Costen’s syndrome – Mandibular joint neuralgia and cervical instability

Ross Hauser, MD

Costen’s syndrome is a somewhat controversial diagnosis. Controversial not in that the symptoms ascribed to it do or do not exist or controversial in the fact that the symptoms this condition describes cannot be treated. The controversy lies in that Costen’s syndrome can be confused with other problems related to the jaw, mouth and ear as it has many of the same symptoms as other mouth, jaw and ear related problems. These overlapping of symptoms can cause confusion in the manner of treatment for the patient.

In the world of temporomandibular disorder (TMD), there are several diagnostic terms that describe a painful problem at the Temporomandibular joint (TMJ). Costen’s syndrome is one of them. Costen’s syndrome is recognized as a problem that can be generated by ear problems including problems with constant noises or sounds generated from the ear and problems of space and balance generated by the signals of the vestibular (inner ear) balance system. What makes a diagnosis of Costen’s syndrome is that there is an apparent cause in the mouth. These can be poorly fitted dentures, overbites, dental occlusions, pain generating from the jaw. In many cases, once the mouth origins of the patient’s symptoms are addressed, then traditional treatments revolving around the resolution of temporomandibular disorder are typically prescribed. But what if the symptoms of Costen’s Syndrome are not coming from the mouth? What if they are coming from cervical spine instability?

Costen’s syndrome

In the video below, Ross Hauser, MD  discusses Costen’s syndrome and his experience treating patients with the various symptoms associated with mandibular joint neuralgia. This article will expand on this video and present explanatory notes.

It was in 1934 that a group of symptoms were first described by Dr. James Costen as “A syndrome of ear and sinus symptoms dependent upon disturbed function of the temporomandibular joint.”(1)

This image is explained below.

The image above describes that various symptoms ascribed to Costen’s Syndrome or Mandibular Joint Pain.

In the image above we see that Costen’s syndrome is a syndrome accepted by the dental profession as a real entity caused by dental malocclusion.

• Dental malocclusion then causes TMJ dysfunction
• TMJ dysfunction then causes a host of neurological symptoms. These symptoms can include:
  • Tinnitus
  • Otalgia (ear pain)
  • Eustachian tube dysfunction with:
    • Hearing loss
    • Ear fullness or stuffiness
    • Noises in their ear: Humming, ringing, crackling sounds. I have had people come in with squishing sounds, high-pitch sounds, low pitch sounds, vibratory sound, vibration.
• Head and neck pain
• Tongue pain or glossodynia
• Burning mouth syndromes symptom types.
• Nose and eye symptoms and pain
• Headaches, especially occipital headaches and occipital pain and top of the head or vertex headaches.
• Vertigo
• TMJ symptoms and pain

A 2021 study (15) confirmed that in compression and dislocation dysfunction of TMJ, there are disorders of the functional state of the salivary glands.

Dr. Costen noted some of  these symptoms and what he was trying to do was try to figure out why people had these neurologic symptoms and how it could relate to the mouth. He suggested that overbite or “deep bite” (malocclusion) would cause deterioration of the TMJ and stress the facial muscles. As the condition worsened, bone and cartilage damage would occur.

As noted in an October 2021 paper by Dr. Kamal G. Effat (14) “Costen concluded that all these symptoms could be attributed to disturbances in the TMJ, its ligaments, and associated muscles.” In supportive research Dr. Effat also connected:

• Hearing loss/ blocked-ear sensation in patients with Costen’s syndrome / TMD.
• Tinnitus in patients with Costen’s syndrome / TMD.
• Vertigo in patients with Costen’s syndrome / TMD.
• Headache in patients with Costen’s syndrome / TMD.
• Facial pain and associated autonomic symptoms in patients with Costen’s syndrome / TMD.

Dr. Effat concluded: “The central nervous system appears to play an essential role in the genesis of otological and alleged sinus symptoms associated with Costen’s syndrome. Maladaptive neuroplasticity in relevant brain pathways stresses the role of affective disorders in the experience of these symptoms associated with temporomandibular disorders.”

