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Hughes syndrome, known more formally as antiphospholipid syndrome (APS), is named for Dr. Graham Hughes, who discovered it in 1983 while working in London. Dr. Hughes and his colleagues detected antiphospholipid antibodies that lead to APS, a disorder of the immune system also known as "sticky-blood" syndrome. The discovery was described in 2004 by researchers in the European Journal of Internal Medicine as "changing the face of neurology."1
In general, lipids are any of a group of organic compounds, including fats, oils and waxes, that are part of the principal structural materials of living cells. More specifically, phospholipids are phosphorus-containing fat molecules found throughout the body, particularly in membranes surrounding each cell. As such, they are critical to cell function.
People with Hughes syndrome have an increased tendency to form clots in the blood vessels and around the organs of the body, a condition that causes wide-ranging and diverse symptoms, such as the following listed online by the Hughes Syndrome Foundation:2
- Headaches, including migraine;
- Giddiness;
- Memory loss;
- Visual disturbances;
- Skin disorders;
- Thrombosis, or blood clots;
- Heart attack;
- Stroke;
- Pulmonary embolism;
- Multiple sclerosis-like features;
- Gastrointestinal disorders; and
- Pregnancy problems.
The disorder is akin to lupus, an autoimmune disease that can affect various parts of the body, including the skin, joints, heart, lungs, blood, kidneys, and brain. According to the Lupus Foundation of America, "In an immune disorder like lupus, the immune system cannot tell the difference between foreign substances and its own cells and tissues. The immune system then makes antibodies directed against itself. These antibodies--called 'auto-antibodies' (auto means 'self')--cause inflammation, pain and damage in various parts of the body."3
The syndrome is also related to conditions such as fibromyalgia, thyroid disease and other clotting disorders. The prevalence of Hughes syndrome in the general population may be as high as 1 percent, and its cause is unknown.
Interestingly, hearing and balance disorders do not appear in the main listing of Hughes syndrome symptoms, and inquiring audiologists will find only a few tantalizing references to the effects of the syndrome on the ear. For example, in a 2009 feature article by Victoria Lambert in Telegraph.Co.UK, the author lists tinnitus, earache and dizzy spells among the many symptoms she had experienced in her own bout with Hughes syndrome.4 She describes the syndrome as "extremely common" but also "under-reported, misunderstood and dangerous to ignore."
She writes that the syndrome is a "relatively new condition that is just beginning to become recognized by the wider medical community outside the specialized area of auto-immune diseases (in which the body's immune system attack itself.)" According to Lambert, two particular organs seem most affected by the blood clots: the placenta and the brain. In the former, the clots may lead to multiple and unexplained miscarriages; in the latter, migraines, memory loss or a general "fogginess" may be experienced.
The discovery has revolutionized treatment of women with recurrent miscarriage, since the offending antibodies can be detected with a simple blood test 80 percent of the time, and treatment for most patients is half an aspirin a day to prevent clotting and resolve the symptoms. Those with more severe problems are offered heparin or warfarin by daily injection. According to Lambert, "Where the antibodies are present, and blood thinners are given, to prevent clotting at the placenta, the rate of successful pregnancy soars from about 20 percent to 80 percent."
Lambert notes that specialists in other areas of neurology and cardiovascular medicine have been less aware of the disorder and its health implications. "We know that it is present in one in five men aged under 45 who had a stroke, and one in five women aged under 35 who have a heart attack. Yet it is simply not being picked up early enough to prevent these illnesses," she writes.
The same lack of awareness of the auditory effects of the syndrome may be true among audiologists, since a review of the medical literature in PubMed turned up only two references to Hughes syndrome and hearing loss. In the 2004 European Journal of Internal Medicine, researchers at the Lupus Research Unit, The Rayne Institute, St. Thomas' Hospital in London, report, "Neurological manifestations are prominent and are often the dominant feature. Headache, migraine and cognitive dysfunction are common while other manifestations such as dementia, epilepsy, chorea, multiple sclerosis (MS), psychiatric disease, transverse myelitis, ocular syndromes, sensorineural hearing loss, and movement disorders are also associated with the syndrome."1
In 2007, researchers at the ENT Department, East Surrey and Crawley Hospitals, Redhill, UK, described in the Journal of Laryngology & Otology a case of bilateral aural symptoms in a 34-weeks pregnant woman with Hughes syndrome. The report "underscores the unpredictable nature of this syndrome and its ENT complications. No previous case of fluctuating hearing loss and otorrhoea [ear drainage] in Hughes syndrome has been described."5
Although there are few medical references for audiologists to turn to regarding Hughes syndrome and hearing loss, online Web sites touting cures for its symptoms such as tinnitus are easy to find. One Web site extols acupuncture and/or herbal medicines, writing, "The tinnitus herbs will regenerates (sic) your kidneys and liver's Qi system and total immune system to over come this tinnitus problem." Readers are advised to "order Tinnitus Herbal Medicine Treatment Cure by-e-mail."
As evidenced-based professionals, audiologists must warn patients against misleading or untested anecdotal information and focus on the latest medical and scientific research related to Hughes syndrome. Awareness of the syndrome's potential effects on hearing function, its detection and treatment are critical first steps in this process.
References
1. Lampropoulos, CE, Hughes, GR. (2004). The antiphospholipid (Hughes') syndrome: changing the face of neurology. European Journal of Internal Medicine, 15(3): 147-150.
2. Hughes Syndrome Foundation. Accessed online at www.hughes-syndrome.org/symptoms.htm
3. Introduction to Lupus. (2009). Lupus Foundation of America. Accessed online at www.lupus.org/webmodules/webarticlesnet/templates/new_aboutintroduction.aspx?
4, Lambert, V. (2009). Hughes Syndrome: simple to treat but all too often ignored. Telegraph.Co.UK. Accessed online at www.telegraph.co.uk/health/4400796/Hughes-Syndrome-simple-to-treat-but-all-too
5. Jovanovic-Bateman, L, Warrington, G. (2007). Fluctuating hearing loss with otorrhoea in Hughes Syndrome. Journal of Laryngology & Otology. 121(6): 535-8. Epub 2007 Feb 13.
Jess Dancer is professor emeritus of audiology at the University of Arkansas at Little Rock. Contact him at jedancer@ualr.edu regarding your personal or professional experiences with Hughes syndrome.
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