The clinical presentation includes the onset of positional vertigo and sensorineural hearing loss of various degree, whereas nystagmus and facial palsy are reported as well [1] [4].

• Surgical Procedure

  METHODS: The clinical charts of 16 patients who underwent surgical procedures for cholesteatoma complicated by labyrinthine fistula between 2003 and 2008 were reviewed retrospectively. In this period, 108 ears were operated for cholesteatoma. [ncbi.nlm.nih.gov]

  Further chapters provide step by step surgical procedures for the ear canal, otitis media, tinnitus, vertigo, and Meniere’s disease. [books.google.de]

  No studies have been reported to account for the substantial placebo effect of a surgical procedure for vertiginous symptoms in patients with suspected PLF, and no appropriate long-term follow up of patients after surgery has been reported. [emedicine.com]

• Falling

  You may sometimes feel as though you cannot keep your balance, or you may feel like you are going to fall to the ground. A common type of dizziness is called vertigo. Vertigo is the sensation that you or your surrounding environment is moving. [edisonstanfordhearing.com]

  Miner's nystagmus also fall into this category. Congenital nystagmus: is often familial, usually horizontal and pendular. The null point in this type of nystagmus is close to the position of forward gaze. [drtbalu.co.in]

  The more significant repercussions of inner ear injuries, is that people can not normally place themselves in space, leaving them susceptible to falls, bumping into objects, crashing into objects while driving in a car, and drowning if disoriented while [deshawlaw.com]

  Most important, do not place yourself in a situation where you might lose your balance and be at risk for a fall and serious injury; stay off of chairs, stools, ladders, roofs, etc. [dizziness-and-balance.com]

• Chronic Infection

  Treatment requires surgical correction of the damage caused by chronic infection of the middle ear and cholesteatoma, but factors such as the size and location of the fistula and the severity of bone damage determine the optimal surgical strategy. [symptoma.com]

• Weight Loss

  loss, muscle spasticity ( latest reports from 15 Labyrinthine fistula patients ). [ehealthme.com]

• Fever

  Symptoms and signs If you experience any chest pains, confusion, a stiff neck, a fever over 101 degrees or dizziness after you hit your head, seek medical attention immediately. [edisonstanfordhearing.com]

• Nausea

  Clinical presentation Symptoms of perilymphatic fistula may include: sensorineural hearing loss vertigo nausea tinnitus spatial disorientation dizziness ear fullness Radiographic features CT may demonstrate fluid-filling in the round window (especially [radiopaedia.org]

  […] most sensorineural Tinnitus Aural Fullness Vertigo Disequilibrium Series of Events Perilymphatic or Labyrinthine Fistula Membrane Rupture Rapid Severe Hearing Loss Loud roaring tinnitus Severe rotational vertigo - visceral symptoms: sweating, pallor, nausea [quizlet.com]

  Vertigo is often incapacitating and accompanied by visceral symptoms (eg, sweating, pallor, nausea, vomiting). Even cursory examination demonstrates marked instability and nystagmus. Audiometric evaluation reveals sensorineural hearing loss. [emedicine.medscape.com]

  The symptoms of PLF include hearing loss, tinnitus, ear fullness, dizziness, vertigo, imbalance, motion intolerance, nausea and vomiting. [tinnitusformula.com]

  The increased pressure exposes cochlear hair cells responsible for sensing movement and balance to progressive damage and paralysis, resulting in attacks of dizziness, often with nausea and vomiting. [aetna.com]

• Vomiting

  […] sensorineural Tinnitus Aural Fullness Vertigo Disequilibrium Series of Events Perilymphatic or Labyrinthine Fistula Membrane Rupture Rapid Severe Hearing Loss Loud roaring tinnitus Severe rotational vertigo - visceral symptoms: sweating, pallor, nausea, vomiting [quizlet.com]

  Vertigo is often incapacitating and accompanied by visceral symptoms (eg, sweating, pallor, nausea, vomiting). Even cursory examination demonstrates marked instability and nystagmus. Audiometric evaluation reveals sensorineural hearing loss. [emedicine.medscape.com]

  The symptoms of PLF include hearing loss, tinnitus, ear fullness, dizziness, vertigo, imbalance, motion intolerance, nausea and vomiting. [tinnitusformula.com]

  The increased pressure exposes cochlear hair cells responsible for sensing movement and balance to progressive damage and paralysis, resulting in attacks of dizziness, often with nausea and vomiting. [aetna.com]

