Autoimmune sensorineural hearing loss (ASHL) is an uncommon autoimmune disorder of the inner ear.
Synonym: autoimmune inner ear disease (AIED)
Criteria involve all of the following:
(1) progressive hearing loss, which may follow a fluctuating course and which can be unilateral
(2) one or more of the following:
(2a) antibodies to 68-kd or other inner ear antigens
(2b) diagnosis of an autoimmune disease
(2c) improvement with corticosteroids
(3) absence of an alternative explanation (noise, drug, infection, etc)
In addition to hearing loss clinical features may include:
(1) tinnitus
(2) vertigo
(3) Menieres
The onset of hearing loss may precede other symptoms of an autoimmune disease so the diagnosis may evolve over time.
Some patients respond to corticosteroids and/or immunosuppression but some do not, with progression to complete deafness.
