Hearing Loss, Ear Health, and Lupus

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What’s that? Hearing loss and other aural phenomenon may impact around 30% of people living with lupus.

“Autoimmune inner-ear disease” (AIED) was first described by Brian McCabe in his landmark 1979 paper. McCabe reported on 18 patients whose lupus symptoms included hearing loss due to damage to the nerves and structures of the inner ear.

Hearing loss is one of the less-appreciated symptoms of lupus and other autoimmune diseases. It is most common in middle-aged and older women. Because of this, autoimmune inner-ear disease is often mistaken for age-related hearing loss. 

Other symptoms of AIED include:

  • dizziness
  • vertigo and challenges with balance that can lead to falls
  • headaches that may muddy the hearing loss symptoms or blend into the other symptoms of lupus
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How do we hear?

Hearing is a surprisingly complicated process

First, sound starts as a vibration in the air. This vibration, which takes the form of a sound wave travels are caught by the outer ear. This ear is cupped to direct the sound wave into the narrow passageway in our head called the ear canal. 

The ear canal leads to the eardrum which is a membrane (thin layer of divider cells) that vibrates. At this point, the sound waves need to be amplified. This is performed by the three smallest bones in the body, named for the shapes they resemble:

  1. The malleus (or hammer)
  2. The incus (or anvil)
  3. The stapes (or stirrups)

These amplifications travel to the cochlea, a snail-like, fluid-filled structure. Fluid carries sound and vibrations better than air. This fluid ripples along a membrane that runs through the whole organ, called the “basilar membrane.” Cells called “hair cells” are located on this membrane, and are named for the tiny hair-like cilia (officially called “sterocilia”) that react to these ripples.

Much like whiskers, hair cells are attached to a neuron. When they react, they send neural impulses to the brain through a bundle of nerves called the “auditory nerve.” 

The brain processes these impulses into what we recognize as sound and puts the sound together with other sensory input and information to interpret sound. Quite the process!

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What non-lupus factors can lead to hearing loss?

Hearing loss can come from problems at any point at this system. Only one — earwax build up in the ear canal — can be reversed. Damage to the inner structures is permanent. Death of the very delicate hair cells in the cochlea is the most common type of damage. It can be caused by noise pollution (loud music or ambient noises overtime) or as a result of aging. The loss of hair cells means that sounds are less able to be transmitted to the brain.

Ear infections, abnormal bone growths, and tumors can also cause hearing loss. Plus, because the eardrum is exposed to the outside world it can be affected by:

  • pressure changes
  • shockwaves
  • accidental perforation by a finger or a foreign object (such as a Q-tip)
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How common are lupus-related hearing issues?

The immune system attacks cells and structures perceived as invaders throughout the body in systemic lupus erythematosus (SLE). Often, these attacks focus on specific organs or structures like the skin and kidneys. But, other parts of the body, including the pieces of the inner ear, are also at risk of damage.

Studies have been conducted to help determine the typical symptoms and prevalence of hearing loss with lupus. One of the first, a self-directed questionnaire given to 84 people in 1998, showed that 31% (26 of 84) of lupus patients experienced some aural symptoms:

  • 15% of participants (13 of 84) reported loss of hearing in one ear (unilateral) with or without tinnitus (ringing)
  • 17% of participants (14 of 84) reported loss of hearing in both ears (bilateral) with or without tinnitus

The study did not find statistically significant differences on other factors, like age duration of lupus, or noise exposure.

Similar results were found in a 2013 study conducted at a teaching hospital in Qazvin City, Iran. 45 participants with lupus were compared to matched controls. Participants were not included in the study if they had been exposed to high levels of noise, had taken certain medications, or had a history of ear infections of head trauma.

26.7% (12 of 45) of people with lupus had sensorineural hearing loss, a more significant progression of the AIED identified by Brian McCabe.


How does lupus contribute to hearing loss?

Vasculitis is the blockage or stiffening of blood vessels in the body. It can be caused by autoimmune damage or a build-up of immune cells and immune cell residues in the circulatory system.

Vasculitis can cause hearing loss by:

  • blocking oxygen flow to hair cells and auditory nerve
  • fatiguing the body and brain (and leading to poorer senses)
  • through small strokes (which damage the brain directly and can affect hearing)

SLE is known to cause vasculitis and increase the risks of heart disease and stroke, making hearing loss a secondary symptom for some patients. It can also be a combination of damage to ear structures and vasculitis, and SLE damage to the neurons of the ear and the brain

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Diagnosing lupus-related hearing loss

Doctors recommend that people should be checking their hearing regularly, especially if they are over the age of 35. This can allow hearing loss to be caught quickly and treated. 

However, determining if the cause is SLE is very difficult. Blood tests and analyzing other symptoms can help (read more about it here). But for people already known to have lupus, normal lupus medications are an option. If taking the medications and avoiding flares improves hearing or reduces the incidence of tinnitus, then it may be SLE-related. However, this is not conclusive and does not affect treatment.


