Autoimmune Inner Ear Disease (AIED)

What is Autoimmune Inner Ear Disease?

Autoimmune inner ear disease is an uncommon cause of sensorineural hearing loss in which the immune system is thought to attack the inner ear.^[1] It is rare, with an incidence of fewer than five cases per 100,000 people, and it mostly affects middle-aged women.^{[1, 2]} It may be a primary ear disease or occur alongside a systemic autoimmune disease.^[2]

Common symptoms

The typical picture is hearing loss in both ears that progresses over weeks to months and responds to immunosuppressive treatment.^[2] Other symptoms can include:

• Tinnitus (ringing, buzzing or roaring in the ears)
• A sense of pressure or fullness in the ear
• Dizziness, vertigo or unsteadiness in some people
• Hearing that fluctuates and then worsens

Hearing loss that comes on quickly needs urgent assessment. See the last section.

Causes and risk factors

The cause is not fully understood. Both cellular and antibody immune responses seem to be involved, and the inner ear protein cochlin may be a target.^[2] AIED can accompany other autoimmune conditions, but there is no reliable blood marker that confirms it.^{[1, 2]}

How it is diagnosed and treated conventionally

AIED is diagnosed largely by excluding other causes of hearing loss, using examination, audiometry, blood tests and imaging.^{[1, 2]} Because no diagnostic marker exists, a good response to steroids often supports the diagnosis.^[2]

Systemic corticosteroids are the first-line treatment and often need to be used long term to prevent relapse.^{[1, 3]} Intratympanic steroids can be used as an adjunct or alternative, and people who do not respond to steroids may benefit from other immune-suppressing or biologic drugs.^[3] A meta-analysis of 10 small studies of disease-modifying drugs such as methotrexate, azathioprine and biologics reported improved hearing measures with relatively low rates of adverse events.^[4] Timely treatment can restore hearing in some patients, but long-term drug side effects have to be balanced against hearing outcomes.^[1] Cochlear implantation is an option for refractory cases.^[2]

Why Autoimmune Inner Ear Disease calls for a whole-person approach

AIED is primarily an immune and ear disease treated by specialists, but its effects reach beyond the ear. Hearing loss, tinnitus and imbalance affect sleep, concentration, mood and social life.

Supportive care can address some of these effects while your ear specialist and other physicians manage the immune disease.

Immune activity

An immune response directed at the inner ear is the proposed mechanism.^[2] This is why steroids and other immune-suppressing drugs are the core of treatment, and why any supplement that might affect immune function should be reviewed by your doctor.

Tinnitus and its distress

Tinnitus is a perception of sound without an outside source, and it is a common problem for which clinical management includes education, relaxation therapy, cognitive behavioral therapy and sound-based therapies.^[5] Distress from tinnitus often disturbs sleep and concentration.

Balance and dizziness

Some people have vertigo or unsteadiness, which can limit activity and raise fall risk.^[2] Vestibular rehabilitation is directed by your medical team.

Sleep, stress and medicine side effects

Long courses of steroids and other drugs can affect sleep, mood and general health, so symptoms need to be reviewed with your medical team.^[1] Poor sleep can make tinnitus feel worse.

Acupuncture as supportive care in Autoimmune Inner Ear Disease

What the research shows

No clinical trials of acupuncture in autoimmune inner ear disease were found in PubMed. The evidence below concerns related symptoms in other groups of people and may not apply to AIED.

For tinnitus, a meta-analysis of 8 trials with 504 participants found no significant difference in the main visual analogue score between acupuncture and control, though two secondary tinnitus questionnaire scores improved. The authors rated the evidence as insufficient because of low quality and small trials.^[6] An earlier review noted that most Chinese trials reported positive results while most English-language trials were negative, and found methodological flaws in many studies; it concluded that acupuncture may offer subjective benefit to some patients but not definitively.^[7] A network meta-analysis of 2,575 patients suggested acupuncture-based treatments may be a better trend treatment, and called for more rigorous trials.^[8]

For sudden sensorineural hearing loss, which is a different condition, a meta-analysis of 12 trials found acupuncture added to standard medical treatment worked better than standard treatment alone, but the evidence was low or very low quality because of bias and small trials.^[9] A second review of 20 trials reported similar findings, with no serious adverse events, and also called for high-quality research.^[10] These trials were mostly done in China and do not address autoimmune hearing loss.

