Tonic tensor tympani syndrome | |
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Other names | TTTS |
Tonic tensor tympani syndrome is a disease of the tensor tympani muscle, described by Klochoff et al. in 1971. [1] [2] The tensor tympani muscle is one of the two middle ear muscles that support the three middle ear bones, called the ossicles. TTTS involves tensor tympani muscle activity being reduced, leading to a decrease in the contraction threshold of the tensor tympani which is exaggerated by high stress levels. This hyper-contraction (or spasms) leads to chronic ear pain, in particular in the case of hyperacusis [3] and acoustic shock. [4] TTTS is considered to be a secondary consequence of temporomandibular disorder and temporomandibular joint dysfunction.
TTTS can present as a variety of symptoms, including sensation of fullness in the ear, tinnitus, rhythmic sounds such as clicks and flutter of the tympanic membrane, ear fullness, a frequent "popping" sensation, hyperacusis, and mild vertigo. [3] These symptoms tend to last for a short period of time. Long-term symptoms include muffled and distorted hearing, ear pain, ear numbness, and burning sensations in and around the ear down to the neck. Burning sensations can extend to the cheek and jaw area. [5] In many people with tinnitus and almost all people with significant hyperacusis, an involuntary hyperactivity or myoclonus (spasm) appears to develop in the tensor tympani muscle as an involuntary 'protective' response to sounds (or other stimuli) subconsciously evaluated as potentially painful. [6]
TTTS can occur involuntarily following exposure to an acoustic incident, which is exposure to a sudden and unexpected loud sound that is perceived as threatening. However, TTTS can also occur following simple actions actions that include the middle ear muscles like talking, chewing, swallowing, or even being startled. [7] Eustachian Tube Dysfunction, which is common amongst those with a history of chronic ear infections, sinus infections, or seasonal allergies can also initiate TTTS due to the disruption of the function of the middle ear system. TTTS symptoms have also been reported in patients with trigeminal nerve irritability or trigeminal neuralgia, which are conditions that affect the nerves of the auditory system.
The tensor tympani muscle is located in the middle ear, and is one of the two middle ear muscles that support the three middle ear bones, the ossicles. The tensor tympani is attached to the malleus, the largest of ossicles. One end of malleus is connected to the tympanic membrane, and the other end is connected to the two other ossicles, the incus and stapes, which connects to the inner ear. The purpose of the middle ear is to deliver the sound waves from the outer ear to the cochlea of the inner ear. When external sound contacts the eardrum, the ossicles vibrate. This vibration can be amplified depending on the volume of the sound. When sound approaches over 70 decibels, the tensor tympani muscle contracts to assist in stabilizing the ossicles and reduce their vibration, which reduces the perceived volume of sound. These actions are called middle ear muscle reflexes or acoustic reflexes. [8]
The tensor tympani is meant to pull the tympanic membrane inward, increasing tension of the tympanic membrane. It's like a calming mechanism that's in place in order to stabilize volume and respond to noise fluctuations, that also provides inner ear protection from loud sounds. It also helps ventilate the inner ear through the Eustachian Tube. When TTTS occurs, the middle ear is contracting involuntarily, which produces an audible sensation called the middle ear myoclonus. When the tensor tympani muscle experiences a spasm without the provocation of loud sounds, it's called the acoustic reflex, which is part of a normal auditory system. [9] However, this reaction can be caused by general anxiety, or anxiety concerning the sudden onset of noises or ear pain, which can follow an acoustic incident.
An otolaryngologist, or ENT, is a healthcare specialist who is able to diagnose TTTS. A challenge in obtaining a diagnosis is being that there is not yet any definitive guideline to assess for the disorder. However, an impedance angiogram can be helpful while diagnosing TTTS by assessing the status of the middle ear. Other diagnostic tools like MRI and CT can be helpful in ruling out other potential causes of TTTS symptoms. A tympanometry is a diagnostic tool that is able to assess the pressure of the middle ear, but if the tensor tympani is not actively contracting, tympanometry will likely not observe it. [10] TTTS can be commonly mistaken for temporomandibular joint disorders.
