What is Tensor Tympani Syndrome?
Tensor tympani syndrome (TTS), sometimes called tensor tympani myoclonus, is a rare condition that leads to a condition known as objective pulsatile tinnitus. This means you perceive rhythmic sounds that sync up with your heartbeat. This happens because a specific muscle in the middle ear, known as the tensor tympani muscle, contracts involuntarily. This muscle is involved in certain hearing processes, such as controlling the movement of a tube in the ear (the eustachian tube) and making the eardrum rigid to decrease noise transmission when exposed to loud sounds.
Now, tinnitus is when you perceive sound that doesn’t come from any outside source. When doctors try to understand tinnitus, they need to identify if it’s subjective or objective and if it’s pulsatile or non-pulsatile. Subjective tinnitus is far more common and is when only you can hear the sound. But with objective tinnitus, both you and potentially the doctor examining you can hear the sound. The kind of hearing loss where you experience a ringing or buzzing noise, known as sensorineural hearing loss, usually results in subjective tinnitus. The sound is only perceptible to you, and not anyone else examining you.
Next, the doctor tries to figure out if your tinnitus is pulsatile (syncing up with your heartbeat) or non-pulsatile. Pulsatile tinnitus is often caused by vascular-related abnormalities, like certain forms of high blood pressure, benign brain pressure conditions, or tumors. These may or may not sync up with the heartbeat. Sensorineural hearing loss, a bone condition of the ear called otosclerosis, and a type of tumor known as acoustic neuromas, can cause non-pulsatile tinnitus. The tensor tympani syndrome is a particular type of pulsatile, objective tinnitus. This means the doctor examining you can hear or witness the tinnitus or muscle twitching while checking your symptoms. It’s sometimes hard for a doctor to observe these contractions and tinnitus firsthand, so they mostly rely on your medical history. They try to listen for the tinnitus and examine the structures of the ear and the eustachian tube.
The tensor tympani muscle receives its instructions via the fifth cranial nerve (CN V), which also controls other muscles involved in chewing and swallowing. This nerve also controls the movement of the tensor tympani muscle, which consequently controls sound intensity while chewing and swallowing. Everyday activities like chewing or swallowing can possibly trigger the tensor tympani muscle, resulting in tensor tympani syndrome, a type of condition characterized by the rhythmic contraction of the muscle in the middle ear (middle ear myoclonus). Another muscle known as the stapedius muscle can also cause this condition. This muscle controls the movement of the stapes, a tiny bone in the ear, to reduce the transmission of loud noises. When your ears are exposed to high-intensity sounds, the tensor tympani and stapedius muscles contract involuntarily as part of a mechanism called the acoustic reflex, which is believed to shield the inner ear cells from damage.
What Causes Tensor Tympani Syndrome?
The tensor tympani syndrome and middle ear myoclonus are conditions affecting the ear. They can be caused by several factors, including unidentified sources, blood vessel problems, disorders that damage the protective covering of nerve fibers (known as demyelinating disorders), trauma, tumors, or infections. There have been many documented cases where people with multiple sclerosis, a demyelinating disorder, also had tensor tympani syndrome in both ears.
One of the main symptoms of these conditions is tinnitus, which is a fancy name for hearing noises or ringing in the ears when no external sound is present. However, exactly how these conditions cause tinnitus is not completely clear yet.
There’s a specific form of tensor tympani syndrome, called tonic tensor tympani syndrome (TTTS), which causes spasms of the tensor tympani, a small muscle in the ear. These spasms often result in tinnitus and an increased sensitivity to sound, known as hyperacusis.
There is a belief that TTTS might be an involuntary condition – that is, it’s something that people can’t control. This belief is based on the observation that people with anxiety disorders often have TTTS. It’s thought that the anxiety causes the tensor tympani muscle to be more easily triggered, leading to the symptoms of TTTS.
Risk Factors and Frequency for Tensor Tympani Syndrome
Tensor tympani syndrome is a condition about which little is known, including its prevalence or who it impacts most often. What we do know is that there is no clear preference for any gender when it comes to middle ear myoclonus, which is similar in nature to this syndrome. Initial information shows that this issue usually crops up more often around the age of 30.
Signs and Symptoms of Tensor Tympani Syndrome
If someone complains of tinnitus, which is a ringing or buzzing sound in the ears, it’s crucial to consider various possibilities since tensor tympani myoclonus, a rare condition related to tinnitus, can often be overlooked. To accurately diagnose this, it is important to record when the tinnitus started, whether it’s in one or both ears, how long it lasts, any related symptoms, and if it is triggered by certain activities, such as loud noises or changes in head position. Even though tensor tympani myoclonus is an uncommon cause of tinnitus, various tests may be needed to rule out more familiar and potentially serious causes of tinnitus.
The tensor tympani and tensor veli palatini (TVP) muscles, which are both supplied by the fifth cranial nerve, play a significant role in this condition. The tensor tympani muscle attaches to the malleus, one of the small bones in the middle ear. When this muscle goes into spasms, the malleus is pulled in a certain direction, causing the eardrum to move inward. These movements can be observed with an otoscope, a medical device used to look into the ears. The TVP muscle helps to lift the palate when swallowing, so its activation could also trigger tinnitus in some cases.
The fifth cranial nerve also supplies the corneal reflex, a safety mechanism of the eye. In some cases, this reflex can trigger muscle spasms in the tensor tympani muscle, resulting in a condition called tensor tympani syndrome. To diagnose this condition, doctors may look for rhythmic movements of the eardrum during a rest state or even when blinking. They will also need to rule out any other conditions that might be causing the tinnitus. In particular, they’ll need to verify if the eustachian tube, a canal that links the middle ear with the back of the nose, is unusually open and causing any echoing of the patient’s own voice. The most reliable way to diagnose any muscle-related disorders in the middle ear involves directly observing the muscle spasm via a procedure known as a tympanotomy. However, this method heavily depends on having the examination at a time when the patient is experiencing the spasm.