Dental malocclusion

Dental malocclusion has probably been described to you at length. Simply, your teeth do not line up. Many years ago, I personally saw many dentists. I had my teeth shaved down, many different types of splints applied, and various other treatments and applications. Over the course of my career, and after seeing many patients, it became apparent that the treatment of overbite or the focus on overbite for many patients was not the right route of treatment. Treating cervical spine instability was.

This point is shown in a patient we treated at Caring Medical. This patient suffered from Ehlers-Danlos Syndrome which caused her to be “over flexible” or “double jointed.” This included problems at the TMJ. We noticed that when this patient put her new mouth guard in, one made for her by her dentist, she had sudden reduced cerebellar function. She lost her balance, coordination, and had difficulties walking. All this occurred when she put the mouth guard in. The opposite effect on her symptoms was occurring. The mouth guard became detrimental to her well-being. This shows the problem for this patient ran deep into cervical instability.

How can this be? One possible explanation is that the mouth guard is causing blood flow disturbance. A 2015 study in the Journal of craniomandibular practice noted the benefits of using a mouth guard: “There has been much published evidence that balance can improve by changing the mandible’s position relative to the maxilla as it comes together with the teeth (or oral device) as the endpoint. . . . ”

What this study did was to examine “whether the physiologic rest position of the jaw (oral device overtop of the teeth as endpoint where the muscles of mastication are optimized) can have an effect on cerebral blood flow and physical balance.” The results of this research: “The physiologic rest position of the mandible might have an effect on balance by showing a trend (demonstrating a tendency) in enhancing cerebral blood flow as measured by transcranial Doppler.(2)

This is a positive aspect of the mouth guard. But why did this one patient suffer the opposite? Because while a mouth guard can increase cerebral blood flow, it can also decrease cerebral blood flow as clinically observed. This would indicate, in many, cervical spine instability and a TMJ situation in concert.

There has been a great deal of emphasis on occlusal devices in the treatment of TMJ because of the tremendous forces that occur with bruxism. Bruxism is defined as the clenching or grinding of the teeth during nonfunctional movements of the mandible. When it occurs during sleep, it is termed nocturnal bruxism. Most individuals engage in nocturnal bruxism activity at some point in their lives. The etiology of nocturnal bruxism by most traditionally-minded practitioners is that it is a sleep disorder related to the patient’s waking emotional state that is centrally mediated and precipitated by emotional stress. It occurs in greater frequency during periods of emotional or physical stress, as well as the anticipation of stress.

Almost everyone I have evaluated for bruxism has clicking and grinding in their TMJ.  While bruxism can cause TMJ, surely TMJ instability can cause bruxism. Most bruxism completely stops once the TMJ instability is resolved. It is imperative that it stops because nocturnal bruxism can generate incredible forces resulting in significant loads to the TMJ complex.

In the image above we describe the potential mechanisms by which TMJ problems can cause neurological-like symptoms or the symptoms of Costen’s Syndrome

It starts with TMJ instability which can lead to:

• Fascia, neck muscle tension
• Forward head posture or cervical kyphosis
• Cranial nerve dysfunction dysautonomia
• Hyoid bone instability
• Styloid-bone ligament calcification enlargement
• Eagle’s or Ernest syndrome

Another path TMJ instability can take in causing neurologic-like symptoms is

• Herniation into external auditory canal
• Discomalleolar ligament (pinto’s ligament” problems) Please see my article: TMJ and Tinnitus: Should we explore the ligament chain from the cervical spine through the neck to the jaw to the ear?
• Esthetician tube dysfunction

I want to pause here so I can briefly present where you can find more information on our website regarding many of the symptoms and disorders just mentioned:

