Neurogenic

SPASMODIC DYSPHONIA:

Overview:

Spasmodic Dysphonia (SD) is part of  a "family" of disorders called "Dystonias" or "Focal Dystonias". A dystonia is when a single muscle or small group of muscles contracts involuntarily and spontaneously, this is when the group of muscles is classified as dystonic.  Dystonias can affect a large set of muscles or be focal.  In this case we will discuss dystonia relating to the voice, but they can relate to the entire body.

Spasmodic Dysphonia (SD) aka: Laryngeal Dystonia, is a common name for a neurologic, involuntary, movement disorder. It is caused by problems in the nervous system. Dystonias can affect many parts of the body and in these qualifications it is affecting the Larynx or vocal folds, they vibrate normally, but spasm during speech.

Here is a video that shows what spasmodic dysphonia looks like.

Cause:

There is no known cause of Dystonia, however it is thought to be linked to genetics (mutation of the DYT1 gene), trauma or certain medications.

NOTE: Spasmodic dysphonia was called “spastic dysphonia" up until about 15 years ago, but now it is Spasmodic Dysphonia or Laryngeal Dystonia.

**Two kinds of laryngeal spasms cause three kinds of Spasmodic Dysphonia.

3 Types of Spasmodic Dysphonia:

Adductor SD:

This is the most common type and this affects the muscle that lies within the vocal folds, the thyroarytenoid muscle, it contracts strongly, but then spasms. The vocal folds squeeze together very tightly, causing a strained, strangled and harsh voice with voice arrests (stopping of the voice).

Abductor SD:

This causes the muscle that brings the vocal folds together or the cricoarytenoid muscle to contract suddenly. This produces a breathy voice and a lot of excess air coming out of the vocal folds. This is the less common form of SD.

Mixed SD:

This is when both types of spasms appear during speech, adductor and abductor. This is the most rare form of these three types.

Voice Quality

Adductor SD: tight, tense, strain strangled with sudden voice stoppages

Abductor SD: breathy, weak voice with sudden bursts or blowouts of air flow.

Symptoms:

People with these types of disorders complain of:

• extreme fatigue
•  effortful voice
• strain/strangled
• no control over their voice
• unintelligible

Treatment:

Currently, there is no cure for Dysphonia, however there are several treatment options.

Oral Medications:

Oral medications provide little relief of Spasmodic Dysphonia, but if one has other Dystonic symptoms sometimes they can help. Medications such as:  Sinemet® (carbidopa/levodopa), Ativan® (lorazepam), Klonopin® (clonazepam), Neurontin® (gabapentin), and Valium® (diazepam), are used to settle muscles or nerves with excess activity. Other categories of medications used in the treatment of dystonia include anticholinergics, benzodiazepines, and baclofen. (www.dysphonia.org).

Botox Injections:

Injection therapy such as Botox (Botulinum Toxin) is found to be effective.

It is most helpful for Adductor Spasmodic Dysphonia. The Botox injections weaken the vocal folds so they don't spasm as much and they occur much less..
After injection the voice quality is breathy and weak due to the weak vocal folds from the Botox, however, over the course of a few weeks the voice becomes more "normal" without spasm. This treatment is noted to last three to four months, so eventually the spams return and more Botox must be injected.

In the case of Abductor Spasmodic Dysphonia the Botox is injected into the Cricoarytenoid muscle to assist in decreasing the spasms, this lasts for approximately three-four months and again re-injection of Botox is needed.

Speech or Voice therapy is helpful in assisting the person to use the voice correctly and reduce their compensating behaviors.

What are the side effects of  Botox and the nervous system?

Botox is injected in such small amounts and only the muscles near the injection site are affected. When our nerves get the signal from the brain to "fire"- neurotransmitters are released to the muscle fibers causing them to contract.  Botox injections prevent the muscles from contracting by weakening them, NOT ENTIRELY eliminating the contractions. Botox doesn't necessarily ware off, but the nerve fibers eventually die from the Botox and new nerve endings grow and this allows the release of neurotransmitters again, hence the need for another injection of Botox..

 These include Benign Essential Tremor and severe Muscle Tension Dysphonia.

Surgeries:

There are a few surgical procedures to help Spasmodic Dysphonia, specifically Adductor SD, there are few procedures to assist Abductor SD.

