| Associated
Conditions: MAIN MENU |
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| Autoimmune
Diseases: Autoimmune Ear Disease |
| This page
was written by Shelley
Ensz, and has not yet been medically edited. Scleroderma
(SD) affects everyone differently. Just because something is
listed here does not mean an individual patient will ever experience
it. See Disclaimer. |
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| 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.
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. |
| 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) |
| Viagra (sildenafil citrate) Information, FDA Warning. FDA received reports of sudden decreases or loss of hearing following the use of PDE5 inhibitors, Viagra, Levitra, Cialis for the treatment of erectile dysfunction and Revatio for the treatment of pulmonary arterial hypertension. U.S. FDA. 10/22/07. (Also see: Raynaud's 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. |
| What You Should Know About Ototoxic Medications. If you have an existing sensorineural hearing loss, regardless of the cause, when using ototoxic medications, you are more vulnerable to aggravation of that hearing loss. Stephen Epstein, M.D. |
| 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). |
| The Ménière's Page. Symptoms, incidence, special diets. Washington University School of Medicine. |
| Symptoms and Incidence of Ménière's disease. Meniere's disease is usually characterized 4 symptoms. 1) Periodic episodes of rotatory vertigo or dizziness. 2) Fluctuating, progressive, low-frequency hearing loss 3) Tinnitus 4) A sensation of "fullness" or pressure in the ear. Washington University School of Medicine. |
| 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. |
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| 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." |
| Research |
| Audiologic Aspects in Systemic Sclerosis. In the majority of SS patients enrolled in this study sensorineural hearing loss was detected. This impairment was localized both inside and outside of the cochlea which could suggest the presence of VIII nerve neuropathy in affected individuals. B. Trzcinska-Butkiewicz. SAT0250 EULAR 2006. |
| New lab research may help those deafened by immune system attack. Study may one day lead to test that could show which patients may be helped by immediate steroid treatment. It may also expand the definition of the condition, known as autoimmune sensorineural hearing loss or AISNHL, and help more people get a firm diagnosis of what's causing their mysterious hearing loss. i-Newswire.com 08/17/05. |
| Autoimmune-mediated sympathetic hearing loss: a case report. Damage to one inner ear is occasionally followed by contralateral sensorineural hearing loss. Observations indicate that this patient suffered sympathetic hearing loss caused by organospecific autoimmunity directed to cochlear outer hair cells. PubMed. Otol Neurotol. 2005 Mar;26(2):161-5. |
| Antiphospholipid antibodies in patients with sensorineural hearing loss. Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Our data suggest that antibodies against beta(2)-glycoprotein seem to coincidence with an acute event, such as sudden sensorineural hearing loss, whereas antibodies against phosphatidylserine IgG are detectable in the prolonged sequel, such as in patients with progressive sensorineural hearing loss and normal tension glaucoma. PubMed. Eur Arch Otorhinolaryngol. 2005 Feb 25. (Also see: Antiphospholipid Syndrome and Eye Involvement) |
| Ear-nose-throat manifestations of autoimmune rheumatic diseases. Ear-nose-throat (ENT) manifestations of connective tissue disorders represent a diagnostic challenge for clinicians as they often constitute the initial sign of an otherwise asymptomatic autoimmune disease. Moreover, in patients with known autoimmune rheumatic diseases, ENT manifestations can be overlooked. PubMed. Clin Exp Rheumatol. 2004 Jul-Aug;22(4):485-94. |
| Cranial nerve VIII involvement in a patient with progressive systemic sclerosis. We report an elderly woman who presented with sensorineural hearing loss and was then diagnosed with the CREST syndrome of progressive systemic sclerosis. PubMed. Tenn Med. 2004 Mar;97(3):117-9. |
| Electrocochleographic findings in cases of autoimmune disease with sensorineural deafness. These results suggest the etiologies of SNHL (sensorineural hearing loss) in cases of ADSD (autoimmune disease with sensorineural deafness) remain unclear. Further investigation is needed to resolve the etiology of SNHL of ADSD. PubMed. Auris Nasus Larynx. 2003 Dec;30(4):349-54. |
| Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. Methotrexate does not appear to be effective in maintaining the hearing improvement achieved with prednisone therapy in patients with AIED. Pub.Med. JAMA. 2003 Oct 8;290(14):1875-83. |
| 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... |
| 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... |
| Media Stories |
| Dizziness can be a sign of more serious problem. The cause of Meniere's disease is still not known. One theory is that there is too much fluid in the inner ear. Another is that there is too much salt in the diet. Still a third theory is that the illness is an autoimmune sickness of the cells that line the balance organ. Staunton News Leader. 08/07/05. |
| Related Links |
| 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. 2006 Jun 9. |