• TMJ dysfunction  and neurological symptoms.
  • Please see my related articles: TMJ and Tinnitus: Should we explore the ligament chain from the cervical spine through the neck to the jaw to the ear? ,  The evidence and comparisons of TMJ injection treatments
• Tinnitus, Ear pain, Eustachian tube dysfunction:
  • Ear pain, ear fullness, sound sensitivity, Tinnitus, Meniere’s Disease and hearing problems caused by neck instability
  • Tinnitus, cervical spine instability, and neck pain
  • Neck pain Chronic Sinusitis and Eustachian Tube Dysfunction
• Tongue pain, Burning mouth syndromes symptom types.
  • Neck-Tongue Syndrome treatments
  • Is Burning Mouth Syndrome a problem of cerebral blood flow?
• Headaches
  •  Finding the missing cause of headaches, dizziness, and facial pain
  •  Occipital neuralgia and Suboccipital headache – C2 neuralgia treatments without nerve block or surgery
  •  Suboccipital headache Getting results from cervical ligament repair and treatment of muscle spasms
• Forward head posture or cervical kyphosis
  • Cervical Myelopathy – Cervical Degenerative Disc Disease – Cervicovagopathy – Cervical Dysstructure Broken neck structure
• Cranial nerve dysfunction – dysautonomia
  • Determining the Cause(s) of Dysautonomia, Vagopathy, and Autonomic Nervous System Imbalances
• Hyoid bone instability – Styloid-bone ligament calcification enlargement – Eagle’s  / Ernest syndrome
  • Eagle Syndrome Treatment – A Different non-surgical approach
  • Ernest Syndrome | Is this the answer to unresolved TMJ, facial, ear and throat pain?
• Nose symptoms
  • Empty nose syndrome
• Vertigo
  •  Cervical Vertigo and Cervicogenic Dizziness – Neck pain and dizziness
  •  Vestibular migraine and spontaneous vertigo – Migraine Associated Vertigo

Bruxism

According to various sources, 8 out of 10 patients coming to the dentist are found to have bruxism (grinding, gnashing or clenching of teeth) or TMD. Pain is the primary symptom of TMD and can occur in the TMJ, front of head, face, ear, forehead, temples, back of head or referred to the cervical spine and shoulder girdle, as well as in the thoracic, lumbar and sacral spine or legs.

The TMJ is unique; it’s the only articular joint of its type in the body. It is actually two joints in one, meaning there are two TMJs (right and left) just in front of the ears that hold the jawbone (mandible in place) and of course help align the teeth together. Teeth alignment can affect the TMJ, but generally the TMJ becomes injured from ligament laxity (whether from the forces of trauma, eating, or bruxism), causing TMJ instability. Once one TMJ has instability, it will affect the opposing TMJ.

Perhaps, the most devastating effects of bruxism are not on the teeth but on sleep. It has been well documented that bruxism causes sleep disturbances from pain, sleep apnea and gastroesopheal reflux.(12) Since continuous positive airway pressure (CPAP) treatment of sleep apnea has been shown to resolve nocturnal bruxism, it is debatable what came first the bruxism or the sleep apnea.(13) Clearly a person’s nervous system would be irritated if they became hypoxic from an obstruction in the pharynx or throat, but likewise a person with post-whiplash pain syndrome involving the TMJ and neck could also lead to bruxism and subsequent sleep problems.

When a person is wearing a mouth guard to reduce bruxism forces on the joint, this scenario plays out all night. While the force on the joint might be decreased due to the night guard appliance (occlusal splint), the joint is now open. So the likelihood of laxity in the lateral ligament increases as the muscles contract during the night because this ligament is pulling the articular disc back posterior while having to fight the pull of the lateral pterygoid on the disc anteriorly. What does this mean? This means that the bite guard you are wearing is most likely causing or increasing your TMJ instability and making your internal derangement worse.

This is an example of a traditional treatment helping reduce symptoms by decreasing the force on the joint, but ultimately it makes the soft tissue structures weaker to the point of causing instability. By forcing the mouth open during sleeping, there is a massive contraction all night of the muscles of mastication, especially the lateral pterygoid. Thus the person is actually increasing the chances of lateral TMJ instability and internal derangement of the internal disc as it gets pulled anteriorly by the lateral pterygoid and posteriorly by the lateral TMJ ligament.

