Autoimmune inner ear disease

What is AIED?
Autoimmune inner ear disease (AIED) occurs when the body produces antibodies against parts of the inner ear that can result in hearing loss and nerve damage. In AIED, the immune system produces white blood cells that produce antibodies that destroy cells within the inner ear mistaking them for microorganisms or viruses. This condition is rare and occurrs in less than one percent of the approximate 28,000,000 patients presenting with a sensorineural (nerve or inner ear related) hearing loss in the United States per year.

Normal Ear function
The ear is composed of three main components: the outer, middle and inner ear. The outer ear begins with the ear lobe that focuses sound onto the opening of the ear canal and ends in the ear drum. The ear drum is a biological barrier that separates the ear canal from the middle ear space. Small bones in the middle ear that connect between the ear dum and a small membrane on the outer part of the inner ear help to conduct the sound waves from the outside to the inner ear. The inner ear contains the cochlea (the hearing organ) which resembles the body of a snail and is filled with fluid, the cochlear (hearing) nerve, which leads to higher center in the brain where sounds are processed into the myriad of language and/or identification centers and association centers to make us interpret what we hear. Any source of sound sends vibrations or sound waves through the air. These waves that initially travel through air are funneled through the ear opening, down the outer ear canal, and end up vibrating the ear drum (tympanic membrane). The vibrations are then conducted to the small bones of the middle ear (ossicles), which vibrate a thin membrane (the oval window) that creates a fluid wave within the cochlea (the hearing organ). This fluid wave then moves little hairs within the inner ear that convert the mechnical energy ito signals that travel through the cochlear nerve to the brain for interpretation of the sound.

What do I feel if I have AIED?
AIED is usually first diagnosed because of a sudden onset of hearing loss in one ear progressing rapidly to the second ear. The hearing loss is usually diagnosed as sensorineural (i.e. coming from the inner ear or beyond) and can worsen over weeks to months. You may feel fullness in your ear and even feel a spinning sensation also known as vertigo where the world feels like it is spinning around similar to being in a round up amusement ride.
In addition, it is not uncommon to feel a ringing, hissing, or roaring sensation in the affected ear. Diagnosis of AIED hinges on the diagnosis of sudden sensorineural hearing loss, thus requiring a hearing test. If you have hearing loss and are seen at an urgent care facility where no clear diagnosis is made, it is best to schedule an appointment with Dr. Dagan. to initiate treatment right away and avoid hearing loss in the other ear. Diagnosis of AIED requires blood tests that detect the antibodies produced by the body against the inner ear.

How is AIED treated?
Dr. Tal Dagan will usually offer an initial treatment with corticosteroids like prednisone. Other immunologic chemotherapeutic drugs may be necessary when the diagnosis is made. A quick response is essential to prevent permanent hearing loss that can result in deafness. Patients who are non-responsive to medication and develop profound hearing loss or deafness may benefit from a cochlear implant.

If you feel you have any type of hearing loss, inquire with Dagan MD for a hearing evaluation, hearing improvement and treatment. We offer a vast array of diagnostic and treatment for hearing related issues. For more information about hearing aids, please click here.