Ernest Syndrome | Is this the answer to unresolved TMJ, facial, ear and throat pain?

Ross Hauser, MD

Ernest Syndrome – the missing diagnosis of unresolved TMJ pain

In the more than 28 years of helping patients with chronic pain, we often find that what people have been diagnosed with is not really the problem causing them their continued and chronic pain. Such is the case of people who were diagnosed with problems of TMJ and still have jaw pain and related issues.

In this article, we will focus on Ernest syndrome as the missing diagnosis in not only what is thought to be TMJ pain, but in simultaneous symptoms to include swelling of the salivary glands and a pain in the throat area.

The problems of Ernest syndrome surrounds the damage and weakening of the stylomandibular ligament, a small but powerful ligament that connects the skull to the jaw by way of the styloid process of the temporal bone to the mandible. The missing diagnosis of Ernest Syndrome as the cause of TMJ related pain is found in that both TMJ and Ernest Syndrome have similar if not identical symptoms.

Dr. Wesley E Shankland 2nd, a dentist who has written extensively on the problems of TMJ, wrote this in the Journal of craniomandibular practice. (1) We have made this similar observation in the patients we see at our center.

It is not uncommon for practitioners who treat craniofacial pain to see patients with undiagnosed throat and submandibular pain. Usually, these patients will already have been seen by their primary care physician and frequently, several other doctors including otolaryngologists, oral and maxillofacial surgeons, and even neurologists. Far too often these patients have three common features:

1. they have endured multiple expensive diagnostic tests;

2. they have received treatment of multiple courses of antibiotics; and

3. no specific diagnosis for their pain complaints has been determined and their pain persists.

He then lists five disorders Ernest syndrome, Eagle’s syndrome, carotid artery syndrome, hyoid bone syndrome, and superior pharyngeal constrictor syndrome that produce common symptoms, making diagnosis difficult, which is often followed by ineffective or no treatment being provided to the patient.

The accumulation of symptoms

What these five conditions can have in common is TMJ, facial pain into the temple, ear pain, throat pain, painful or difficult swallowing, facial pain. In the many patients, we see cervical spine instability and ligament damage that is causing them significant and to some point “mysterious,” symptoms which I discuss below.

Patients come in and they discuss with us:

From our published research: Dextrose Prolotherapy and Pain of Chronic TMJ Dysfunction. Hauser R, Hauser M, Blakemore K. Dextrose prolotherapy and pain of chronic TMJ dysfunction. Practical Pain Management. 2007; November/December:49-55.


Ernest syndrome

This myriad of confusion surrounding the displayed symptoms leads to the understanding that patients may be suffering from a yet unidentified syndrome.

Ernest syndrome was first identified by the dentist, Edwin A. Ernest, III, DMD, in 1981 and subsequently written up in the 1982 edition of the medical textbook An Orthopedic & Neurological Approach to Diagnosis and Management, 1st Ed. 1982. pp. 114. It would be best to let Dr. Ernest describe the syndrome named after him. This comes from the October 2006 issue of the medical journal Practical Pain Management. (2)

The progression of symptoms of Ernest Syndrome typically presents as follows:

In the early stages, patients may present with symptoms of:

In the later stages, patients may present with symptoms of:

Ernest syndrome may not be so rare

Recently doctors at the University of Valencia in Spain wrote in the medical journal Neurología (3) that Ernest syndrome symptoms that they see:

Are common to the myriad of disorders that TMJ and cervical neck instability can produce, including the tenderness on pressing into “the behind the ear area,” while implicating a problem of Ernest Syndrome, will still and often confuse clinicians that Ernest Syndrome is being caused by other types of orofacial pain including TMJ. Ernest Syndrome  the researchers conclude: “may, therefore, be more prevalent than the literature would indicate.”

Stylomandibular ligament damage and Ernest Syndrome

In our clinics, we are very aware of what damaged ligaments can do, so it is reassuring to us that medical research is documenting some of the same things that we have seen over the decades. In regard to Ernest Syndrome, this problem may be much more prevalent than doctors think, so maybe we should not call this a “rare,” disorder. Further, patients with unresolved TMJ and jaw pain should be examined for stylomandibular ligament damage.

