Labyrinthine fistula

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Labyrinthine fistula
Cochlea-crosssection.svg
Cross-section of cochlea. Perilymph is located in the scala tympani and scala vestibuli – the aqua regions at the top and bottom of the diagram.
Specialty ENT, Neurology

A labyrinthine fistula is an abnormal opening in the inner ear. This can result in leakage of the perilymph into the middle ear. [1] This includes specifically a perilymph fistula (PLF), an abnormal connection between the fluid of the inner ear and the air-filled middle ear. This is caused by a rupture of the round window or oval window ligaments separating the inner and middle ear. [1]

Contents

Another type of labyrinthine fistula is the superior semicircular canal dehiscence, which allows the inner ear to be influenced by the intracranial pressure directly.

Signs and symptoms

PLF usually induces one or all the following pathological states: aural fullness, fluctuating or non-fluctuating hearing loss, tinnitus and dizziness, which may sometimes include vertigo and balance disorders. [2]

Causes

Labyrinthine fistula can be both congenital or develop over time with the thinning of the otic capsule by the persistent pulsations of the intracranial pressures against the bones of the skull. Finally, medical conditions (e.g. cholesteatoma) can result in a labyrinthine fistula. [3] Traumatic events, with excessive pressure changes to the inner ear such as in scuba diving, [4] head trauma, or an extremely loud noise can lead to rupture and leakage. [1] The most common causes of PLF are: head or ear traumas, rapid increases of intracranial pressure, congenital abnormalities (in children), complication of stapedectomy, barotraumas (e.g. slap/suction, scuba diving, skydiving, strong and repetitive nose-blowing or sneezing, heavy lifting). [2]

Diagnosis

When diagnosing, PLF should be differentiated from Ménière's disease. Tympanostomy has been reported to be a way to diagnose [5] and cure PLF.

Treatment

Patients are advised to treat with bed rest and avoiding activities that increase intracranial pressure (i.e. weightlifting, Valsalva maneuver, scuba diving, flying in airplanes) with the hopes of the membrane healing on its own. Appropriate Physical therapy / vestibular rehabilitation techniques can be helpful in managing symptoms of movement sensitivity. [1]

Related Research Articles

This is a glossary of medical terms related to communication disorders which are psychological or medical conditions that could have the potential to affect the ways in which individuals can hear, listen, understand, speak and respond to others.

<span class="mw-page-title-main">Inner ear</span> Innermost part of the vertebrate ear

The inner ear is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the temporal bone of the skull with a system of passages comprising two main functional parts:

<span class="mw-page-title-main">Cholesteatoma</span> Medical condition

Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. Cholesteatomas are not cancerous as the name may suggest, but can cause significant problems because of their erosive and expansile properties. This can result in the destruction of the bones of the middle ear (ossicles), as well as growth through the base of the skull into the brain. They often become infected and can result in chronically draining ears. Treatment almost always consists of surgical removal.

<span class="mw-page-title-main">Eardrum</span> Membrane separating the external ear from the middle ear

In the anatomy of humans and various other tetrapods, the eardrum, also called the tympanic membrane or myringa, is a thin, cone-shaped membrane that separates the external ear from the middle ear. Its function is to transmit sound from the air to the ossicles inside the middle ear, and then to the oval window in the fluid-filled cochlea. Hence, it ultimately converts and amplifies vibration in the air to vibration in cochlear fluid. The malleus bone bridges the gap between the eardrum and the other ossicles.

<span class="mw-page-title-main">Fistula</span> Abnormal connection between two epithelialized surfaces, often organs

A fistula in anatomy is an abnormal connection between two hollow spaces, such as blood vessels, intestines, or other hollow organs. Types of fistula can be described by their location. Anal fistulas connect between the anal canal and the perianal skin. Anovaginal or rectovaginal fistulas occur when a hole develops between the anus or rectum and the vagina. Colovaginal fistulas occur between the colon and the vagina. Urinary tract fistulas are abnormal openings within the urinary tract or an abnormal connection between the urinary tract and another organ such as between the bladder and the uterus in a vesicouterine fistula, between the bladder and the vagina in a vesicovaginal fistula, and between the urethra and the vagina in urethrovaginal fistula. When occurring between two parts of the intestine, it is known as an enteroenteral fistula, between the small intestine and the skin as an enterocutaneous fistula, and between the colon and the skin as a colocutaneous fistula.