Treatments of Costen’s syndrome

Doctors in Russia published a paper in The Korsakov’s Journal of Neurology and Psychiatry (3) where they made some interesting connections between Costen’s syndrome, cochleovestibular syndrome, and TMJ. First let’s explain cochleovestibular syndrome better. In a paper (4) from the Department of Otolaryngology, Vanderbilt University, cochleovestibular syndrome, is described. “Cochleovestibular nerve compression syndrome is the term used to describe a group of audiovestibular symptoms thought to be due to a vessel compressing the cochleovestibular nerve. These symptoms include recurrent vertigo, continuous disequilibrium and acquired motion intolerance. ”

Returning to the Russian study, the doctors wrote that their objective was to develop an integrated therapy for the combination of pain syndrome and cochleovestibular syndrome  in patients with temporomandibular joint dysfunction. They titled their paper “Comprehensive treatment of Costen syndrome.” What these doctors found was patients who received pharmacological and osteopathic therapy; splint therapy and orthodontic treatment in varying combinations of treatment had “less complaints of pain, tinnitus, dizziness and nystagmus.” Further  patients should be treated in joint participation of dentist, neurologist and ENT doctor with comprehensive, medical, orthodontic and osteopathic components.

A 2020 paper in the journal Advances in Dentistry & Oral Health (5) also suggested that conservative treatment should be always the first option for the treatment of Costen’s syndrome. Surgical treatment should be considered as the last option. Further the authors noted, few people are good surgical candidates. They researchers do note that “Recently, there has been a great evolution in results, especially in multi-disciplinarity care. Procedures such as occlusal adjustment, orthodontics, electrotherapy, botulinum toxin, laser therapy, pharmacological treatment, acupuncture, cryotherapy, heat therapy, muscle relaxant drugs, chiropractic, psychological treatment must be taken in account.”

BOTOX® 

Botulinum or BOTOX®  injection is often given in TMD treatments. Research surrounds its use in the lateral pterygoid muscle. A muscle that is responsible for chewing. A 2017 study in the Journal of dentistry (6) suggested moderate success in helping some patients with TMD related disorders, specifically pain from muscle spasm. A 2019 paper in the Journal of stomatology, oral and maxillofacial surgery (7) suggested Botulinum injections would help quality of life issues in patients for 1 to 3 months and would be better utilized as a supportive therapy to control pain. But what if the problems presented in Costen’s syndromes are not originating at the lateral pterygoid muscle, but the spasms of the lateral pterygoid muscle are the result of neck instability?

Electrotherapy or nerve stimulation

The use of electrotherapy or nerve stimulation therapy for TMD disorders has been studied for years. A 2015 paper in the journal Pain Physician (8) wrote: “Patients affected with TMJ syndrome who do not respond to conservative treatments may find a solution in peripheral nerve stimulation, a simple technique with a relatively low level of complications.”

A July 2021 study in the Journal of oral rehabilitation (9) found that low level laser had better short-term efficacy than TENS in the treatment of pain caused by TMD. Better results can be achieved with higher wavelengths.

Sleep and anxiety

In my experience, it isn’t malocclusion, it isn’t TMJ, it is a stress that these people suffer from. We talk to people in our office about reducing stress before sleep. This may reduce their teeth grinding or bruxism. Reducing external stressors is something we do discuss with patients.

A 2021 paper (10) studied “the role of patient’s psychological status as a significant factor influencing the occurrence and complications of TMD (Costen’s Syndrome), as evidenced by an increase in stress, anxiety, and depression in patients with the dysfunction.” The researchers noted that understanding the psychological status and developing treatment “can significantly alleviate the severity of the disease, reduce complications, and shorten the rehabilitation time.”

This was not the first paper to point this out. In 2016 researchers wrote (11) : “Disturbed sleep and psychological distress symptoms are common in TMD patients. Disturbed sleep, anxiety and stress are possible risk indicators for myofascial pain, compared with disc displacement and arthralgia or degenerative joint diseases.”

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• Surgery
• NSAIDs
• Cortisone Shots
• R.I.C.E.