Testing for Tensor Tympani Syndrome
If your doctor is suspecting you may have tensor tympani syndrome, a rare condition that could cause symptoms like tinnitus (ringing in the ears), they will first ask about your medical history and do a physical examination. Following that, they may conduct a number of tests to exclude other potential causes for your symptoms.
Because tensor tympani syndrome is so rare, there isn’t a clear-cut way to diagnose it. That said, some experts have proposed a method for figuring out if a patient might have it. The types of tests they might use aren’t necessarily meant to diagnose tensor tympani syndrome, but they can help with the treatment and rule out other conditions.
One of these tests is audiometry. Audiometry can’t directly diagnose tensor tympani syndrome, but it can be a tool in the treatment process and help rule out other possible causes of the ringing in your ears.
Next, they might use a test called tympanometry. This test measures the pressure in your middle ear. If the tensor tympani muscle (a tiny muscle inside your ear) isn’t contracting when it’s supposed to, the tympanometry test might not pick up on it. So, in some cases, your doctor might use a form of tympanometry that measures pressure over an extended period. If this test shows a specific pattern, resembling a saw tooth on a graph that represents the movement of your eardrum, it could be a clue you have tensor tympani syndrome.
Your doctor might also do an impedance audiogram to understand the health of your middle ear, which could help in diagnosing the condition.
To exclude other potential causes of tinnitus, your doctor might order imaging tests like magnetic resonance imaging (MRI) or a computed tomography (CT) scan. These tests will help them see detailed images of your ear and eliminate other possible conditions that could be causing your symptoms.
Treatment Options for Tensor Tympani Syndrome
In layman’s terms, middle ear myoclonus (MEM) is a rare condition involving repetitive and involuntary contractions of muscles in the middle ear, causing an array of symptoms. The severity of these symptoms and what the patient hopes to achieve with treatment can determine the course of action for treating the condition, specifically in cases of tensor tympani syndrome, a form of MEM.
Treatment for this disorder doesn’t come with a strict set of guidelines and we lack definitive data on its effectiveness. However, there are several medical and surgical options available to help reduce the symptoms. The treatment options include usage of medication, with each medication working differently. Some of the commonly used medications include benzodiazepines and orphenadrine citrate. They can potentially alleviate symptoms by reducing anxiety and also functioning as muscle relaxants.
Another treatment method works with a substance called botulinum toxin, which prevents the release of specific chemicals that trigger muscle contractions. It is a relatively new treatment for MEM, but it has shown promise in other otolaryngology-related conditions, like facial spasms and voice box disorders (also known as laryngeal pathology). Let’s note that the results provided by botulinum toxin for MEM are still under review given the limited usage of this treatment option to date. In some reported instances, its effects on reducing symptoms appeared to work for about three months. But like with any treatment methods, its efficiency may vary from patient to patient.
Surgical intervention also helps to manage this syndrome. The most common procedure related to tensor tympani syndrome and stapedius myoclonus, another form of MEM, typically involves making a small incision in the ear (tympanotomy) and cutting the tendons of overactive ear muscles (tenotomy). This is believed to minimize or even eliminate some symptoms like tinnitus (ringing in the ears). So far, studies have shown that a significant number of patients have experienced symptom relief from this procedure.
In a series of patient cases, it was found that three-quarters of patients who were first treated with medication reported reduced symptoms based on different evaluation scales. Among the rest who didn’t improve with medication alone, the majority underwent the surgical procedure, reporting improvement afterwards. It should be noted, however, that the collected data is quite limited for now, and more research is needed to get a clearer picture of how effective these different treatments are.
In conclusion, although more studies are required to confirm the efficiency of these methods, some suggest starting with a three-month trial of medication and supportive therapy. If the symptoms still persist afterwards, surgical intervention could be considered.
What else can Tensor Tympani Syndrome be?
When a doctor is examining a patient who is experiencing ringing or buzzing noises in their ear, known as tinnitus, their past medical history and current health checkup will help pinpoint the likely cause. A medical expert needs to think about all possible causes of tinnitus that can be detected by hearing or touch during an examination.
Conditions such as Stapedius myoclonus and Palatal myoclonus, often found in patients with Tensor Tympani Syndrome, should be highly considered. These conditions can all present similar symptoms. A medical history review, a physical check-up, and a specific hearing test known as tympanometry can guide a doctor towards one or more potential diagnoses.
However, the most effective way to confirm the diagnosis of Tensor Tympani Syndrome is by directly observing a muscle spasm in the middle ear. Doctors also need to take into account other conditions which might cause similar symptoms. These include:
- A patulous Eustachian tube, which means the tube is abnormally open
- Arteriovenous fistula or malformations, which are abnormal connections between arteries and veins
- Glomus tumors, which are tumors occurring in the ear or at the base of the skull
- Carotid artery stenosis, which is narrowing of the neck arteries
- Other conditions related to artery hardening (atherosclerotic diseases)
However, hearing tests and imaging techniques like MRI or CT scan can help more definitively distinguish these other conditions, as they may present with different medical history and physical examination findings.
What to expect with Tensor Tympani Syndrome
Tinnitus, a condition where you hear a constant ringing, buzzing, or other noise in your ears, is not directly harmful. However, it can sometimes be an indication of a more serious underlying health issue like multiple sclerosis. Beyond this, tinnitus can result in emotional distress for people, with effects ranging from severe debilitation to little or no distress at all. It’s also important to note that we don’t yet have enough data to predict whether tinnitus symptoms will lessen or stop over time, even if we know what’s causing the condition.
