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Hearing Loss and other aural phenomenon may impact around 30% of people living with lupus. AIED- autoimmune inner-ear disease was first described by Brian Mccabe in his 1979 report. McCabe reported on 18 patients whose lupus symptoms included hearing loss due to damage of the nerves and structures of the inner ear. Aural symptoms are prevalent among patients with lupus. Asymmetric symptoms and hearing loss are most common. The cause may relate to immune-complex disease and/or vasculitis (an inflammation of the blood vessels which occurs due to antibodies of the immune system attacking blood vessels). It is most common in middle aged and older women. Because of this autoimmune inner ear disease is often mistake for age related hearing loss. AIED symptoms also include dizziness, vertigo, balance problems, and headaches that may muddy the hearing loss symptoms or blend into the other symptoms of lupus.

Hearing is a Surprisingly Complicated Process

Sound waves travel into the ear canal until they reach the eardrum which is a membrane that vibrates. The eardrum passes the vibrations through the middle ear bones which are the three smallest bones in the body. The bones are named after the shapes they resemble. one is the malleus or hammer, second is the incus or anvil and third is the stapes or stirrups. Once the vibrations cause the fluid inside the cochlea to ripple, a traveling wave forms along the basilar membrane. Hair cells – sensory cells sitting on top of the basilar membrane – ride the wave. Hair cells near the wide end of the snail-shaped cochlea detect higher-pitched sounds, such as an infant crying. Those closer to the center detect lower-pitched sounds, such as a large dog barking. The auditory nerve carries this electrical signal to the brain, which turns it into a sound that we recognize and understand.

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. This damage can be caused by loud music and ambient noises overtime but the death of the very delicate hair cells in the conchlea is the most common type of damage. The loss of the hair cells means that sounds are less able to be transmitted to the brain. Other things can also cause hearing loss… ear infections, abnormal bone growth, and tumors. Since the eardrum is exposed to the outside world it can be also be affected by pressure changes, shock-waves, and accidental perforation by a finger or a foreign object such as a Q-tip.

How Common Are Lupus-related Hearing Issues?

Studies have been conducted to help determine the typical symptoms and prevalence of hearing loss with lupus.

1998 Study

In 1998 a self directed questionnaire given to 84 people. The presence of tinnitus, hearing loss, and fluctuating hearing was evaluated by a self-directed questionnaire in patients aged 65 or less from a lupus clinic. Patients reporting aural symptoms were compared with those reporting none, by use of demographics, and disease duration. Audiometry was offered to all patients with lupus reporting aural symptoms and was completed in 10 of them. 31% patients with lupus reported aural symptoms. Unilateral hearing loss with or without tinnitus in 15% and bilateral hearing loss with or without tinnitus in 17%. No statistical difference was measured between symptomatic and asymptomatic patients when compared by average age, duration of disease, history of noise exposure, head trauma, and infectious ear diseases. Statistically significant differences were detected only when comparing average creatinine and C3 levels. Of those patients tested by audiometry, 70% had abnormal pure-tone thresholds. Asymmetric findings were present in 6 of these 7 patients tested. The study did not find statistically significant differences on other factors like age duration of lupus or noise exposure.

2013 Study

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 level of noise, had taken certain medications, or had a history of ear infections or head trauma. 27% 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. Lupus (systemic lupus erythematosus) is a condition in which antibodies attack your own tissues. Vasculitis can cause hearing loss by 1) blocking oxygen flow to hair cells and auditory nerve, 2) fatiguing the body and brain, 3) through small strokes that damage the brain directly. Systemic lupus erythematosus (SLE) is known to cause vasculitis and increase the risks of heart disease and stroke. Vasculitis and SLE can be a combination of ear damage with damage to the neuron of the ear and the brain.

Diagnosing Lupus-related Hearing Loss

In order to catch hearing loss quickly and treated anyone over the age of 35 should be checking their hearing regularly. However, if the cause of the hearing loss is related to Lupus is very difficult to diagnose. Blood test and analyzing other symptoms can help but people known to have lupus and taking their medication to avoid flareups, should be able to improve hearing or reduce the incidence of tinnitus. If they still do experience hearing loss, it may be SLE related.

Treatment for Lupus-related Hearing Loss

Different treatments for SLE-related hearing disorders have often been proposed. These treatments are mainly focused on prevention, especially for slowly progressive hearing loss, and hearing restoration for cases of sudden hearing loss. Corticosteroid therapy is the most prevalent therapy for sudden hearing loss. Other treatments of hearing disorders in patients with SLE include plasmapheresis or cyclophosphamide and these drugs have some success.

The following are a few options to improve hearing or restore some function…

  • Hearing aids are speakers that amplify the sound .
  • Cochlear implants are surgically implanted microphones that hook directly into the auditory nerve. As long as the auditory nerve is functional even people who are other wise deaf to “hear will be helped. It does require practice to interpret the sensations they get form the device.

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The Cincinnati Rheumatic Disease Study Group (CRDSG) is an organization of practicing rheumatologists dedicated to improving the care of patients with arthritis and other rheumatic diseases. It strives to do this by performing rigorous and ethical clinical research with the goal of developing better treatments for all patients with these conditions.
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