Some degree of hearing loss is common with age. Most types are irreversible, but autoimmune inner ear disease (AIED) is an exception.
Maria C. Buniel and colleagues at the Cleveland Clinic provided a review.
“AIED is one of the few treatable forms of sensorineural hearing loss and is diagnosed on clinical suspicion,” Buniel wrote. The hearing loss develops over the course of weeks to months, in both ears, often with one ear affected more than the other one.
Some patients experience temporary facial paralysis. Vertigo is less common. The primary treatment is corticosteroids.
Not everyone responds to corticosteroids. Shresh Pathak of the Feinstein Institute for Medical Research in New York and colleagues investigated factors that may determine which patients can benefit from treatment.
Pathak identified two subgroups of sensorineural hearing loss: AIED and sudden sensorineural hearing loss (SSNHL).
The SSNHL patients usually experience an isolated hearing loss event in only one ear. The cause may be autoimmune, but is more likely to be viral. “Nonetheless, the immunologic responses may be similar,” Pathak wrote.
Approximately 70 percent of patients initially respond to corticosteroids, but only 14 percent remain responsive after 34 months.
Pathak found that patients who do not respond to steroids have higher levels of the immune molecule IL-1beta in their blood. This finding offers guidance to researchers who are investigating new treatments.
“To date, no effective treatment has been identified for patients with either AIED or SSNHL who do not respond to steroids,” he concluded.
The Vestibular Disorders Association offers more information and support online. Their web site reports that other autoimmune disorders can also affect the ears:
2.Systematic lupus erythematosus