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Autoimmune Ear Diseases

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Causes: Scleroderma
Causes: Medications
Ménière's Disease
A Patient's Perspective
Personal Stories
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Overview: What is Autoimmune Ear Disease?

Photo of an earScleroderma can affect every part of the body, including the ears. Many autoimmune diseases can cause Autoimmune Ear Disease. Treatment goals in autoimmune inner ear disease include improving speech thresholds to levels treatable with hearing aids in severely affected patients and recovery of hearing to near normal levels in those with mild to moderate losses. (Also see: Autoimmune Diseases and What is Scleroderma?)

If caught early, and with aggressive medical management, hearing stabilization and possible improvement are feasible.

See source: Autoimmune Ear Disease by Dr. Douglas D. Backous.

Autoimmune Inner Ear Disease (AIED). Both allergy and traditional autoimmune disease such as Ankylosing spondylitis, Behçet's, Systemic Lupus Erythematosis (SLE), Sjoegren's syndrome (dry eye syndrome), Cogan's disease, ulcerative colitis, Wegener's granulomatosis, relapsing polychondritis, rheumatoid arthritis, and scleroderma can cause or be associated with AIED. Dizzinessandbalance.com. 12/08/12.
Sensorineural hearing loss as a common manifestation in patients with mixed connective tissue disease. Raynaud's phenomenon, antiphospholipid and antiendothelial cell antibodies, increased serum levels of proinflammatory cytokines and a reduced number of regulatory T cells were found to be associated with sensorineural hearing loss in mixed connective tissue disease patients. International Journal of Clinical Rheumatology. (Also see: Mixed Connective Tissue Disease)

Causes of Hearing Loss: Systemic Scleroderma

Hearing loss and vestibular disturbances are prevalent in systemic sclerosis. Symptoms may include hearing loss, headaches, vertigo, dizziness, tinnitus (ringing in the ears), and lack of balance.

Usually the hearing impairment begins as a gradual loss in the high-frequencies. Since this type of hearing loss can be subtle, and patients are often distracted dealing with other pressing scleroderma symptoms, their ear and vestibular damage is often not noticed until it is quite pronounced.

Therefore, it is a good idea for scleroderma patients to have their hearing screened on a regular basis, even in the absence of noticeable or bothersome problems with hearing or vestibular disturbances (like dizziness or loss of balance.)

Researchers Report Audiovestibular Dysfunction in Systemic Sclerosis Patients. There is an association between systemic sclerosis and inner ear function resulting in audiovestibular abnormalities. The Egyptian Rheumatologist, 02/26/2015. (Also see: What is Scleroderma?)
(Case Report) Scleroderma and sudden sensorineural hearing loss. In all cases of bilateral sudden sensorineural loss, AIED must be kept in mind as one of the cause, investigated and treated accordingly. Indian Journal of Otology, 2014, Volume 20, Issue 3, Pages 124-125. (Also see: What is Scleroderma?)
Hearing loss in diffuse cutaneous systemic scleroderma. Patients with diffuse scleroderma have a high prevalence of sensorineural audiometric hearing impairment and otological complaints, suggesting that the cochlea is an additional target organ in this disease. PubMed, Scandanavian Journal of Rheumatology, 2011 Sep 15. (Also see: What is Scleroderma? and Diffuse Scleroderma)
Auditory neuropathy in systemic sclerosis (SSc): a speech perception and evoked potential study before and after cochlear implantation. For subjects affected by SSc, the restoration of synchronous neural discharge could be achieved by electrical stimulation through cochlear implant. PubMed, Eur Arch Otorhinolaryngol.

Causes of Hearing Loss: Medications

Sudden Hearing Losses Prompt Label Change for PDE5 inhibitors. The FDA said it found 29 reports of sudden hearing loss, sometimes temporary, associated with the erectile dysfunction agents after scouring the Adverse Event Reporting System. There have also been cases of sudden hearing loss reported in patients using Revatio (sildenafil) for pulmonary arterial hypertension. Medpage Today. 10/22/07. (Also see: FDA Warnings and Pulmonary Hypertension)
Ototoxic Drugs Can Damage Hearing. Ototoxic drugs, which are medications that are toxic to the ear, have the potential to cause permanent or temporary hearing loss. Approximately 200 prescription and over-the-counter drugs are ototoxic, including some antibiotics, chemotherapy medications, anesthetics, cardiac medications, glucocorticosteroids, mood altering drugs, and some vapors and solvents. American Speech-Language-Hearing Association.
You Might Get Better, But You Might Lose Hearing: Ototoxic Drugs Cause Hearing Loss. An ototoxic hearing loss happens when someone takes or is given a drug that causes loss of hearing as one of its side effects, such as some antibiotics, chemotherapy drugs, and anti-inflammatories. About.com.