Thyroplasty: This procedure is done to physically separate the vocal folds in a person Adductor SD, to prevent them from contacting one another at midline. Results vary from case to case, however it is known to result in a weak voce, but imrpoved fluency.

Denervation Operation:

This is an operation in which the doctor cuts the Recurrent Laryngeal nerve (part of the Vagus Nerve (Cr. 10): the nerve that goes from the brain stem to the larynx), this causes weakness of the larynx on one side and also can produce even further hoarseness.  It has varying results among patients.

Selective Laryngeal Adductor Denervation-Rennervation (SLAD-R):

In this procedure the surgeon cuts the nerves on both sides of the larynx, which weakens the muscles. The recurrent laryngeal nerve gets cut away from the thyroarytenoid (TA) and lateral cricoarytenoid(LCA) muscles. An unaffected nerve is connected to the "stumps" of the nerves that have been cut  out and this prevented the muscles from atrophy or weakness- this is called reinnervation. The procedure is done by making an incision in the neck and creating a small "window" into the laryngeal cartilage, this exposes the underlying nerves and muscles. This has to be done carefully so the back part of the cartilage that protects the nerve branches to the breathing muscles are not damaged. It take approximately three to four hours and under general anesthesia.

Outcome of SLAD-R surgery: Patients first report breathiness for approximately two to four months post surgery and over the year they report a stronger voice. Many people have reported more stability in their voice and others have reported a negative change in their voice. It depends on the person.

Speech Therapy:

Targets use of voice: gentle onset, vocal exercises (humming, vibrational focus at the lips), recommendation of an FM system, stretching to relax the oral vocal musculature, recommend using voice in the morning, volume control, environmental control (modify soft vs.. loud environment background noise exposure), confidence in the voice one has!

Additional Information:

Prevalence of SD is 1 case per 100,000 population. About 30% of patients with SD also have a co occurring vocal tremor.

References:

Chan SW., Baxter, M., Oates, J., Yorston, A,. (2009). Long term results of Type II Thyroplasty for adductor spasmodic dysphonia. The Larygoscope. V 114 (9) 1604-1608.

De Conde,A., Long,J., Armin, B., Berke, G. (2012). Functional reinnervation of vocal folds and selective laryngeal adductor denervation-reinnervation surgery for spasmodic dysphonia. Journal of Voice. V 25(5), 602-603.

Hajioff, D., RatenburyH., Carrie, S., Carding, P., Wilson, J. (2001). The effect of Isshiki type 1 thyroplasty on quality of life and vocal performance. Clinical Otolaryngology and Allied Sciences. Vol 25, Issue 5, 418-422.

http://www.pluralpublishing.com/media/media_vtcs_SamplePages.pdf

www.dystonia-foundation.org

www.voicedoctor.net

www.dysphonia.org

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BENIGN ESSENTIAL VOICE TREMOR:

Overview:

Benign Essential Voice Tremor causes shaking of the voice. It can affect the larynx, vocal folds or throat. This type of disorder will not harm your over all health since it is benign, but it will affect your ability to communicate confidently with others. Essential means that the tremor is not associated with any other state of a disease. It is a disorder of involuntary movement. The tremor is rhythmic and can be oscillatory. The cycle can vary in timing and occur as much as 12 times per second. The tremor may only be heard when the person speaks, unless it is present in other parts of the body such as the hands, head or other body parts.

This video shows someone with a benign essential voice tremor.

Cause:

There is no specific test to diagnose tremor, but it is caused by a central nervous system problem. or it could be hereditary.  Benign Essential Tremor sometimes occurs along with Spasmodic Dysphonia, so that there are vocal fold spasms as well as continuous shaking. The reason for this is unknown.

Symptoms:

Symptoms of benign essential voice tremor include the following:

• Poor voice quality, with "old-sounding" characteristics
• Vocal weakness and low volume
• Vocal fatigue increasing with voice use
• Embarrassment

The voice can be qualified as wobbling or a steady shaky quality, it can be gentle or harsh like a “hiccup” sound. It is rhythmic and steady, it may vary in intensity with pitch changes in volume and it can get worse in stressful situations, similar to most voice disorders.

Treatment:

There is not one specific treatment that will eliminate vocal tremor. The patient will often try to control the tremor when speaking which can do more harm to the vocal musculature and make the tremor worse.

Functional voice therapy:

This can help reduce the vocal compensation and improve the speaking voice.

