After sixteen years of unexplained and intense pain, physicians at Rambam Health Care Campus (Rambam) finally identified the rare syndrome affecting Dana—one that strikes fewer than one percent of the population.
Thirty-five-year-old Dana* from Northern Israel began suffering at age 19, with recurring headaches, earache, facial pain, and other unexplained sensations, without any apparent cause. The pain was so intense that at one point she had to stop doing what she loved most—singing. Over the years, she consulted many physicians, underwent numerous tests, and even had surgery, yet the mysterious pain continued to affect her life.
Eventually, a team of multidisciplinary physicians from the Department of Oral and Maxillofacial Surgery, the Department of Neurology, and the Division of Medical Imaging at Rambam, discovered that Dana was suffering from a syndrome so rare and mysterious that the medical community is divided over its very existence. The condition is known as Eagle syndrome.
Diagnosis is often delayed for years
“Eagle syndrome is a clear example of a rare medical condition that can present with symptoms resembling other conditions: facial, neck, jaw, and throat pain; difficulty swallowing and speaking; tinnitus, and a sensation of pressure around blood vessels in the neck area,” explains Dr. Dani Noy, a senior physician in Rambam’s Department of Oral and Maxillofacial Surgery, who treated Dana. “Because there are so few documented cases, identifying it is extremely difficult, and diagnosis is often delayed for years.”
“The variety of symptoms makes it difficult for both doctors and patients to realize that the source of the problem is anatomical,” adds Dr. Noy. “The condition is caused either by abnormal elongation of the styloid—a thin bone beneath the ear, behind the tongue—or by calcification of the muscle attached to it. Normally, the styloid is about three centimetres long; in one case it was nearly seven centimetres!
Adjusting to life without constant pain
Such elongation can exert pressure on nearby nerves, muscles, and blood vessels. In some cases, major blood vessels that supply or drain the brain are affected, increasing the risk of dizziness, fainting, loss of consciousness, and even stroke.
Once the process begins, a cycle develops: chronic inflammation—part of the body’s healing response—triggers calcification, which causes additional pain and inflammation, leading to further calcification.
Once physicians diagnose the syndrome, they usually perform a short surgical procedure to remove the affected bone or calcified tissue. Patients typically experience significant relief, although their bodies need time to adjust to life without constant pain.
Dana’s surgery was successful, and she is recovering well. Despite its rarity, Dana is one of several patients with this syndrome recently treated at Rambam—all with positive outcomes.
Cases like Dana’s highlight the importance of multidisciplinary expertise—something Rambam is committed to providing so that patients with rare, hard-to-diagnose conditions can finally get answers and effective treatment.
*Name changed for patient privacy