Treatment for lupus-related hearing loss

SLE-related hearing loss can be treated with anti-inflammatories or other medications. Steroids, azathiprine, cyclophosphamide, and methotrexate have had some success. Outside of normal medical treatment for lupus, there are several options to treat hearing loss. No treatment can fully restore hearing or reverse the damage, but there are a few options to improve hearing or restore some function:

  • Hearing Aids are speakers that can amplify the sounds coming into the ear. They “turn up the volume”
  • Cochlear Implants are surgically implanted microphone systems that hook directly into the auditory nerve to send the sounds as electrical impulses into the brain. As long as the auditory nerve is functional, this allows even people who are otherwise completely deaf to “hear.” It does require practice to interpret the sensations they get from the device.

There is active research on hearing-improving prosthetics and other treatment methods that could restore hearing more, so keep your ears open ????

Comments (16)

16 thoughts on “Hearing Loss, Ear Health, and Lupus

  1. Have known I have lupus for 29 years, since 35 years old, which is about when hearing loss started in one ear. And I had never heard what you say in this article. Was told at the time that the loss was due to inflammation and would also happen in the other ear. I didn’t believe it because the effected ear had the bad sun exposure and the skin peeled and many other flare symptoms happened. Tinnitus and the hearing loss has gotten a lot worse lately. This article helps.

    1. Glad it could help and sorry to hear about your hearing challenges. There are a lot of aspects of lupus that seem to be rarely discussed – and we want to share! Thanks for reading

  2. Thank you, thank you, thank for this article. I was diagnosed with severe hearing loss earlier this year. There was no “cause” known, however I was diagnosed with Lupus in 1999, when I was 38. It all makes more sense now.

  3. Thank you for this article. I live in the U.K there is not a lot of research for lupus is done so your articles have helped me more than you can know.

  4. Wow, I have hearing loss in both ears, have had to wear hearing aids for many years No rhyme or reason for loss ,had suffered tinnitus for some time, never knew it could be related to Lupus. Thank you for the article, Even doctors had no explanation for the loss. Might have the answer now.

  5. It was helpful to read the part of your article that talks about things like shockwaves and pressure changes being a big reason for hearing loss. My uncle who has worked in an environment that leaves him exposed to heavy shockwaves from large equipment has finally decided to retire. However, I think the rigid nature of his work has caught up with him and his hearing has become worse than before. It’s nice to read this affirmation of my thoughts, so I’ll look for any place that can help me get some hearing aids for him.

  6. I’ll don’t seem to make any earwax in my right eat recently .I have SLE and am in pain from sinusitis have done a’biotics + Olive oil I’m sure you know. The Rheumatology consultant says this is nothing to do with lupus. HELP my hearing is getting worse

  7. Thank you for your article. I was officially diagnosed with lupus about four years ago, but I have had symptoms for the past 20 years or more. I have been experiencing tinnitus for the past year or so, but every time I mention it to my rheumatologist and/or his PA, they insist that lupus has absolutely nothing to do with tinnitus and that I am experiencing age related hearing issues. But this is the same doctor who said they can’t see any issues with my hips in the images taken, while the orthopedist I just saw diagnosed severe tendonosis. I would get another rheumatologist, but the practice doesn’t allow changing doctors and they pretty much have a monopoly in our community.
    How can I present this information to my doctor and get them open their eyes and minds?

  8. I was diagnosed, here in the UK, with SLE 4 years ago. My main Lupus problem is Nephritis and never knew that my Tinnitus (which I have had for decades) could be associated with SLE until very recently when it got even worse after a big flare. The care I’ve been getting from the Renal and Rheumatology teams has been fantastic (in the UK we are fortunate in that all medical services are entirely free of charge so I get to see lots of different experts, as needed) but I will keep you informed what kind of reaction I get when raise the fact that I am getting worse tinnitus than ever. Thanks to the Lupus Corner team for putting this excellent site together.

  9. I have had Lupus for over 20 years. I am 48. My right ear has a high piched ringing loud….n decreased hearing. It’s been goin on for about 7 hrs now. I guess I should go to the doctors office. This disease is such a monster.

  10. I have had Lupus for over 20 years. I am 48. My right ear has a high piched ringing loud….n decreased hearing. It’s been goin on for about 7 hrs now. I guess I should go to the doctors office. This disease is such a monster.

  11. I have had Lupus for over 20 years. I am 48. My right ear has a high piched ringing loud….n decreased hearing. It’s been goin on for about 7 hrs now. I guess I should go to the doctors office. This disease is such a monster.

  12. I have had Lupus for over 20 years. I am 48. My right ear has a high piched ringing loud….n decreased hearing. It’s been goin on for about 7 hrs now. I guess I should go to the doctors office. This disease is such a monster.

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