Autoimmunity is a sad and debilitating condition where a person’s immune system turns against itself and attacks its own body’s tissues and organs. There are various types of autoimmunity – some that lead to the slow destruction of cells and tissues, some that accelerate abnormal growth, and some that interfere with organ function.
This is why autoimmune diseases are generally classified into organ-specific diseases, and the main organs and tissues affected include the endocrine glands, connective tissues like the skin, muscles, and joints, the adrenal glands, and red blood cells. Autoimmunity in any of these areas generally affects a single organ.
According to the American Autoimmune Related Diseases Association (AARDA), there are currently 100+ autoimmune diseases known to medical science, and at least 50 million Americans suffer from one autoimmune condition. Among these, autoimmunity of the endocrine system (mainly the thyroid gland) is rare and was not discovered until 1835.
It was commonly called Graves’ disease, after Robert James Graves, the Irish doctor who discovered it. It was only later that a peculiar connection of thyroid autoimmunity with the eye was observed (Graves’ Eye Disease). In Graves’ disease, an overproduction of thyroid hormones (hyperthyroidism) affects the body’s metabolism.
But can it lead to hearing loss? Keep reading, as this article explores the connection between thyroid autoimmunity and hearing-related issues.
Understanding the Multi-Faceted Graves’ Disease
Hyperthyroidism may result from several diseases, but Graves’ disease is the most common. The thyroid gland is a vital part of the endocrine system, which secretes certain hormones that affect the metabolism of the entire body. It essentially controls metabolic rate or speed – the process by which the body converts food into energy.
When a person develops autoimmunity of the thyroid gland in the form of Graves’ disease, their metabolism is disturbed. This could manifest through the following symptoms –
- Constant mood swings, irritability, and anxiety
- Lethargy and fatigue
- An increased heat sensitivity along with a high rate of perspiration
- A tremor in the fingers and hands
- Unexplained weight loss, despite healthy eating habits
- Erratic menstrual cycles
- Reduced libido or erectile dysfunction
- Goiter or enlargement of the thyroid gland
- Insomnia or sleep disturbances
- Increased and irregular heartbeat
- Frequent bowel movements
- Development of thick, red skin (which usually appears on the top of the feet or the shins)
One unique aspect of Graves’ thyroid autoimmunity is that at least 30% of people with this disorder also show symptoms of Graves’ ophthalmology. This condition is commonly known as Thyroid Eye Disease (TED) in which hyperthyroidism has affected the patient’s eyes, particularly the tissues and muscles surrounding each eye. The common symptoms include –
- Double vision
- Pain or pressure in the eyes
- Bulging of the eyes or proptosis
- Gritty sensation
- Retracted eyelids that cause puffy eyes
- Redness and swelling
- Light sensitivity
- In severe cases, there may be vision loss, and the bulging could be so bad that a patient is unable to close their eyelids.
Treatment Options and Prognosis: A Boon or a Bane?
Graves’ disease and its ophthalmologic condition are typically diagnosed through blood tests, ultrasound of the eyes, and imaging tests like the CT scan and MRI. If the patient is only suffering from Graves’ disease (with the eye unaffected), the treatment goal is to reduce or block the production of thyroid hormones and to manage the effect of these hormones on the body. The top treatment methods include –
- Radioactive Iodine Therapy – This therapy is based on the fact that the thyroid gland requires iodine to produce its hormones. Radioactive iodine or radioiodine is consumed orally, and as the thyroid gland attempts to use this iodine, the radiation kills the overactive thyroid cells. Graves’ disease symptoms begin to reduce after a few weeks of treatment.
- Anti-thyroid Medications – These medication drugs work by interfering with the thyroid gland’s use of iodine for hormone secretion. Anti-thyroid medications are often used as a supplement treatment (before or after radioactive iodine therapy) because, as a standalone method, they may cause a relapse in the future.
- Beta Blockers – These are also medications, but they do not interfere with the thyroid gland’s hormone production process. Rather, they block the ‘effect’ of those hormones on the body. These drugs are suitable for immediate relief from symptoms like rapid heartbeat, mood swings, heat sensitivity, and anxiety. They are not recommended for patients with diabetes and asthma as they may complicate disease management in such cases.
- Surgery – If Graves’ disease is severe, partial (subtotal thyroidectomy) or complete removal (thyroidectomy) of the thyroid gland becomes mandatory. There are certain risks involved, such as damage to the parathyroid glands or the nerve controlling the vocal cords. However, such complications are rare. After recovery, the patient will have to consume thyroid medication for the rest of their lives (to supply the body with the necessary amounts of thyroid hormones).
