Objective: To assess the demographics, clinical presentations, auditory and vestibular findings, value of serologic tests, and treatment outcome in autoimmune inner ear disease (AIED).
Study design: Retrospective chart review.
Settings: Tertiary care centre.
Patients: Sixty patients with confirmed AIED, with and without systemic disease.
Interventions: Diagnostic auditory, vestibular, and serologic tests and treatment with steroids.
Outcome measures: Auditory, vestibular, and serologic findings and treatment outcomes.
Results: The female to male ratio was 2:1. Forty-nine patients presented with unilateral or bilateral hearing loss; 28 patients also had vestibular symptoms. Eleven patients had vestibular symptoms only. Hearing loss was progressive in most, rapid in onset in one, and of sudden onset in two. Approximately 25% of patients had confirmed systemic autoimmune disease. Patients without systemic disease had serologic tests to confirm the diagnosis of AIED. The level of antinuclear antibodies was high, with a speckled pattern, in 38 patients, and 9 patients had a high rheumatoid factor. The positive yield of other detailed tests was low. Vestibular tests showed a peripheral type of change. Steroid treatment produced an excellent response in 33% and a good response in 16% without systemic disease. Only 25% of those with systemic disease had a similar response. Patients with vestibular symptoms only had an excellent response to steroids.
Conclusions: Patients with AIED present with varied symptoms, and some have only vestibular symptoms. Limited serologic tests used in the diagnosis of systemic autoimmune diseases are valuable in establishing the diagnosis of AIED. Fifty percent of patients with AIED have an excellent response to steroids. Those with systemic disease have a lower response rate. Those with vestibular symptoms only are responsive to steroids.