Management of patients with TTTS varies depending on the severity of symptoms and expectations. [11]
The most common procedure for tonic tensor tympani syndrome is tympanotomy with tensor tympani tenotomy. This is a relatively simple surgical procedure that involves endoscopically cutting the tensor tympani muscle to eliminate spasms. A tensor tympani tenotomy is the preferred method of treatment due to being a safe and reliable procedure with very high success rates, providing longterm relief from TTTS. [12]
Medications like benzodiazepine s, botulinum toxin, piracetam, and carbamazepine have been used for treatment of TTTS with variable efficacy. Benzodiazepines target the anxiolytic component of the disorder and acts as a muscle relaxant, potentially decreasing symptoms. Botulinum toxin prevents the release of acetylcholine from pre-synaptic neurons and is well-documented in the use of non-cosmetic otolaryngology-associated disease processes like hemi-facial spasms. However, researchers are still examining the efficacy of botulinum toxin for TTTS. [13]
Holistic treatment options include relaxation techniques, sound, and tinnitus restraining therapies. Methods of relaxation are frequently included in comprehensive treatment plans with TTTS, and can aid in changing the way the brain reacts to the pain of a TTTS episode. Sound therapy can help with symptom desensitization, which can help reduce the ear pain and discomfort associated with TTTS, and improve anxiety levels [8] . Some patients have also seen relief with botox injections to help reduce tonic tensor tympani spasms. Holistic approaches are typically least desired by patients with TTTS due lack of definitive success rates and varying results.
Gender disparities for TTTS are not apparent, a multi-clinic study stated that its patients were 51.9% male and 48.1% female. [3] When the disorder is not the result of an acoustic incident, it can more commonly occur in the third decade of life. No other demographic disparities have been observed in TTTS. The number of how many people affected by TTTS hasn't yet been documented. However, around 83% of those with TTTS also suffered from hyperacusis. [3]
TTTS is not necessarily life-threatening in any way. The symptoms are unpleasant, but TTTS is not a harmful disorder when it exists alone. TTTS can be mentally debilitating, causing a high amount of distress in those who suffer from it, reducing quality of life. Tonic tensor tympani syndrome can occur at any age, with the chances of occurrence raise drastically if an acoustic incident takes place. [14]
One upcoming treatment that is in the process of being developed is Percutaneous Electrical Nerve Stimulation (PENS). As of 2024, there are no public literature for this treatment that is currently being developed and tested in Tampa, Fl by Dr. Josh Hanson, DACM. [15]
An article on TTTS refers to a study containing 44 patients that were initially treated with medications for treatment. 65% of the patients reported reduced symptoms, and 9 out of the 11 patients who did not improve underwent a tenotomy, where the results were unspecified. [16]
In a multi-clinic prevalence study, the prevalence of co-existing disorders and an acoustic incident trigger for TTTS were both studied. It was shown that 42.3% of patients had hyperacusis in addition to TTTS, and 24.1% of patients reported having initially developed TTTS following an acoustic incident trigger. [3]
Ramsay Hunt syndrome type 2, commonly referred to simply as Ramsay Hunt syndrome (RHS) and also known as herpes zoster oticus, is inflammation of the geniculate ganglion of the facial nerve as a late consequence of varicella zoster virus (VZV). In regard to the frequency, less than 1% of varicella zoster infections involve the facial nerve and result in RHS. It is traditionally defined as a triad of ipsilateral facial paralysis, otalgia, and vesicles close to the ear and auditory canal. Due to its proximity to the vestibulocochlear nerve, the virus can spread and cause hearing loss, tinnitus, and vertigo. It is common for diagnoses to be overlooked or delayed, which can raise the likelihood of long-term consequences. It is more complicated than Bell's palsy. Therapy aims to shorten its overall length, while also providing pain relief and averting any consequences.
Bell's palsy is a type of facial paralysis that results in a temporary inability to control the facial muscles on the affected side of the face. In most cases, the weakness is temporary and significantly improves over weeks. Symptoms can vary from mild to severe. They may include muscle twitching, weakness, or total loss of the ability to move one or, in rare cases, both sides of the face. Other symptoms include drooping of the eyebrow, a change in taste, and pain around the ear. Typically symptoms come on over 48 hours. Bell's palsy can trigger an increased sensitivity to sound known as hyperacusis.
This is a glossary of medical terms related to communication disorders which are psychological or medical conditions that could have the potential to affect the ways in which individuals can hear, listen, understand, speak and respond to others.
The middle ear is the portion of the ear medial to the eardrum, and distal to the oval window of the cochlea.
The outer ear, external ear, or auris externa is the external part of the ear, which consists of the auricle and the ear canal. It gathers sound energy and focuses it on the eardrum.
The ossicles are three irregular bones in the middle ear of humans and other mammals, and are among the smallest bones in the human body. Although the term "ossicle" literally means "tiny bone" and may refer to any small bone throughout the body, it typically refers specifically to the malleus, incus and stapes of the middle ear.