A stapedectomy is a surgical procedure of the middle ear performed in order to improve hearing.

<span class="mw-page-title-main">Barotrauma</span> Injury caused by pressure

Barotrauma is physical damage to body tissues caused by a difference in pressure between a gas space inside, or contact with, the body and the surrounding gas or liquid. The initial damage is usually due to over-stretching the tissues in tension or shear, either directly by an expansion of the gas in the closed space or by pressure difference hydrostatically transmitted through the tissue. Tissue rupture may be complicated by the introduction of gas into the local tissue or circulation through the initial trauma site, which can cause blockage of circulation at distant sites or interfere with the normal function of an organ by its presence.

<span class="mw-page-title-main">Subdural hematoma</span> Hematoma usually associated with traumatic brain injury

A subdural hematoma (SDH) is a type of bleeding in which a collection of blood—usually but not always associated with a traumatic brain injury—gathers between the inner layer of the dura mater and the arachnoid mater of the meninges surrounding the brain. It usually results from tears in bridging veins that cross the subdural space.

<span class="mw-page-title-main">Ear</span> Organ of hearing and balance

An ear is the organ that enables hearing and, in mammals, body balance using the vestibular system. In mammals, the ear is usually described as having three parts—the outer ear, the middle ear and the inner ear. The outer ear consists of the pinna and the ear canal. Since the outer ear is the only visible portion of the ear in most animals, the word "ear" often refers to the external part alone. The middle ear includes the tympanic cavity and the three ossicles. The inner ear sits in the bony labyrinth, and contains structures which are key to several senses: the semicircular canals, which enable balance and eye tracking when moving; the utricle and saccule, which enable balance when stationary; and the cochlea, which enables hearing. The ears of vertebrates are placed somewhat symmetrically on either side of the head, an arrangement that aids sound localization.

<span class="mw-page-title-main">Labyrinthitis</span> Medical condition

Labyrinthitis is inflammation of the labyrinth – a maze of fluid-filled channels in the inner ear. Vestibular neuritis is inflammation of the vestibular nerve – the nerve in the inner ear that sends messages related to motion and position to the brain. Both conditions involve inflammation of the inner ear. Labyrinths that house the vestibular system sense changes in the head's position or the head's motion. Inflammation of these inner ear parts results in a sensation of the world spinning and also possible hearing loss or ringing in the ears. It can occur as a single attack, a series of attacks, or a persistent condition that diminishes over three to six weeks. It may be associated with nausea, vomiting, and eye nystagmus.

<span class="mw-page-title-main">Conductive hearing loss</span> Medical condition

Conductive hearing loss (CHL) occurs when there is a problem transferring sound waves anywhere along the pathway through the outer ear, tympanic membrane (eardrum), or middle ear (ossicles). If a conductive hearing loss occurs in conjunction with a sensorineural hearing loss, it is referred to as a mixed hearing loss. Depending upon the severity and nature of the conductive loss, this type of hearing impairment can often be treated with surgical intervention or pharmaceuticals to partially or, in some cases, fully restore hearing acuity to within normal range. However, cases of permanent or chronic conductive hearing loss may require other treatment modalities such as hearing aid devices to improve detection of sound and speech perception.

<span class="mw-page-title-main">Sensorineural hearing loss</span> Hearing loss caused by an inner ear or vestibulocochlear nerve defect

Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ or the vestibulocochlear nerve. SNHL accounts for about 90% of reported hearing loss. SNHL is usually permanent and can be mild, moderate, severe, profound, or total. Various other descriptors can be used depending on the shape of the audiogram, such as high frequency, low frequency, U-shaped, notched, peaked, or flat.

<span class="mw-page-title-main">Vertigo</span> Type of dizziness with a sensation of rotation when there is none

Vertigo is a condition in which a person has the sensation of movement or of surrounding objects moving when they are not. Often it feels like a spinning or swaying movement. This may be associated with nausea, vomiting, sweating, or difficulties walking. It is typically worse when the head is moved. Vertigo is the most common type of dizziness.

