Autoimmune Diseases

Autoimmune Ear Diseases

Overview: What is Autoimmune Ear Disease?

Scleroderma 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.

Effect of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) on sudden sensorineural hearing loss (SSNHL). Comorbid SLE or RA may negatively affect the prognosis of SSNHL. PubMed, Laryngoscope, 12/04/2019. (Also see Symptoms and Complications of Lupus and Symptoms and Complications of Rheumatoid Arthritis)

Untreated hearing loss tied to cognitive decline in older adults. While age–related hearing loss has long been linked to cognitive decline, a UK study suggests hearing aids may help minimize the risk of problems like impaired memory or executive function. Reuters Health, 10/16/2018.

Sound therapy may train the brain to ignore tinnitus. A sound–emitting device worn in the ear during sleep may train the brain to ignore an annoying chronic ringing in the ears, a new study suggests. Reuters Health, 01/19/2018.

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.)

Hearing loss in patients with scleroderma (SSc): associations with clinical manifestations and capillaroscopy. Subjective and objective hearing loss were higher in patients with SSc compared to the control group and also sensorineural hearing loss, abnormal pure tone audiometry, and abnormal speech reception threshold. PubMed, Clin Rheumatol, 06/02/2018.

(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. (Also see What is 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

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.

Ménière's Disease

Ménière's Disease. Ménière'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.

Diagnostic and therapeutic strategies for Ménière's disease of the Japan Society for Equilibrium Research. Treatment option of the disease should be selected in order of invasiveness, according to the severity of the disease and the response to each treatment. PubMed, Auris Nasus Larynx, 2020 Oct 29;S0385-8146(20)30277-7.

Tympanosclerosis

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."

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…

SCLERO.ORG was the world's leading nonprofit for trustworthy research, support, education and awareness for scleroderma and related illnesses from 1998 to 2021. It was a grassroots movement from the original Scleroderma from A to Z web site, which was founded by Shelley Ensz. We were a 501(c)(3) U.S.-based public charitable foundation. We closed this web site and our nonprofit agency in April 2021.