Ménière's Disease

Ménière's Disease. Meniere's disease is an abnormality of the inner ear causing a host of symptoms, including vertigo or severe dizziness, tinnitus or a roaring sound in the ears, fluctuating hearing loss, and the sensation of pressure or pain in the affected ear. The disorder usually affects only one ear and is a common cause of hearing loss. National Institute on Deafness and Other Communication Disorders (NIDCD).
Meniere's Disease. Meniere's disease is a disorder of the inner ear that causes spontaneous episodes of vertigo — a sensation of a spinning motion — along with fluctuating hearing loss, ringing in the ear (tinnitus), and sometimes a feeling of fullness or pressure in your ear. Mayo Clinic.
Meniere's disease might be an autoimmune condition? Meniere's disease is characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure. Proposed theories of causation include viral infections and immune system-mediated mechanisms. Autoimmun Rev, 2012 Jan 28.
Study links higher caffeine consumption with lower tinnitus risk. Tinnitus, an annoying ringing or buzzing in the ears, was less likely to occur in women who consumed the most caffeine, in a large study of nurses. Reuters, 08/15/2014.
High Prevalence of Systemic Autoimmune Diseases in Patients with Menière's Disease. Despite some limitations, Meniere's Disease (MD) displays an elevated prevalence of systemic autoimmune diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and ankylosing spondylitis (AS). PLoS One, 2011;6(10):e26759.


Tympanosclerosis refers to a scarring of the ear drum. The scarring can look like "water on the ear" but that is just an optical illusion. In some cases, it may result in hearing loss but nothing can be done to prevent this. Tympanosclerosis can be caused by either inflammation or infection.
In fancy terms it is, "Submucosal hyaline degeneration in the tympanic membrane and middle ear  mucosa. Extensive involvement of the TM and ossicles may result in conductive hearing loss. On rare occasion middle ear surgery is advised to restore hearing. Medical therapy and PE tubes do not prevent progression of disease." From Common Diseases of the External and Middle Ear by Baylor College of Medicine. Department of Otorhinolaryngology and Communicative Sciences.
Although scleroderma may cause tympanosclerosis in some cases, the presence of tympanosclerosis does not "count" as a diagnostic criteria for any form of scleroderma.

A Patient's Perspective

by Denise D. in Digest 3-22-98, Reprinted with permission of author.
When inflammation is complicated by bacteria and/or candida infections, it can cause the ears to be dry, itchy and flaky. A good prescription ointment for this is called Kenacomb Ointment (NOT CREAM). You use a Q-tip to carefully wipe a very thin layer over the inner ear. It really does stop the itching!
Kenacomb is a corticosteroid, anti inflammatory, antipruritic, antibacterial and anti fungal ointment and is indicated for use only for: "Corticosteroid-responsive inflammatory or pruritic dermatoses caused, threatened or complicated by infection due to bacteria and/or candida."


Extraintestinal Crohn's Disease Mimicking Autoimmune Inner Ear Disease: A Histopathological Approach. We report histopathological and immunohistochemical findings of the inner ear of a patient with a granulomatous inner ear disease suffering from Crohn's disease that was nonresponsive to treatment and who underwent surgery for bilateral cochlear implants. M Dettmer. (PubMed) Audiol Neurootol, 2010 Jun 3;16(1):36-40.

Personal Stories

Craig R: CREST Scleroderma I had my first scleroderma symptoms at age twenty-two, which included severe Raynaud's with some ulceration and tissue loss...
Gayle: Deaf with Limited Systemic Scleroderma (CREST) I am looking for another deaf person, female or male, with scleroderma to talk, share, write, e-mail to communicate with...
Marina F: Sjogren's Syndrome and Vitiligo (Italy) For quite a few years now I have had problems such as eczema in my ear, vitiligo, nodules in the thyroid, dry eyes with a positive Schirmer's test, and vertigo...
(Italiano) Marina F: Sindrome di Sjogren, Vitiligo Ho 47 anni e da diversi anni soffro di vari problemi quali: eczema alle orecchie, vitiligine, noduli tiroidei, secchezza oculare con test di schirmer positivo, e vertigini...
An explanation of autoimmune inner ear disease. Autoimmune inner ear disease occurs when a person's immune system attacks body cells rather than fights infection in the body. Autoimmune attacks in the inner ear result in progressive hearing loss. Bismarck Tribune. 12/25/2011.
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