Beta-2 transferrin is a carbohydrate-free (desialated) isoform of transferrin, which is almost exclusively found in the cerebrospinal fluid. It is not found in blood, mucus or tears, thus making it a specific marker of cerebrospinal fluid, applied as an assay in cases where cerebrospinal fluid leakage is suspected.

Tullio phenomenon, sound-induced vertigo, dizziness, nausea or eye movement (nystagmus) was first described in 1929 by the Italian biologist Prof. Pietro Tullio. (1881–1941) During his experiments on pigeons, Tullio discovered that by drilling tiny holes in the semicircular canals of his subjects, he could subsequently cause them balance problems when exposed to sound.

The semicircular canal dehiscence (SCD) is a category of rare neurotological diseases/disorders affecting the inner ears, which gathers the superior SCD, lateral SCD and posterior SCD. These SCDs induce SCD syndromes (SCDSs), which define specific sets of hearing and balance symptoms. This entry mainly deals with the Superior SCDS.

<span class="mw-page-title-main">Cerebrospinal fluid leak</span> Medical condition

A cerebrospinal fluid leak is a medical condition where the cerebrospinal fluid (CSF) surrounding the brain or spinal cord leaks out of one or more holes or tears in the dura mater. A cerebrospinal fluid leak can be either cranial or spinal, and these are two different disorders. A spinal CSF leak can be caused by one or more meningeal diverticula or CSF-venous fistulas not associated with an epidural leak.

Facial nerve decompression is a type of nerve decompression surgery where abnormal compression on the facial nerve is relieved.

Inner ear decompression sickness, (IEDCS) or audiovestibular decompression sickness is a medical condition of the inner ear caused by the formation of gas bubbles in the tissues or blood vessels of the inner ear. Generally referred to as a form of decompression sickness, it can also occur at constant pressure due to inert gas counterdiffusion effects.

Middle ear barotrauma (MEBT), also known to underwater divers as ear squeeze and reverse ear squeeze, is an injury caused by a difference in pressure between the external ear canal and the middle ear. It is common in underwater divers and usually occurs when the diver does not equalise sufficiently during descent or, less commonly, on ascent. Failure to equalise may be due to inexperience or eustachian tube dysfunction, which can have many possible causes. Unequalised ambient pressure increase during descent causes a pressure imbalance between the middle ear air space and the external auiditory canal over the eardrum, referred to by divers as ear squeeze, causing inward stretching, serous effusion and haemorrhage, and eventual rupture. During ascent internal over-pressure is normally passively released through the eustachian tube, but if this does not happen the volume expansion of middle ear gas will cause outward bulging, stretching and eventual rupture of the eardrum known to divers as reverse ear squeeze. This damage causes local pain and hearing loss. Tympanic rupture during a dive can allow water into the middle ear, which can cause severe vertigo from caloric stimulation. This may cause nausea and vomiting underwater, which has a high risk of aspiration of vomit or water, with possible fatal consequences.

References

  1. 1 2 3 4 O'Sullivan, Susan B. (2007). Physical Rehabilitation (Fifth ed.). Philadelphia: Davis Company. p. 1024.
  2. 1 2 Sarna B, Abouzari M, Merna C, Jamshidi S, Saber T, Djalilian H (2020). "Perilymphatic fistula: a review of classification, etiology, diagnosis and treatment". Frontiers in Neurology. 11. doi: 10.3389/fneur.2020.01046 . PMID   33041986.
  3. Chen Z, Wu Y, Shi H, et al. (June 2009). "Surgical treatment of labyrinthine fistula caused by cholesteatoma with semicircular canal occlusion". Acta Otolaryngol. 130 (1): 75–8. doi:10.3109/00016480902875083. PMID   20082558.
  4. Donoghue, P; Knight, J. "The causation of perilymph fistulae in divers". Journal of the South Pacific Underwater Medicine Society. 10 (2): 13–14. Archived from the original on September 22, 2013. Retrieved 2013-09-22.{{cite journal}}: CS1 maint: unfit URL (link)
  5. Goto F, Ogawa K, Kunihiro T, Kurashima K, Kobayashi H, Kanzaki J (January 2001). "Perilymph fistula--45 case analysis". Auris Nasus Larynx. 28 (1): 29–33. doi:10.1016/S0385-8146(00)00089-4. PMID   11137360.