Spasmodic dysphonia

Spasmodic dysphonia (or laryngeal dystonia) is a voice disorder characterized by involuntary movements of one or more muscles of the larynx (vocal folds or voice box) during speech.^[1]

Types of spasmodic dysphonia

The three types of spasmodic dysphonia (SD) are adductor spasmodic dysphonia, abductor spasmodic dysphonia and mixed spasmodic dysphonia.

Adductor spasmodic dysphonia

In adductor spasmodic dysphonia, sudden involuntary muscle movements or spasms cause the vocal folds (or vocal cords) to slam together and stiffen.^[1] These spasms make it difficult for the vocal folds to vibrate and produce voice. Words are often cut off or difficult to start because of the muscle spasms. Therefore, speech may be choppy and sound similar to stuttering. The voice of an individual with adductor spasmodic dysphonia is commonly described as strained or strangled and full of effort. Surprisingly, the spasms are usually absent while laughing, speaking at a high pitch, speaking while breathing and singing, but singers can experience a loss of range or the inability to produce certain notes of a scale or with projection. Stress, however, often makes the muscle spasms more severe.^[2]

Abductor spasmodic dysphonia

In abductor spasmodic dysphonia, sudden involuntary muscle movements or spasms cause the vocal folds to open.^[1] The vocal folds can not vibrate when they are open. The open position of the vocal folds also allows air to escape from the lungs during speech. As a result, the voices of these individuals often sound weak, quiet and breathy or whispery. As with adductor spasmodic dysphonia, the spasms are often absent during activities such as laughing or singing but singers can experience a loss of range or the inability to produce certain notes of a scale or with projection.^[2]

Mixed spasmodic dysphonia

Mixed spasmodic dysphonia involves muscles that open the vocal folds as well as muscles that close the vocal folds and therefore has features of both adductor and abductor spasmodic dysphonia.^[1]

Origins

The exact cause of spasmodic dysphonia (SD) is unknown.^[1] According to the National Institute on Deafness and Other Communication Disorders "research has revealed increasing evidence that most cases of spasmodic dysphonia are in fact neurogenic or having to do with the nervous system (brain and nerves)."^[2]

SD is a neurological disorder rather than a disorder of the larynx, and as in other forms of dystonia, interventions at the end organ (i.e., larynx) have not offered a definitive cure, only symptomatic relief. The pathophysiology underlying dystonia is becoming better understood as a result of discoveries in genetically based forms of the disorder, and this approach is the most promising avenue to a long-term solution.^[3]

The National Institute of Neurological Disorders and Stroke (NINDS) and the American Academy of Neurology (AAN) classify SD as a neurological disorder.^[4] However, because the voice can sound normal or near normal at times, some practitioners believe it to be psychogenic, that is, originating in the affected person's mind rather than from a physical cause. No medical organizations or groups take this position. A comparison of SD patients compared with vocal fold paralysis (VFP) patients found that 41.7% of the SD patients met the DSM-IV criteria for psychiatric comorbidity compared with 19.5% of the VFP group.^[5] However, another study found the opposite, with SD patients having significantly less psychiatric comorbidity compared to VFP patients: "The prevalence of major psychiatric cases varied considerably among the groups, from a low of seven percent (1/14) for spasmodic dysphonia, to 29.4 percent (5/17) for functional dysphonia, to a high of 63.6 percent (7/11) for vocal cord paralysis."^[6] A review in the journal Swiss Medicine Weekly states that "Psychogenic causes, a 'psychological disequilibrium', and an increased tension of the laryngeal muscles are presumed to be one end of the spectrum of possible factors leading to the development of the disorder".^[7] Alternatively, many investigators into the condition feel that the psychiatric comorbidity associated with voice disorders is a result of the social isolation and anxiety that patients with these conditions feel as a consequence of their difficulty with speech, as opposed to the cause of their dysfluency.^[8] The opinion that SD is psychogenic is not upheld by experts in the scientific community.^[6][9]

Evidence for a neurological basis

SD is formally classified as a movement disorder, one of the focal dystonias, and is also known as laryngeal dystonia. ^[10] Supporting evidence that SD is a neurological disorder includes:

