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.
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.
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.
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).Adductor SD:
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:
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.
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.
http://www.pluralpublishing.com/media/media_vtcs_SamplePages.pdf
www.dystonia-foundation.org
www.voicedoctor.net
www.dysphonia.org
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.
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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