Botox injections:

Injections can be helpful to reduce the strain and harsh voice quality, but the tremor will not be eliminated. Again, this depends on the severity of tremor. The patient is injected with botox into the thyroarytenoid muscle of the vocal fold which controls the strength and  “hiccup” quality of the tremor.  The person will have to have consistent Botox injections every 4-6 months. There are two injections, one for each side.

How does the Doctor know he is injecting into the correct muscle?

The Doctor knows he/she is injecting into the correct muscle with the help of Electromyographic (EEG) guidance. The needle is attached to a wire that sends a signal to the EEG machine that gives a signal about the activity of the muscle. The patient will be asked to say “eee or ahhh” this will cause the muscle to contract and this sends a signal through the wire to the EEG machine. A “crackling” noise is made which tells the doctor that the needle is in the correct muscle.  The person is “grounded” for the electrical signal by having electrodes placed on their clavicle area.

How long does it take?

It only takes a few minutes, but they ask the patient to wait 15 minutes to be sure they feel OK!

Does it hurt?

It is a small amount of pain, like a tetanus shot. No anesthesia is used because it makes it hurt more.

How long does it take to feel the effects of the injection?

The injection can take up to three days to feel its effects, but again this depends on the person. Some people report that they feel the difference the next day.

What are the side effects?

Side effects are minimal since the amount of Botox injected is generally a small amount. Patients will notice weak and breathy voice quality for about a week and over time it should get stronger.  The voice tremor will be  more gentle, but it will no disappear. The only noticeable side effect is often that the vocal folds may appear weak.

Oral medications:

Medications such as anti-seizure drugs, beta-blockers and psychotropic drugs can help with tremor, but it depends on each individual case as well as severity of the tremor and whether it exists in other parts of the body. People with vocal tremor must consult  their physician before taking any medications, because  these drugs can also have a side effect of tremor as well.

References:

Gironell, G., Kulisevsky, J.  (2009). Diagnosis and Management of essential tremor and dystonic temor. Ther Adv. Neurological Disorders. 2(4) 215-222.

Lester RA, Barkmeier-Kraemer, J, Story BH. (2013). Physiologic and acoustic patterns of essential voice tremor. Journal of Voice. doi:pii: S0892-1997(13)00003-9. 10.1016/j.jvoice.2013.01.002.

www.voiceproblem.org

www.voicedoctor.net

www.essentialtremor.org

www.diseases-explained.com/EssentialTremor/index.html

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PARALYSIS/PARESIS OF THE VOCAL FOLDS:

Overview:

Paralysis of the vocal folds means that one cannot move either one, or both of the vocal folds. Paresis means either one or both of the folds is weak. Usually there is a gap between the folds due to inadequate closure causing excess air to flow through them.


Figure 1A (left): The vocal fold on the right of this photo is paralyzed after a thyroid operation.
Figure 1B (right): An effort to make voice moves the other fold to the midline, but a substantial 
gap remains between the two. This makes for a soft, breathy voice.


www.voicemedicine.com

Cause:

Damage to the Vagus Nerve (Cranial Nerve 10) causes paralysis or paresis. The Vagus nerve has three branches: the Recurrent Laryngeal nerve, Pharyngeal branch and the Superior Laryngeal nerve.  These nerves travel from the brain to the pharynx, larynx and along the carotid artery. Vocal fold paralysis or paresis can result from surgeries in the brainstem, head, or neck. Thyroid, pulmonary, cardiac or spinal surgeries can affect the vocal folds. Specifically, the Recurrent Laryngeal Nerve is most often damaged, since it hooks under the aortic arch of the heart.

Symptoms:

The voice may be characterized differently depending on the severity of the paralysis/paresis of the vocal folds and nerves in the laryngeal/pharyngeal area.

Vocal Quality:

• weak
• breathy
• rough
• hoarse
• diplophonic (sounds like two pitches occurring at the same time) inability to sustain voiced sounds

People with paralysis or paresis often complain of the following symptoms:

• Lack of volume
• Breathy voice
• Inability to speak loudly
• Choking or coughing while eating
• Inability to sustain or support phonation for a long time (“ahhhh”)
• Food or liquid could be aspirated into the lungs causing pneumonia since the vocal folds don’t close properly

Diagnosis:

An endoscope is used to examine this vocal folds and throat area. This tiny scope with a camera and a light on the end of it is inserted through the nose or mouth. It is used to look inside at the structure and function of the area at rest and during phonation (sound production). It helps determine if paresis or paralysis exists.

