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:

  • Tenderness and discomfort below the ear lobe
  • Pressure simulating a new wisdom tooth trying to squeeze in.

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

  • Molar teeth on the same side ache and throb
  • Ear exhibits a sense of fullness and pain
  • Throat soreness
  • TMJ condyle pain
  • Coronoid process and temporal tendon pain at zygomatic arch. (The Coronoid process is where the jaw’s powerful temporal muscle attaches to the lower jaw. It attaches by way of the temporal tendon. If the temporal tendon is damaged, the muscle will not function correctly and the damage will cause pain at the zygomatic arch, more simply the cheekbone area.)
  • Temple headache
  • Eye pain or pain near the eye and photophobia ( light sensitivity or intolerance to light).
  • Ernest Syndrome can occur with or be mistaken for including Barre-Lieou syndrome.

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:

  • the preauricular area (external ear)
  • mandibular angle (jaw),
  • pain radiating to the neck, shoulder, and eye on the same side.
  • palpation of the stylomandibular ligament, gently pressing on it, will cause pain.

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

  • When we see patients with unresolved TMJ pain we think of injury to the stylomandibular ligament. This injury can cause some incredible symptoms like those problems mentioned above because of the impact this injury creates on the cranial nerves, specifically the glossopharyngeal nerve, the vagus nerve, and the Hypoglossal nerve (Cranial nerve XII).
  • The Styloid process. 
    • The styloid process is a small bone that projects out from the temporal bone at the base of the skull. Below the TMJ joint. It sits just below your ear. It is here that there a number of ligament and tendons attach themselves to provide movement for the jaw.
      • stylohyoid ligament
      • stylomandibular ligament
      • styloglossus muscle (tongue muscle which pulls upsides of the tongue to form a scoop which aids in swallowing. It is innervated by the hypoglossal nerve.)
      • stylohyoid muscle (which helps elevate the tongue. It is innervated by the facial nerve Cranial nerve VII).
      • stylopharyngeus muscle (vital to the swallowing process. It is innervated by the glossopharyngeal nerve).

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

  • (0:56) Focus on the stylomandibular ligament
    • The stylomandibular ligament is a really important ligament even though it doesn’t get a lot of publicity. It is important, especially now, that we use cell phones and are in a constant head down position. The stylomandibular ligament holds the mandible in place. When it is damaged, the mandible “floats,” and is unstable.
    • (3:18) Bone spurs. When the stylomandibular ligament is under constant pressure from instability of the mandible or pressure on the mandible exhibited in the head-down position, it distributes this stress into the styloid process. This stress causes the development of bone spurs. Formation of bone spurs at the styloid process can impinge on the vagus nerve, the glossopharyngeal nerve,  and the hypoglossal nerve. This impingement can be a leading culprit in the myriad of symptoms the patient suffers from.

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

  • Once a physical examination and palpation of the stylomandibular ligament reveal tenderness and pain. We will look at the patient’s cervical spine under digital motion x-ray and high definition ultrasound of the TMJ and cervical spine area. Many of the symptoms displayed in patients with suspected Ernest Syndrome are also symptoms of cervical spine instability. This connection is described further below.

Digital motion X-Ray C1 – C2

The digital motion x-ray is explained and demonstrated below

  • Digital Motion X-ray is a great tool to show instability at the C1-C2 Facet Joints
  • The amount of misalignment or “overhang” between the C1-C2 demonstrates the degree of instability in the upper cervical spine.
  • This is treated with Prolotherapy injections (explained below) to the posterior ligaments that can cause instability.
  • At 0:40 of this video, a repeat DMX is shown to demonstrate the correction of this problem.

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.

  • “Injury to the stylomandibular ligament is a real and frequent disorder causing craniomandibular pain.”
  • Of the patients in this study, 77.94% were treated successfully via nonsurgical management of their complaints. Resolution of this disorder is usually accomplished by a combination of a diagnostic injection of local anesthetic at the insertion of the ligament, localized injection of cortisone substitute, and placing the patient on a soft diet.

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.

Caring Medical research

If you have a question about Ernest Syndrome, get help and information from our Caring Medical staff

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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