Tinnitus is a condition when a person hears a ringing sound or a different variety of sound when no corresponding external sound is present and other people cannot hear it. Nearly everyone experiences faint "normal tinnitus" in a completely quiet room; but this is of concern only if it is bothersome, interferes with normal hearing, or is associated with other problems. The word tinnitus comes from the Latin tinnire, "to ring". In some people, it interferes with concentration, and can be associated with anxiety and depression.
In the anatomy of humans and various other tetrapods, the eardrum, also called the tympanic membrane or myringa, is a thin, cone-shaped membrane that separates the external ear from the middle ear. Its function is to transmit changes in pressure of sound from the air to the ossicles inside the middle ear, and thence to the oval window in the fluid-filled cochlea. The ear thereby converts and amplifies vibration in the air to vibration in cochlear fluid. The malleus bone bridges the gap between the eardrum and the other ossicles.
The Eustachian tube, also called the auditory tube or pharyngotympanic tube, is a tube that links the nasopharynx to the middle ear, of which it is also a part. In adult humans, the Eustachian tube is approximately 35 mm (1.4 in) long and 3 mm (0.12 in) in diameter. It is named after the sixteenth-century Italian anatomist Bartolomeo Eustachi.
Myoclonus is a brief, involuntary, irregular twitching of a muscle, a joint, or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus describes a medical sign and, generally, is not a diagnosis of a disease. It belongs to the hyperkinetic movement disorders, among tremor and chorea for example. These myoclonic twitches, jerks, or seizures are usually caused by sudden muscle contractions or brief lapses of contraction. The most common circumstance under which they occur is while falling asleep. Myoclonic jerks occur in healthy people and are experienced occasionally by everyone. However, when they appear with more persistence and become more widespread they can be a sign of various neurological disorders. Hiccups are a kind of myoclonic jerk specifically affecting the diaphragm. When a spasm is caused by another person it is known as a provoked spasm. Shuddering attacks in babies fall in this category.
In vertebrates, an ear is the organ that enables hearing and body balance using the vestibular system. In humans, the ear is described as having three parts: the outer ear, the middle ear and the inner ear. The outer ear consists of the auricle and the ear canal. Since the outer ear is the only visible portion of the ear, the word "ear" often refers to the external part (auricle) alone. The middle ear includes the tympanic cavity and the three ossicles. The inner ear sits in the bony labyrinth, and contains structures which are key to several senses: the semicircular canals, which enable balance and eye tracking when moving; the utricle and saccule, which enable balance when stationary; and the cochlea, which enables hearing. The ear canal is cleaned via earwax, which naturally migrates to the auricle.
Piriformis syndrome is a condition which is believed to result from nerve compression at the sciatic nerve by the piriformis muscle. It is a specific case of deep gluteal syndrome.
The acoustic reflex is an involuntary muscle contraction that occurs in the middle ear in response to loud sound stimuli or when the person starts to vocalize.
Hyperacusis is an increased sensitivity to sound and a low tolerance for environmental noise. Definitions of hyperacusis can vary significantly; it often revolves around damage to or dysfunction of the stapes bone, stapedius muscle or tensor tympani (eardrum). It is often categorized into four subtypes: loudness, pain, annoyance, and fear. It can be a highly debilitating hearing disorder.
The stapedius is the smallest skeletal muscle in the human body. At just over one millimeter in length, its purpose is to stabilize the smallest bone in the body, the stapes or stirrup bone of the middle ear.
The tensor tympani is a muscle within the middle ear, located in the bony canal above the bony part of the auditory tube, and connects to the malleus bone. Its role is to dampen loud sounds, such as those produced from chewing, shouting, or thunder. Because its reaction time is not fast enough, the muscle cannot protect against hearing damage caused by sudden loud sounds, like explosions or gunshots, however some individuals have voluntary control over the muscle, and may tense it pre-emptively.
Stiff-person syndrome (SPS), also known as stiff-man syndrome, is a rare neurological disorder of unclear cause characterized by progressive muscular rigidity and stiffness. The stiffness primarily affects the truncal muscles and is characterised by spasms, resulting in postural deformities. Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms.
The tympanic cavity is a small cavity surrounding the bones of the middle ear. Within it sit the ossicles, three small bones that transmit vibrations used in the detection of sound.
Acoustic shock is the set of symptoms a person may experience after hearing an unexpected, loud sound. The loud sound, called an acoustic incident, can be caused by feedback oscillation, fax tones, or signalling tones. Telemarketers and call centre employees are thought to be most at risk.
The semicircular canal dehiscence (SCD) is a category of rare neurotological diseases/disorders affecting the inner ears, which gathers the superior SCD, lateral SCD and posterior SCD. These SCDs induce SCD syndromes (SCDSs), which define specific sets of hearing and balance symptoms. This entry mainly deals with the superior SCDS.