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Nystagmus, Benign Paroxysmal Positional


National Organization for Rare Disorders, Inc.

Synonyms

  • BPPN
  • Cupulolithiasis
  • Labyrinthine Positional Nystagmus
  • Paroxysmal Positional Nystagmus

Disorder Subdivisions

  • None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

  • Meniere Disease
  • Vestibular Neuronitis of Dix and Hallpike

General Discussion

Benign Paroxysmal Positional Nystagmus is a disorder of the vestibular system in the middle ear that causes dizziness due to altered function of the semicircular canals, usually involving the posterior canal but sometimes involving the horizontal canal. The dizziness, which is accompanied by abnormal eye movements or nystagmus, occurs suddenly and without warning; thus, it is "paroxysmal". It is also "positional" because the symptoms increase with certain movements of the head or body. It is the position of the head, rather than the movement, that causes the symptoms.

Symptoms

Benign Paroxysmal Positional Nystagmus (BPPN) is characterized by episodes of sudden, extreme dizziness triggered by certain positions of the head, and often accompanied by nausea, vomiting and impaired muscle coordination (ataxia). Involuntary rhythmic movements of the eyes (nystagmus) in horizontal, vertical or circular directions usually also occur. An attack may last for 30 seconds or less, but the symptoms may linger over a few weeks or months. Symptoms may disappear as spontaneously as they appeared. Frequently, there are recurring episodes.

Causes

The fundamental cause of BPPN is not known. The problem resides in the fluid-filled chamber (vestibular chamber) of the inner ear that controls balance. One of several different mechanisms affecting the semicircular canals of the inner ear may be present. These may include spontaneous degeneration of the membranes in the labyrinth of the ear, head injuries, serious middle ear infection, ear surgery, and closing off of the anterior vestibular artery to the inner ear.

Some clinicians attribute attacks of BPPN to stone-like calcium deposits found within the posterior semicircular canals of the inner ear.

Affected Populations

BPPN primarily affects females during middle or late adulthood.

Related Disorders

Meniere Disease is a disorder characterized by recurrent prostrating attacks of dizziness (vertigo), possible hearing loss and ringing sounds (tinnitus). (For more information on this disorder, choose "meniere" as your search term in the Rare Disease Database.)

Vestibular Neuronitis of Dix and Hallpike is a disorder of unknown cause, with abrupt onset during young adulthood and continuing through the fifth decade of life. It is characterized by dizziness, nausea and vomiting. Head movements may make the symptoms more severe. Hearing is usually not impaired. This disorder is often associated with upper respiratory tract infections and fever.

Standard Therapies

Patients with BPPN are often advised to avoid head movements and positions that could bring on the attacks. Medications may decrease dizziness and control nausea or vomiting. If the dizziness is caused by bacterial infection in the ear, antibiotics may help.

Some clinicians recommend a five-step maneuver known as the canalith repositioning procedure to relieve positional vertigo. This treatment involves, in five distinct steps, moving from a sitting to a reclining position on a flat surface and rolling over while the head is extended downward at a 45 degree angle.

Investigational Therapies

A surgical treatment used in some cases of acoustic nerve compression is known as the Jannetta procedure, which may be of some help in some cases of BPPN. Dr. Margareta Moller, who was one of the foremost proponents of this neurosurgical procedure while at the University of Pittsburgh, no longer practices surgery. However, she has indicated that she would be pleased to correspond with persons interested in the Janetta procedure to make referrals to neurosurgeons experienced in this procedure.

Her address in Dallas, where she now lives, is:

Margareta Moller, MD, DMS
6934 Desco Place
Dallas, TX 75225

References

TEXTBOOKS
Thoene JG., ed. Physicians' Guide to Rare Diseases. Montvale, NJ: Dowden Publishing Company Inc; 1995:281

Larson DE. ed. Mayo Clinic Family Health Book. New York, NY: William Morrow and Company, Inc; 1996:584-85.

Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:300.

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:679.

REVIEW ARTICLES
Dieterich M, et al., Vestibulo-ocular reflex. Curr Opin Neurol. 1995;8:83-88.

Brandt T, et al., Current view of the mechanism of benign paroxysmal positional vertigo: cupulolithiasis or canalolithiasis? J Vestib Res. 1993;3:373-82.

JOURNAL ARTICLES
Baloh RW, et al., Horizontal semicircular canal variant of benign positional vertigo. Neurology. 1993;43:2542-49.

Ushio K, et al., Three-component analysis of benign paroxysmal positional nystagmus. Acta Otolaryngol Suppl. 1995;519:107-09.

Yagi T, et al., Nystagmus in benign paroxysmal positional vertigo: a three component analysis. Acta Otolaryngol Suppl. 1995;520 Pt 2:238-40.

Baloh RW, et al., Persistent direction-changing positional nystagmus: another variant of benign positional nystagmus? Neurology. 1995;45:1297-301.

Nuti D, et al., Benign paroxysmal positional vertigo of the horizontal canal: a form of canalolithiasis with variable clinical features. J Vestib Res. 1996;6:173-84.

Steddin S, et al., Horizontal canal benign paroxysmal positional vertigo (h-BPPV): transition of canalolithiasis to cupulolithiasis. Ann Neurol. 1996;40:918-22.

Vannucchi P, et al., Treatment of horizontal semicircular canal benign paroxysmal positional vertigo. J Vestib Res. 1997;7:1-6.

Lida M, et al., Evaluation of the vertical semicircular canal function by the pendular rotation test: a study on patients with benign paroxysmal positional vertigo. ORL J Otorhinolaryngol Relat Spec. 1997;59:269-71.

Nuti D, et al., The management of horizontal-canal paroxysmal positional vertigo. Acta Otolaryngol. 1998;118:455-60.

Resources

Vestibular Disorders Association (VEDA)
P.O. Box 13305
Portland, OR 97213
USA
Tel: 5032297705
Fax: 5032298064
Tel: 8008378428
Email: veda@vestibular.org
Internet: http://www.vestibular.org

EAR (Education and Auditory Research) Foundation
P.O. Box 330867
Nashville, TN 37203
Tel: (615)627-2724
Tel: (800)545-4327
TDD: (615)284-7849
Email: suzanne@earfoundation.org
Internet: http://www.earfoundation.org

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

American Nystagmus Network, Inc.
303-D Beltine Place SW #321
Decatur, AL 35603
USA
Email: webmaster@nystagmus.org
Internet: http://www.nystagmus.org

American Academy of Audiology
11730 Plaza America
#300
Reston, VA 20190
Tel: (703)790-8466
Fax: (703)790-8631
Tel: (800)222-2336
Email: info@audiology.org
Internet: http://www.audiology.org

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  5/30/2000
Copyright  1987, 1989, 1998, 2000 National Organization for Rare Disorders, Inc.



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