• SD may co-occur with other neurological movement disorders such as blepharospasm (excessive eye blinking and involuntary forced eye closure), tardive dyskinesia (involuntary and repetitious movement of muscles of the face, tongue, body, arms and legs), oromandibular dystonia (involuntary movements of the jaw muscles, lips and tongue), torticollis (involuntary movements of the neck muscles), or tremor (rhythmic, quivering muscle movements).^[1]
• Spasmodic dysphonia runs in some families and is thought to be inherited. Research has identified a possible gene on chromosome 9 that may contribute to the spasmodic dysphonia that is common to certain families.^[1]
• Histological examination of the nerve to the vocal cords in patients with SD demonstrates that the percentage of abnormally thin nerve fibers was higher than in normal controls^[11]
• Functional MRI signal is reduced in sensorimotor cortices associated with movement of the affected body part in laryngeal dystonia, supporting a dystonic basis for this voice disorder.^[12]

Diagnosis

Unfortunately, diagnosis of spasmodic dysphonia is often delayed due to lack of recognition of its symptoms by screening physicians. Most patients who are correctly diagnosed are evaluated by a team that usually includes an otolaryngologist, a speech-language pathologist and a neurologist. The otolaryngologist examines the vocal folds to look for other possible causes for the voice disorder. Fiberoptic laryngoscopy, a method whereby a small lighted flexible tube is passed through the nose and into the throat, is a helpful tool that allows the otolaryngologist to evaluate vocal cord movement during speech. Additional diagnostic testing may include stroboscopy, which allows the physician to view the vibrations of the vocal cords in slow motion. The speech-language pathologist evaluates the patient's voice and voice quality. The neurologist evaluates the patient for signs of other movement disorders.^[2]

Treatment

There is no known cure for spasmodic dysphonia.^[1]

The most effective treatment for reducing the symptoms of spasmodic dysphonia is injections of very small amounts of botulinum toxin (sold commercially under the brand names Botox, Dysport, and Myobloc) directly into the affected muscles of the larynx. The toxin weakens muscles by blocking the nerve impulse to the muscle. The botulinum toxin injections generally improve the voice for a period of three to four months after which the voice symptoms gradually return. This treatment requires continual injections to maintain a good speaking voice.^[2]

SD is sometimes confused with other hyperfunctional voice disorders that may respond to voice therapy, however voice therapy is not effective in treating SD.^[9]

An operation that cuts one of the nerves of the vocal folds (the recurrent laryngeal nerve) has improved the voice of many for several months to several years but the improvement is often temporary.^[2]

Notable people with spasmodic dysphonia

• Scott Adams, the creator of the comic strip Dilbert.^[13]
• Frank Allison, musician, leader of Frank Allison and the Odd Sox^[14]
• Johnny Bush, country & western musician and songwriter^[15]
• Keath Fraser, Canadian author who has documented the challenges and treatment of his condition in the book The Voice Gallery: Travels With a Glass Throat (2002).^[16]
• Sjors Fröhlich, former Dutch radio presenter, who had to give up his job as a presenter due to this disease.
• Robert F. Kennedy, Jr.,^[17] son of United States Senator and presidential candidate Robert F. Kennedy, political and environmental activist
• Fred Lavery, a music producer, writer, musician, and recording studio co-owner from Cape Breton, Nova Scotia. Was lead singer of recording group Road and was later a solo artist, but developed the condition in the 1980s, and was forced to quit singing. Receives injections to keep voice fairly normal.
• Mary Lou Lord^[18]
• Andy MacWilliams, former radio broadcaster for the Cincinnati Stingers, Chicago Blackhawks and Cincinnati Cyclones.
• Darryl McDaniels of the rap group Run DMC^[19]
• Diane Rehm, host of the The Diane Rehm Show on National Public Radio (NPR).^[20]
• Mark Stuart, American rock musician (Audio Adrenaline)^[21]
• Linda Thompson, British folk-rock musician.^[22]