 Treatment:

Voice Therapy:

 It is administered by a speech pathologist and they work on breath support, phonation and loudness.  The SLP focuses on proper placement of the vocal mechanism for optimal voicing. For example, turning the head either to the right or the left should bring the vocal folds together and the client as well as the SLP can determine which side the voice sounds better on. Manipulating or performing Circumlaryngeal massage helps to relax the musculature in the neck and upper chest area. This assists in improving voice quality and output.

Surgery:

The goal of having surgery is to move the paralyzed vocal fold closer to mid line so it can interact with the working vocal fold to help produce vibration.

Injections:

Injections can also be used to treat weakness or paralysis of the vocal folds, the immovable vocal fold is injected with a substance to plump it up and move it toward the center.

Permanent Injection materials include:

Teflon – this tends to be controversial because it is known to migrate to other parts of the body and it can produce a granuloma (benign tumor). Fat is also thought to be another permanent injection substance, though it does get reabsorbed by the body, so more research needs to be done.

Temporary Injection materials include:

Gelfoam, collagen, Calciumhydroxylapatite (CaHA), various gels and fat get reabsorbed by the body over time 2- 12 months. Collagen, hyaluronic acid & CaHA are other materials, they are more liquid –like and therefore more easily administered right through the skin. One has the downside of being a bovine (cow) product so has some risk of an allergic reaction.

Implants:

Another procedure for a weak or paralyzed vocal fold is called a thyroplasty, this is when a small hole or “window” is cut into the cartilage of the larynx and a solid piece of material is passed through it and placed in the damaged vocal fold to assist in bringing it more midline. The person is awake during this procedure so the voice can be tested and the implant adjusted as need. Different materials are used such as Silastic, hydroxadhesive and goretex.

Reinnervation:

Another option for unilateral vocal fold immobility due to nerve dysfunction is reinnervation. A nerve is “borrowed” from one of the neck muscles and attached to the recurrent laryngeal nerve. It often takes 6-12 months for the nerve to start functioning to provide voice improvement. Often a vocal fold injection is used to bulk it up temporarily and move it toward the middle, the vocal fold often will not move, but it will have excellent tone, bulk and muscle.

Recovery from Injections:

At the end of the injection ones voice may seem tight and stiff. It stays swollen for about a week and therefore the voice is worse, until the swelling subsides. Recovery from injection therapy depends on whether the substance was permanent or temporary.  Permanent materials should reach a stable state in 1 month, whereas temporary injections only last 3-6 months and the voice deteriorates over time.

Instructions during healing

Limit talking for a few days, but over time your symptoms should improve. If you develop swelling or redness of the skin at the injection site or if you feel like you are getting short of breath, you should call your physician immediately.

References:

Bastian RW; Delsupehe KG. Indirect larynx and pharynx surgery: a replacement for direct laryngoscopy. Laryngoscope 1996 Oct;106(10):1280-6

Boone, D., McFarlane, S., Von Berg S. (2005). The voice and voice therapy (7th ed.). New York: Allyn & Bacon

Colton, R, Casper J., Leonard, R. (2006). Understanding voice problems: A physiological perspective for diagnosis and treatment (3rd ed.). New York: Lippincott Williams and Wilkins

Roth, F.P and Worthington, C.K. (2001). Intervention for voice and alaryngeal speech. In Treatment resource manual for speech-language pathology, 2^nd ed. Albany, NY; Singular Thomson Learning.

 www.voicemedicine.com

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OTHER NEUROGENIC DISEASES:

Overview & Cause:

Other Neurologic Diseases may cause voice disorders or voice problems may develop as a symptom of the following disorders:

