A Case of Bilateral Sudden Deafness Caused by Wernicke Encephalopathy

Abstract

Wernicke encephalopathy, also known as thiamine deficiency, is characterized by a triad of symptoms: ophthalmoplegia, ataxia, and confusion. However, additional symptoms may manifest depending on the area affected by the lesion. Although multiple cranial neuropathies are possible, sudden onset bilateral hearing loss has been described in rare situations as the first manifestation of Wernicke encephalopathy. Here, we present a case report detailing the clinical experience of a patient diagnosed with Wernicke encephalopathy, whose initial presentation included sudden bilateral deafness. The patient was a 57-year-old man with alcoholism who was successfully diagnosed with Wernicke encephalopathy, and his hearing improved after high-dose intravenous thiamine therapy. Our case study results and a literature review indicate that video findings and suppression head impulse tests can be used to evaluate Wernicke encephalopathy.

Keywords: Central hearing loss; Sudden deafness; Thiamine; Wernicke encephalopathy.

Fig. 1.

T2-weighted/fluid-attenuated inversion recovery (T2/FLAIR) axial images at the level of pons (A), inferior colliculus (B), third ventricle (C), thalamus (D, E), and lateral ventricle (F). They illustrate symmetrically increased signal intensity detected at the dorsomedial thalami (①), inferior tectal plate (②), and periaqueductal grey matter within the dorsal midbrain (③). Furthermore, diffusion-weighted image (DWI) reveals heightened signal intensity present in both the bilateral dorsomedial thalami and inferior tectal plate of the dorsal midbrain.

Fig. 2.

Audiological testing. A: On hospital day 2, pure tone audiometry shows high-frequency sensorineural hearing loss in both ears. The hearing of mid-to-low frequencies are within normal limit. B: Loudness discomfort levels for pure tones are increased at all frequencies. C: Acoustic reflex appears normally in both ears to ipsi- as well as contralateral stimulation.

Fig. 3.

Due to the presence of ophthalmoplegia, the video-nystagmography (VNG) does not detect any eye movements during vestibulo-ocular assessments (A) of spontaneous nystagmus, positional nystagmus, and head-shaking nystagmus; and during ocular motor tests (B) including saccades, smooth pursuit, and optokinetic (OPK) nystagmus tests.

Fig. 4.

Video head impulse test (vHIT). A: vHIT exhibits reduced gain without overt/covert corrective saccade in all semicircular canals, especially both vertical ones. Decreased vestibulo-ocular reflex (VOR) gains are defined as <0.8 for the horizontal canal and <0.7 for the vertical canals. B: Conversely, the suppression head impulse (SHIMP) test yields reduced gain without anticompensatory saccade in both lateral semicircular canals.