Autoimmune Vestibulopathy-A Case Series

Abstract

Autoimmune inner ear disease (AIED) is a rare clinical entity. Its pathogenicity, heterogenous clinical presentation in the context of secondary systemic autoimmune disease and optimal treatment avenues remain poorly understood. Vestibular impairment occurring in the context of AIED is rarely subject to detailed investigation given that the auditory symptoms and their responsiveness to immunosuppression are the focus of the few proposed diagnostic criteria for AIED. We present three cases of vestibulopathy occurring in the context of autoimmune inner ear conditions, including the first known report of autoimmune inner ear pathology arising with a temporal association to administration of the Pfizer-BioNTech SARS-CoV2 vaccination. We review the available literature pertinent to each case and summarise the key learning points, highlighting the variable presentation of vestibular impairment in AIED.

Keywords: AIED; COVID-19; Cogan syndrome; autoimmune; ulcerative colitis; vaccination; vestibular; vestibulopathy.

Figure 1

Two of the audiovestibular investigations performed at our Centre 18 months after original onset of symptoms. (A). Pure tone audiogram demonstrating moderate-to-severe hearing loss on the right (red trace) and moderate-to-profound loss on the left (blue trace). (B). Video head impulse test (vHIT) in Case 1 showing reduced gain (eye velocity: head velocity) of the vestibular ocular reflex across horizontal (top panel), and vertical (RALP plane, middle panel; LARP, bottom panel) planes. Note normal vestibulo-ocular reflex (VOR) gains using this vHIT device (GN Otometrics, Taastrup, Denmark); ≥0.8 for horizontal planes and ≥0.7 for vertical planes [20]. The traces show emergence of covert saccades occurring during the head movements, particularly on the right, reflective of dynamic vestibular compensation. Data not shown from impulsive rotational testing and oculomotor examination via videonystagmography.

Figure 2

Axial Real Inversion Recovery (IR) MRI image 4 h post-intravenous gadolinium showing asymmetric increased enhancement of the right cochlea. The (right side) shows cochlear (red) and vestibular (blue) endolymphatic hydrops. The (left side) also shows cochlear (purple) and vestibular (green) endolymphatic hydrops extending into the lateral semicircular canal (yellow). Many centres worldwide have adopted similar protocols in delayed contrast-enhanced 3D FLAIR MRI imaging for suspected endolymphatic hydrops [21,22].

Figure 3

Audiogram and vHIT undertaken at initial presentation (A,C respectively) and almost two years later (B,D) in Case 2. Audiometry showed a bilateral profound hearing loss initially, which improved markedly on the left with time, particularly in the low frequencies. vHIT showed globally reduced gain (normal being ≥0.8 in the lateral plane, ≥0.7 in the vertical planes) in all six semicircular canals tested which marginally improved over time.

Figure 3

Audiogram and vHIT undertaken at initial presentation (A,C respectively) and almost two years later (B,D) in Case 2. Audiometry showed a bilateral profound hearing loss initially, which improved markedly on the left with time, particularly in the low frequencies. vHIT showed globally reduced gain (normal being ≥0.8 in the lateral plane, ≥0.7 in the vertical planes) in all six semicircular canals tested which marginally improved over time.

Figure 4

Axial T1 volumetric interpolated brain examination (VIBE) fat-suppressed (FS) MRI post-intravenous gadolinium showing bilateral cochlear enhancement (yellow right, red left), more on the left than the right and left-sided vestibular and lateral semicircular canal enhancement (blue). The left intracanalicular cochlear nerve shows increased contrast enhancement (green).

Figure 5

Vestibular investigations undertaken five months following Pfizer-BioNTech SARS-CoV2 vaccination after the onset of imbalance and ataxia in Case 3. (A). vHIT showed reduced gain (0.38) with refixation saccades in the right lateral plane. (B). Cervical Vestibular Evoked Myogenic Potential (cVEMP) response was absent on the right (red traces, left panel). (C). Monothermic caloric testing demonstrated 100% right-side canal paresis (note almost absent nystagmus on right warm and right cool traces).

Figure 5

Vestibular investigations undertaken five months following Pfizer-BioNTech SARS-CoV2 vaccination after the onset of imbalance and ataxia in Case 3. (A). vHIT showed reduced gain (0.38) with refixation saccades in the right lateral plane. (B). Cervical Vestibular Evoked Myogenic Potential (cVEMP) response was absent on the right (red traces, left panel). (C). Monothermic caloric testing demonstrated 100% right-side canal paresis (note almost absent nystagmus on right warm and right cool traces).