4+ | 6 | NA | NA | NA | NA | NA | NA | Improved |
| Jarius et al. [14] | 19 | M | Caucasian | MOG+ | 0.17 | ON and myelitis$ | NA | NA | Recurrent ON, myelitis, disorientation, headache, and fever | T2 lesions in the pons, basal ganglia, corpus callosum, periventricular, pulvinar thalami, rostral putamen, optic chiasm, optic tract, and leptomeningeal contrast enhancement | IV mPD | Fully recovered |
| Bonnan and Cabre [18] | 53 | F | Caribbean | AQP4+ | 0.75 | ON | No | Unilateral (right) SNHL | Area postrema syndrome, tinnitus, and vertigo | Right eighth cranial nerve and the adjacent meninges are enlarged and enhanced | IV high-dose steroid, followed by mitoxantrone infusion | Highly improved |
| Shaw et al. [23] | 54 | F | Caucasian | AQP4+ | 9 | NA | NA | Right-sided low-to-mid-frequency moderate SNHL and mild low-frequency left-sided SNHL with 1-year interval | Left-sided vestibular hypofunction | No lesions | High-dose oral mPD | Left SNHL normalized and right SNHL showed unchanged |
| Tugizova et al. [15] | 54 | F | Japanese-American | AQP4+ | NA | Area postrema syndrome and LETM | None | Bilateral, mild to moderate SNHL at 3000-8000 Hz in the right ear and at 6000-8000 Hz in the left ear | Vertigo and tinnitus | NA | IV mPD, several months after SNHL | Fully recovered |
| 26 | F | Caucasian | AQP4+ | -1 | ON | None | Unilateral (left) mild CHL at low frequency | Bilateral tinnitus | NA | Worsening after RTX | Persisted |
| 45 | F | Hispanic | MOG+ | NA | ON | RTX, IVIG, PE, MMF, AZA, and steroids | Unilateral (right) mild SNHL at 1500-6000 Hz | Tinnitus | Symmetric enhancement of the bilateral distal internal auditory canals and cochlea | Oral prednisone | Resolved |
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