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Autoimmune inner-ear disease is a rare and treatable cause of vertigo, hearing loss and vestibular loss.
The clinical hallmark of autoimmune inner-ear disease is acute, sequential, asynchronous and asymmetrical impairment of auditory and vestibular function in both ears.
In a patient with recurrent spontaneous vertigo or unexplained vertigo, audiometry and vestibular assessments are essential to make the diagnosis
The diagnosis is helped by a thorough history regarding inflammatory and autoimmune symptoms, by laboratory investigations and by an ophthalmological assessment for inflammatory or vasculitic changes within the eye.
A 69-year-old man presented to the Emergency Department with 1 day of acute spontaneous vertigo, left-sided tinnitus and hearing loss. He had no otalgia, previous vertigo or hearing problems but did have a background of diabetes and hypertension. On examination, with fixation, there was no nystagmus, but without visual fixation (using Frenzel’s goggles), there was primary position left-beating nystagmus. The horizontal head-impulse test was negative, but his left Dix-Hallpike positional test was positive, provoking a paroxysm of up-beating, torsional nystagmus with vertigo. Otoscopy was normal with no vesicles. Weber’s test was positive to the right and Rinne’s was bilaterally negative. Smooth pursuit, saccades and cerebellar examinations were normal.
Audiometry identified a left-sided profound and high-frequency and moderate-to-severe mid-frequency sensorineural hearing loss; right-ear audiometry identified only a mild high-frequency sensorineural hearing loss (figure 1A). He had acoustic reflexes in both ears at normal thresholds.
Contributors BN, GMH and MSW contributed to the study conception and design. BN, RG and MSW consulted with the patient. Figure preparation was done by BN, ASY and MSW. Manuscript preparation, data collection and analysis were performed by BN, ASY, GMH and MSW. The first draft of the manuscript was written by BN, and all authors commented on previous versions of the manuscript.
Provenance and peer review Not commissioned; externally reviewed by Diego Kaski, London UK.
Competing interests None declared.
Patient consent for publication Consent obtained directly from patient.