“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. There are different levels of hearing loss, from “mild hearing loss,” where a person can hear someone talking but not softer sounds, to “profound hearing loss” where only very loud sounds can be heard. All levels of hearing loss are possible for people with lupus.
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:
- vertigo and challenges with balance that can lead to falls
- headaches, which may muddy the hearing loss symptoms or blend into the other symptoms of lupus.
You can read more about headaches and lupus here.
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, called “ossicles,” named for the shapes they resemble:
- The malleus (or hammer)
- The incus (or anvil)
- The stapes (or stirrups)
Next, these amplified vibrations need to be turned into a form that the brain can understand.
So, the vibrations then 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!
Hearing loss can occur at various points in this process, and are considered to be multiple types.
Sensorineural hearing loss is hearing loss caused by damage to the neural connection between the inner ear and the brain. In a review of 9 studies with a total of 7654 patients, people with SLE have a higher rate of sensorineural hearing loss.
Conductive hearing loss happens when sound cannot get through the outer and middle ear due to fluid build-up, plugged ears, or irritation from infection.
Mixed hearing loss is caused by damage or issues with multiple parts of the ear.
What Non-Lupus Factors Can Lead to Hearing Loss?
Hearing loss can happen in one or both ears, and can come from problems at any point at this system. Hearing loss can also come on suddenly or gradually over time. 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
- accidental perforation by a finger or a foreign object (such as a Q-tip)
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.
Diagnosing Lupus-Related Hearing Loss
Doctors recommend that people should check their hearing regularly, especially if they are over the age of 35. This allows 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 stay tuned!
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