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Autoimmune Inner Ear Disease

What Is AIED?

Autoimmune inner ear disease (AIED) is an inflammatory condition of the inner ear. It occurs when the body’s immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occurring in less than one percent of the 28 million Americans with a hearing loss. If this occurs, an Autoimmune Inner Ear Disease Specialist in NYC can help.

How Does The Healthy Ear Work?

The ear has three main parts: the outer, middle and inner ear. The outer ear (the part you can see) opens into the ear canal. The eardrum separates the ear canal from the middle ear. Small bones in the middle ear help transfer sound to the inner ear. The inner ear contains the auditory (hearing) nerve, which leads to the brain.

Any source of sound sends vibrations or sound waves into the air. These funnel through the ear opening, down the ear, canal, and strike your eardrum, causing it to vibrate. The vibrations are passed to the small bones of the middle ear, which transmit them to the hearing nerve in the inner ear. Here, the vibrations become nerve impulses and go directly to the brain, which interprets the impulses as sound (music, voice, a car horn, etc.).

Symptoms Of AIED

The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing loss can progress over weeks or months. Patients may feel fullness in the ear and experience vertigo. In addition, a ringing, hissing, or roaring sound in the ear may be experienced. Diagnosis of AIED is difficult and is often mistaken for otitis media until the patient develops a loss in the second ear. One diagnostic test that is promising is the Western blot immunoassay.

Treatment For AIED?

Most patients with AIED respond to the initial treatment of steroids, prednisone, and methotrexate, a chemotherapy agent. Some patients may benefit from the use of hearing aids. If patients are unresponsive to drug therapy and hearing loss persists, a cochlear implant maybe considered. It is important to consult with an Autoimmune Inner Ear Disease Specialist in NYC

History Of AIED

Until recently it was thought that the inner ear could not be attacked by the immune system. Studies have shown that the perisacular tissue surrounding the endolymphatic sac contains the necessary components for an immunological reaction. The inner ear is also capable of producing an autoimmune response to sensitized cells that can enter the cochlea through the circulatory system.

AIED Research

A multi-institutional clinical study, Otolaryngology Clinical Trial Cooperative Group (OCTCG) co-sponsored by the NIH and the American Academy of Otolaryngology-Head and Neck Surgery Foundation, is being conducted to measure the benefits and risks of treating AIED with two different immunosuppressive drugs: prednisone and methotrexate, a chemotherapy drug.

If you suffering from this condition, contact an Autoimmune Inner Ear Disease Specialist in NYC today.

Frequently Asked Questions

Autoimmune Inner Ear Disease (AIED) is an inflammatory condition where the immune system mistakenly attacks the cells in the inner ear, mistaking them for harmful viruses or bacteria. It is a rare disease affecting less than one percent of Americans with hearing loss.

Symptoms of AIED include sudden hearing loss that starts in one ear and rapidly progresses to the other, feelings of fullness in the ear, vertigo, and ringing or hissing sounds (tinnitus). The hearing loss may progress over weeks or months, making early diagnosis challenging.

Diagnosing AIED can be difficult as it is often mistaken for other ear infections like otitis media. One promising diagnostic approach is the Western blot immunoassay, which helps detect specific immune responses related to the disease, especially once hearing loss affects both ears.

Treatment for AIED typically includes immunosuppressive drugs like steroids (prednisone) and methotrexate. Patients may also benefit from hearing aids, and in cases where drug therapy is ineffective, cochlear implants might be recommended. It is important to consult an AIED specialist for appropriate management.

The ear consists of outer, middle, and inner parts. Sound waves enter through the outer ear and cause the eardrum to vibrate. These vibrations travel through small bones in the middle ear to the inner ear, where the auditory nerve converts them into nerve impulses sent to the brain, interpreting them as sound.

If you suspect you have Autoimmune Inner Ear Disease, it's essential to seek evaluation and treatment from an Autoimmune Inner Ear Disease Specialist. Specialists, particularly in NYC as referenced, have the expertise to diagnose and manage this rare condition effectively.

A multi-institutional clinical study sponsored by the NIH and the American Academy of Otolaryngology-Head and Neck Surgery Foundation is evaluating the benefits and risks of treating AIED with immunosuppressive drugs such as prednisone and methotrexate to better understand effective treatments.

Dr Robert Pincus

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