See also

• Dysphonia
• Spasm

References

1. ^ ^{a b c d e f g h} "Definition of Spasmodic dysphonia". MedTerms medical dictionary. MedicineNet.com. http://www.medterms.com/script/main/art.asp?articlekey=13856. Retrieved 2007-05-11. 
2. ^ ^{a b c d e f} "Spasmodic Dysphonia". Publication No. 97-4214. National Institute on Deafness and Other Communication Disorders. http://www.nidcd.nih.gov/health/voice/spasdysp.htm. Retrieved 2007-05-11. Includes text taken directly from the website. As a work of the National Institutes of Health, part of the United States Department of Health and Human Services, this text constitutes a work of the U.S. federal government, therefore it is in the public domain.
3. ^ 1: Curr Opin Otolaryngol Head Neck Surg. 2004 Dec;12(6):543–8. PMID: 15548915
4. ^ Dystonias Fact Sheet: National Institute of Neurological Disorders and Stroke (NINDS)
5. ^ Gündel H, Busch R, Ceballos-Baumann A, Seifert E (2007). [PubMed Psychiatric comorbidity in patients with spasmodic dysphonia - a controlled study.]. J Neurol Neurosurg Psychiatry. PubMed. 
6. ^ ^{a b} Newswise Medical News | Patients with Selected Voice Disorders Are Subject to Psychiatric Problems
7. ^ Seifert, E.; Kollbrunner, J. (2005-07-09). [PubMed Stress and distress in non-organic voice disorder.]. Swiss Med Weekly. PubMed. Retrieved 2007-05-11. 
8. ^ Ann Otol Rhinol Laryngol. 2001 Oct;110(10):941–5.
9. ^ ^{a b} Spasmodic Dysphonia is a Neurological Disorder Current Evidence and References, by Christy L. Ludlow, Ph.D.
10. ^ Otolaryngol Head Neck Surg. 2005 Nov;133(5):654–65. PMID: 16274788
11. ^ Otolaryngol Head Neck Surg. 1999 Jan;120(1):129–33
12. ^ Neurology. 2005 Nov 22;65(10):1562–9 PMID: 16301482
13. ^ "'Dilbert' creator recovers from rare disorder". Associated Press. 2006-10-27. http://www.msnbc.msn.com/id/15446515/GT1/8618/. Retrieved 2007-07-30. 
14. ^ Frank Allison
15. ^ Beal Jr., Jim (1998-04-29). "Golden country Johnny Bush hasn't run dry after almost 50 years of heartaches and honky-tonks". San Antonio Express-News. http://nl.newsbank.com/nl-search/we/Archives?p_product=SAEC&p_theme=saec&p_action=search&p_maxdocs=200&p_topdoc=1&p_text_direct-0=0EAFE8396F19EF1A&p_field_direct-0=document_id&p_perpage=10&p_sort=YMD_date:D&s_trackval=GooglePM. 
16. ^ The Voice Gallery - Thomas Allen Publishers - Thomas Allen & Son Limited
17. ^ Liebovich, Mark (2006-06-25). "Another Kennedy Living Dangerously". The New York Times. http://www.nytimes.com/2006/06/25/fashion/25bobby.html?ex=1308888000&en=4366351657b8045b&ei=5090&partner=rssuserland&emc=rss. Retrieved 2007-07-30. 
18. ^ Sullivan, Jim (2004-02-19). "Hello, Mary Lou – Goodbye, Heartache". Boston Globe. http://nl.newsbank.com/nl-search/we/Archives?p_product=BG&p_theme=bg&p_action=search&p_maxdocs=200&p_topdoc=1&p_text_direct-0=100D6882D4430C7F&p_field_direct-0=document_id&p_perpage=10&p_sort=YMD_date:D&s_trackval=GooglePM. 
19. ^ Heffernan, Virginia (2006-02-25). "After the Hit Records, A Search for His Roots". The New York Times. 
20. ^ "About Diane Rehm". WAMU 88.5 American University Radio. http://wamu.org/programs/dr/diane_rehm/. Retrieved 2007-07-30. 
21. ^ "Mark Stuart informs his Facebook followers that he has Spasmodic Dyphonia". http://www.facebook.com/permalink.php?story_fbid=150322168318129&id=116116964187. 
22. ^ "Thompson's Trials: UK Folk Rock Great Makes Triumphant Return to the Stage". Tim Perlich, NOW Toronto. 2002-10-17. http://www.nowtoronto.com/issues/2002-10-17/music_feature3.php. Retrieved 2007-08-23. 

External links

• Spasmodic Disphonia Online Community (VoiceMatters.net)
• American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS)
• American Speech-Language-Hearing Association (ASHA)
• National Spasmodic Dysphonia Association, Inc. (NSDA)
• American Academy of Neurology
• Worldwide Education and Awareness for Movement Disorders (WE MOVE)
• Spasmodic Dysphonia section of VoiceInfo.org
• Dystonia Society Network Group – for sufferers and carers to share experiences
• Society for the Management of Spasmodic Dysphonia