Acupuncture has also been studied for sleep. A meta-analysis of 24 insomnia trials found, in the 15 trials that compared acupuncture with sleep medicines, better sleep scores with acupuncture, with large differences between trials.^[11]

How acupuncture may work

No mechanism for AIED has been studied. Proposed general explanations involve the nervous system and blood flow, but these are hypotheses.

What treatment involves

Acupuncture uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes. A short series of visits is followed by reassessment of symptoms. Acupuncture does not replace hearing tests or the steroid treatment prescribed for AIED, and any sudden change in hearing needs a doctor first.

In prospective studies, about 9 in 100 patients had a minor reaction such as bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[12] Tell the practitioner first if you have a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker (relevant to electroacupuncture). Also tell us if you take immunosuppressants, since they can raise infection risk.

Ayurvedic medicine as supportive care in Autoimmune Inner Ear Disease

The Ayurvedic view

Ayurveda has no classical name for autoimmune inner ear disease. Practitioners may describe hearing loss, tinnitus and dizziness as disturbances of Vata, the principle linked with movement and the nervous system. These are traditional frameworks, not biomedical diagnoses.

Therapies that may be used

Care is gentle and supportive, and may include:

• Regular meals, sleep and daily routine
• Calming practices such as breathing exercises
• Individually prescribed herbs, only if your physician agrees

Nothing should be placed in the ear unless your ear specialist has cleared it. Panchakarma is not appropriate during pregnancy, acute illness or frailty, or while taking high-dose steroids or other immunosuppressants without your physician's agreement.

What the research shows

No studies of Ayurvedic treatment in AIED were found, so its effect on this condition is unknown. About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic, so product quality is a real concern.^[13] Any Ayurvedic herb that affects bleeding or immune function raises the same interaction questions described in the herbal section below.^[14]

Herbal medicine as supportive care in Autoimmune Inner Ear Disease

Herbs and formulas that have been studied

No clinical trials of Chinese or Ayurvedic herbs in AIED were found. Ginkgo biloba is an herb that has been tested in trials for a related symptom, tinnitus.^[5]

What the research shows

A Cochrane review of 12 trials with 1,915 participants found that the benefits and harms of Ginkgo biloba for tinnitus are uncertain compared with placebo, with low to very low certainty evidence and high or unclear risk of bias.^[5] It is not evidence for or against herbs in AIED.

Safety and herb-drug interactions

Herbs are active substances and can interact with medicines. Ginkgo has been reported to cause bleeding with warfarin or aspirin, and St John's wort lowers blood levels of ciclosporin and tacrolimus.^[14] Many people with AIED take steroids, methotrexate, azathioprine or biologics, so every herb needs review by your prescribing physician.

Herbs should come from tested sources and be prescribed individually.^[13] Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

The links drawn here are interpretation, not equivalence. Chinese medicine often relates hearing loss and tinnitus to kidney deficiency, liver fire or phlegm obstructing the ear, and Ayurveda to Vata imbalance. Modern medicine describes immune-mediated inflammation of the inner ear.^[2] No study has shown that a traditional pattern corresponds to a specific antibody or hearing finding in AIED.

How this care fits with your medical care

Complementary care for AIED is supportive only. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Do not stop or change prescribed medicines, including steroids and immunosuppressants, without your prescriber, and keep your hearing tests. We do not diagnose the cause of hearing loss or manage the disease itself.

Please bring your diagnosis, recent audiograms, a full list of medicines and supplements, and the name of your ear specialist.

When to seek urgent medical care

Sudden hearing loss and rapidly progressive hearing loss need prompt medical assessment, because AIED responds best to timely treatment.^{[1, 2]}

• Sudden hearing loss in one or both ears, over hours to days
• Hearing that is getting rapidly worse over weeks
• Severe vertigo with vomiting, or repeated falls
• Facial weakness, double vision or trouble speaking
• Eye redness or pain along with hearing loss
• Fever with ear pain or severe headache

If any of these occur, call 911 or go to the nearest emergency department.