Four patients do well with cortisone

A September 2020 case report in The Journal of Craniofacial Surgery (4) examined the cases of four patients with chronic pain that radiated to the TMJ and temple and did not respond to conservative management. All patients after diagnosed with local anesthetic block were given methylprednisolone injection at the insertion of stylomandibular ligament. Complete remission of pain was seen at 12 months of follow-up-without any recurrence. Craniofacial surgeons involved in the treatment of various head and neck pain should include this less documented syndrome in their differential diagnosis when treating TMJ disorders.

Note: People do respond to cortisone. Here four people were able to avoid a TMJ surgery with cortisone. People like this are usually not the people we see at our center. We do see patients who have responded to cortisone injection but repeated injections did not help them.

Ross Hauser, MD presents an overview of diagnosis and treatment for Ernest Syndrome and Eagle Syndrome.

Eagle Syndrome is medically known as the elongation of the styloid process and stylohyoid ligament calcification. This typically occurs with aging and often results in sharp, intermittent pain along the glossopharyngeal nerve that is located in the hypopharynx and at the base of the tongue. This is a stylohyoid ligament problem and is further explained in our accompanying article on Eagle Syndrome.

Summary video transcript

Focus on the stylomandibular ligament and development of bone spurs at the styloid process 

Digital Motion X-Ray at 5:05 – the cervical spine instability connection is explored

Digital motion X-Ray C1 – C2

The digital motion x-ray is explained and demonstrated below

Treatments – Surgery or injections

In the October 2006 issue of Practical Pain Management, which I cited above, Dr. Ernest described two patients who were inappropriately operated on for TMJ. When TMJ issues did not resolve, the two patients were ultimately diagnosed with Ernest Syndrome resulting from motor vehicular trauma. Dr. Ernest summarized these case studies with this warning to doctors and dentists: “This paper serves to emphasize for physicians and dentists that inclusion of Ernest Syndrome in the differential diagnosis of ear symptoms, TMJ pain, and other craniofacial pains will help to prevent unwarranted surgery of the TM Joint.”

Another paper from Dr. Wesley E Shankland 2nd, described a detailed clinical analysis of 68 patients diagnosed as suffering from Ernest syndrome. (5) This is an often-cited paper by researchers exploring the problems of treatment of Ernest Syndrome.

While we will agree that injection therapy will help the patients, we would prefer the injection of simple dextrose as opposed to cortisone. Cortisone acts and an anti-inflammatory and is not curative in nature. Dextrose injections or Prolotherapy is an injection that seeks to strengthen weakened ligaments by causing inflammatory repair.

Prolotherapy Treatment of Ernest syndrome

In this section, we are going to discuss a possible treatment. That treatment is Prolotherapy. In simple terms, Prolotherapy repairs ligaments. In the case of Ernest syndrome, the cervical spine ligaments.

In the research and clinical observations explained above, we have been able to demonstrate that some neurological symptoms are related to the compression of the cervical nerves. In Eagle syndrome this compression may be caused by a weakened or damaged set of cervical ligaments, here focused on the stylohyoid ligament complex. Prolotherapy injections at the stylomandibular ligament bony attachments will start the repair process. Once the stylomandibular ligament is strengthened, the chronic ear-mouth pain, tinnitus, dizziness, vertigo, and other pain complaints subside.

Research on cervical instability and Prolotherapy

Caring Medical has published dozens of papers on Prolotherapy injections as a treatment in difficult to treat musculoskeletal disorders. Here are some.