• Parkinson's Disease (PD): a degenerative disorder of the central nervous system, symptoms include motor impairments due to death of dopamine generating cells in the mid brain. Motor impairments include: shaking, rigidity, slowness of initiation to start moving or speaking. Can see tremor in the hands or entire body and gait when walking. The voice can become dysphonic or in severe cases aphonic. The voice may be hoarse and breathy, monopitch and monoloud.
• Myasthenia Gravis (MG): chronic autoimmune disease causing weakness and fatigue. Can affect voluntary muscles of the body such as: the eyes, the mouth, the throat and the limbs. Voice may be breathy, monotone. monoloudness, voice fatigues with use- recover with rest.
• Amyotrophic Lateral Sclerosis (ALS, a.k.a. Lou Gherig's disease): neurodegenerative disease that affects nerve cells in the brain and spinal cord, upper motor neuron (UMN) or lower motor neuron (LMN). Symptoms include: LMN symptoms: atrophy, fasciculations, weakness and UMN symptoms: tight, stiff muscles, spasticity/rigidity and exaggerated reflexes (Hypereflexia) dysphagia (swallowing difficulty),  and difficulty speaking. These patients can exhibit pseudobulbar affect or "emotional lability". Voice quality can present in a mixed fashion since this is characterized as a Mixed Dysarthria: the voice can be hypernasal, strain/strangled, slow effortful, monopitch, monoloudness, decreased range of motion.
• Stroke (Cerebrovascular Accident CVA): limited or no brain function due to loss of blood supply to the brain. This can be due to Ischemia - lack of blood flow caused by, a blockage (Thrombosis or Arterial Embolism or hemorrhage. Symptoms of strokes vary as does severity but they include inability to move one side of the body, inability to understand or formulate speech and inability to see one side of the visual field. Voice qualities and problems can vary dependent on the stoke, lesion location and severity.
• Traumatic Brain Injury (TBI): this is a closed or penetrating injury to the brain which can have varying symptoms and outcomes. It can be widespread  or focal damage. It is a major cause of disability and death. It is more common in males than females. Voice disorders concomitant with TBI vary depending on each patient. Often times a voice disorder that coexists with these disorders is termed as dysarthria.

Symptoms:

Parkinson's Disease:

The following symptoms may be present:

• Breathy voice
• Monopitch
• Monoloudness
• Short, fast rushes of speech
• Trouble with initiating speech

Myasthenia Gravis:

The following symptoms may be present:

• Monotone
• Monoloudness
• Fatigue with vocal use

Amyotrophic Lateral Sclerosis:

The following symptoms may be present:

• Strained and strangled sounding voice
• Hyponasal vocal quality
• Slow and effortful speech
• Monoloudness (reduced volume)
• Monopitch
• Breathy voice

Stroke/CVA:

The quality of voice and symptoms depend on the location and the severity of the stoke.

Traumatic Brain Injury:

The quality of voice and symptoms depend on the location and the severity of the injury.

Treatment:

In regards to these neurogenic disorders treatment varies depending on the person and how they are affected. The treatment given may help the primary disease or disorder and in addition a voice treatment will be given such as Botox injections or surgery.  See the Treatment sections on Spasmodic Dysphonia and Paralysis/Paresis for some applicable information.

Functional Voice Therapy will be helpful for all these types of Neurogenic Disorders- depending on the symptoms presented voice therapy would work on: pitch, rate, timing, prosody, volume, stress (word and sentence), word finding, vocabulary and several different communication modalities: sign language, communication boards, Alternative Augmentative Communication devices (AAC) would also be an option for a patient who loses their ability to speak or if it becomes effortful over time. You can find more information here.


Parkinson's Disease, patients with voice disorders with PD have benefitted from the Lee Silverman Voice Treatment (LSVT ). This treatment is specifically directed toward PD and focuses on intensive high effort speech exercises and increasing vocal loudness in a healthy way. For more information on LSVT, click here.

Here are two videos that may be helpful:

•       Video 1: Think Big

•       Video 2: Think Big, 2 Years Later

Myasthenia Gravis (MG) can be treated in different ways. As fatigue is an associated symptom that affect both gross motor movements and speech productions, oral medications that reduce fatigue are helpful. ALS, stroke & traumatic brain injury are all treated in various different ways, as the symptoms themselves are treated depending on their severity.

References:

Ramig, L., Verdolini, K. (1998). Treatment Efficacy Voice Disorders. Journal of Speech, Language and Hearing Research, (41), S101-S116.


Ramig, LO., Fox, C., Sapir, S. (2004). Parkinsons disease: speech and voice disorders and their treatment with the Lee Silverman Voice Treatment. Semin Speech Language. (2) 169-80.


Watts, C., Vanryckeghem, M. (2001).Laryngeal Dysfunction in ALS: a review and case report. BMC Ear, Nose and Throat Disorders. 1;1.


http://www.ninds.nih.gov/disorders/stroke/preventing_stroke.htm

http://www.alsa.org/about-als/


www.voiceproblem.org


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC60006/pdf/1472-6815-1-1.pdf