If a patient suffers from the ophthalmologic condition of Graves’ thyroid autoimmunity, the treatment options will depend upon the phase and stage of the disorder. In general, there are two phases of TED – active and passive. In the active phase, the disease is acute and progresses rapidly, whereas the passive phase is a chronic stage where disease progression may stop, but some symptoms remain.
There are also three different stages of Thyroid Eye Disease – mild, moderate, and severe. In the mild stage, only some symptoms like dry eyes, redness, and swelling may appear (even then, they do not impact daily life). In the moderate stage, the patient suffers from proptosis, eye misalignment, and double vision. When the disease becomes severe, the eyelid retraction and proptosis are so bad that the patient is unable to close their eyelids. Plus, tissue scarring (behind the eyes) occurs at this stage. The healthcare practitioner will recommend the best treatment option(s) based on the patient’s unique case.
The top treatment methods for TED include –
Teprotumumab or Tepezza
The most promising (new) therapy used to treat TED is a drug manufactured by Horizon Therapeutics called Tepezza. A single therapy session (consisting of eight infusions) is effective for even moderate-to-severe TED cases, but here’s where the hearing-related problems come in.
The drug received approval from the Food and Drug Administration (FDA) in 2020, and the first-ever Tepezza lawsuit against the manufacturer was filed in August 2022 by an Arizona resident, Daniel Weibel, who alleged that the drug caused permanent hearing loss.
The National Institutes of Health (NIH) confirms this claim as Tepezza led to hearing-related problems in 46% of patients tested in a study. According to TruLaw, numerous more lawsuits were filed over the past year, and there is now an official Tepezza class-action holding the manufacturer accountable for the otologic injuries caused by the drug.
Also known as glucocorticoids, corticosteroids are drugs used to treat TED’s active phase. They are strong anti-inflammatory drugs that reduce pain and swelling in the acute stages of the disorder. A case in point would be a patient whose TED has rapidly progressed to the point of optic nerve compression.
If the inflammation is not managed, the patient may lose their vision permanently. To save the patient’s eyesight, the doctor will recommend high doses of corticosteroids (which bring down the swelling quickly).
Orbital Decompression Surgery
The surgical option of treating TED is reserved for the most severe cases, mainly those in which vision loss is inevitable. The goal of the surgery is to prevent or reverse vision loss and reduce bulging and eyelid retraction.
An incision is made on the upper eyelid to remove the bony wall behind the eye. The extent of decompression depends entirely upon the proptosis. The prognosis is usually good and patients may take two to four weeks for recovery.
Is There a Way to Prevent the Inevitable?
While the good news is that there is more than one treatment option for Graves’ disease, even Graves’ eye disease, they are dependent upon how far a patient’s condition has progressed. Since the link between the Tepezza drug and hearing-related issues is proven, it is best to avoid this treatment option (and treat it as the last resort).
Thyroid eye disease is still a largely misunderstood condition (given its rarity), and the Food and Drug Administration (FDA) has only approved Teprotumumab-trbw or Tepezza as a suitable treatment regardless of disease duration or activity. Other treatment methods are the most effective for early-stage disease, not the more advanced stages.
If your hyperthyroidism is such that it has progressively affected the eye, try discussing with your healthcare practitioner to opt for a half-dose therapy. A case study on a half-dose therapy was conducted on a 64-year-old woman with moderate-to-advanced TED. Though initially, she faced sensorineural hearing loss after four infusions of Tepezza, there was a significant reduction in side effects when the treatment was restarted a year later at half the initial dosage.
For those with no proptosis or glucocorticoid-intolerance issues, Intravenous Glucocorticoid Therapy (IVGC) is also a good option when the TED is moderate-to-severe.
Teprotumumab, or Tepezza, has shown a high efficacy rate with TED management. However, this treatment option is what directly links thyroid autoimmunity to hearing loss and other otologic issues like tinnitus.
In the case of patients with advanced TED issues, an individualized approach is the most recommended – one based on factors like disease severity, impact on daily living, proptosis, patient age, disease duration, and most importantly, treatment costs.
A new consensus statement on Thyroid Eye Disease was released by the European Thyroid Association (ETA) and the American Thyroid Association (ATA). Amongst other things, the statement assured that novel therapies and treatments for TED are on the horizon, which will make it a more manageable disease in the years to come.