In our paper Chronic Neck Pain: Making the Connection Between Capsular Ligament Laxity and Cervical Instability we wrote: 

“To date, there is no consensus on the diagnosis of cervical spine instability or on traditional treatments that relieve chronic neck instability issues like those mentioned above. In such cases, patients often seek out alternative treatments for pain and symptom relief. Prolotherapy is one such treatment that is intended for acute and chronic musculoskeletal injuries, including those causing chronic neck pain related to underlying joint instability and ligament laxity. While these symptom classifications should be obvious signs of a patient in distress, the cause of the problems are not so obvious. Further and unfortunately, there is often no correlation between the hypermobility or subluxation of the vertebrae, clinical signs or symptoms, or neurological signs or symptoms. Sometimes there are no symptoms at all which further broadens the already very wide spectrum of possible diagnoses for cervical instability.”

In other words, Prolotherapy may treat the very difficult to treat.

Further reading: Cervical Spine Realignment and restoring loss of cervical lordosis

In our article Cervical Spine Realignment and restoring loss of cervical lordosis, Dr. Hauser presents the case that cervical spine, upper cervical spine, and lower cervical spine instability may be an unexplored cause of your neurologic-like, vascular-like and psychiatric-like conditions and symptoms. It is very likely that you are reading this article as a continuation of your research into your symptoms and conditions and you came here from other pages on this website or that you landed here because a doctor, in many cases a chiropractor, mentioned to you that you have C1-C2 instability or C3-C7 instability. That this cervical instability has caused you to lose the natural curve or LORDOSIS in your neck. Further, that you have started to comes to terms with the understanding that of all the tests that you have had performed by neurologists and cardiologists and the prescriptions offered to you by psychiatrists have never offered a “grand unifying theory” or help in diagnosing what was really wrong with you.

Further reading: Symptoms and conditions of Craniocervical Instability

In this article, Symptoms and conditions of Craniocervical Instability, Dr. Hauser has put together a summary of some of the symptoms and conditions that we have seen in our patients either previously diagnosed or recently diagnosed with Craniocervical Instability, upper cervical spine instability, cervical spine instability, or simply problems related to neck pain.

For many of these people, symptoms, and conditions extended far beyond the neck pain, radiating pain of cervical radiculopathy, headaches, TMJ, and possibly dizziness usually associated with problems in the neck. These people’s symptoms and conditions extend into neurologic-type problems that may include digestion, irregular cardiovascular problems, hallucinogenic sensations, vision problems, hearing problems, swallowing problems, excessive sweating, and a myriad of others.

I want to point out that the symptoms and conditions you will see described below can be caused by many problems. This article demonstrates that craniocervical instability and cervical neck instability can be one of them.

Caring Medical research

Summary and contact us. Can we help you? How do I know if I’m a good candidate?

We hope you found this article informative and it helped answer many of the questions you may have surrounding Ernest Syndrome. Just like you, we want to make sure you are a good fit for our clinic prior to accepting your case. While our mission is to help as many people with chronic pain as we can, sadly, we cannot accept all cases. We have a multi-step process so our team can really get to know you and your case to ensure that it sounds like you are a good fit for the unique testing and treatments that we offer here.

Please visit the Hauser Neck Center Patient Candidate Form

References:

1 Shankland II WE. Anterior throat pain syndromes: causes for undiagnosed craniofacial pain. CRANIO®. 2010 Jan 1;28(1):50-9. [Google Scholar]
2 Ernest EE. Ernest Syndrome and Insertion of the SML at the Mandible. PPM 2006 Volume 6, Issue #7
3 Peñarrocha-Oltra D, Ata-Ali J, Ata-Ali F, Peñarrocha-Diago MA, Peñarrocha M. Treatment of orofacial pain in patients with stylomandibular ligament syndrome (Ernest Syndrome). Neurologia. 2013 Jun;28(5):294-8. doi: 10.1016/j.nrl.2012.06.009. Epub 2012 Aug 14. [Google Scholar]
4 Jose A, Rawat A, Nagori SA. Insertion Tendinosis of Stylomandibular Ligament: Ernest Syndrome. The Journal of Craniofacial Surgery. 2020 Sep 1. [Google Scholar]
5 Shankland WE. Ernest syndrome as a consequence of stylomandibular ligament injury: a report of 68 patients. Journal of Prosthetic Dentistry. 1987 Apr 1;57(4):501-6. [Google Scholar]

 

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