  The symptoms of perilymph fistula may include dizziness, vertigo, imbalance, nausea, and vomiting. Usually however, patients report an unsteadiness which increases with activity and which is relieved by rest. [dizziness-and-balance.com]

• Muscle Spasticity

  spasticity ( latest reports from 15 Labyrinthine fistula patients ). [ehealthme.com]

• Ankylosis

  Abstract The author reports a case of chronic otitis with cholesteatoma complicated by a fistula of the lateral semicircular canal and a stapedo-ovalar ankylosis, probably of otosclerotic origin, in which the decision to take advantage of the fistula [ncbi.nlm.nih.gov]

• Tinnitus

  Suddenly he noticed a low pitched tinnitus in his left ear. He was not trying to Valsalva at the time. When he left the water the tinnitus disappeared. [archive.rubicon-foundation.org]

  Primary symptoms were hypoacusis, otorrhea, vertigo, tinnitus, and otalgia. All patients underwent preoperative CT scans and preoperative audiometry. [ncbi.nlm.nih.gov]

  Aural Fullness Vertigo Disequilibrium Series of Events Perilymphatic or Labyrinthine Fistula Membrane Rupture Rapid Severe Hearing Loss Loud roaring tinnitus Severe rotational vertigo - visceral symptoms: sweating, pallor, nausea, vomiting - Examination [quizlet.com]

  Tinnitus, a roaring in your ears, can be the result of loud noises, medicines or a variety of other causes. Meniere's disease may be the result of fluid problems in your inner ear; its symptoms include tinnitus and dizziness. [icdlist.com]

• Benign Paroxysmal Positional Vertigo

  The diagnosis mandates a thorough diagnostic workup, as the condition may mimic benign paroxysmal positional vertigo (BPPV) and Meniere's disease. [symptoma.com]

  The canalith repositioning procedure: for treatment of benign paroxysmal positional vertigo. Otolaryngol Head Neck Surg 1992; 107:399–404. CAS PubMed Google Scholar Nuti D, Vannucchi P, Pagnini P. [link.springer.com]

  Diagnostic value of repeated Dix-Hallpike and roll maneuvers in benign paroxysmal positional vertigo. Braz J Otor-hinolaryngol. 2017; 83(3): 243-8. [ Links ] 31. Brandt T, Diferential Diagnostics: Peripheral Versus Central Vertigo. [scielo.org.co]

  paroxysmal positional vertigo (also referred to BPPV) and labyrinthine concussion. [internationalbrain.org]

• Sudden Hearing Loss

  Vertigo or sudden hearing loss can occur from a PLF. [vestibular.org]

  Fluctuating or sudden hearing loss with or without vestibular symptoms in a patient with a previous stapedectomy, for example, is highly suggestive of perilymphatic fistula (PLF). [emedicine.medscape.com]

  Surgery is also indicated if there is a sudden hearing loss without improvement and/or continued symptoms beyond one month from a presumptive PLF. [internationalbrain.org]

  Fistulae can also cause sudden hearing loss, as well as other hearing related symptoms (such as tinnitus). [dizziness-and-balance.com]

• Ear Fullness

  Clinical presentation Symptoms of perilymphatic fistula may include: sensorineural hearing loss vertigo nausea tinnitus spatial disorientation dizziness ear fullness Radiographic features CT may demonstrate fluid-filling in the round window (especially [radiopaedia.org]

  The symptoms of PLF include hearing loss, tinnitus, ear fullness, dizziness, vertigo, imbalance, motion intolerance, nausea and vomiting. [tinnitusformula.com]

  His pioneering work indicated that the leak sites could be microfissures in the area just in front of the oval window or in the floor of the round window niche. 1 SYMPTOMS The symptoms of a PLF most commonly include ear fullness, fluctuating or “sensitive [vestibular.org]

• Hennebert's Sign

  Meniere's disease – (Hennebert's sign) 3. Hypermobile stapes footplate Which conditions can give rise to false-negative Fistula test? False-negative fistula test may be seen in following cases in spite of the presence of fistula: 1. [specialist-ent.com]

  "Positive Hennebert's sign in Meniere's disease." Arch Otolaryngol 103 (9): 524-30. Ostrowski VB, Hain TC, Wiet R: Pressure Induced Ocular Torsion. Archives in Otolaryngology-Head and Neck Surgery. 1997. [dizziness-and-balance.com]

• Vertigo

  Primary symptoms were hypoacusis, otorrhea, vertigo, tinnitus, and otalgia. All patients underwent preoperative CT scans and preoperative audiometry. [ncbi.nlm.nih.gov]

  Keywords: Labyrinthine fistula ; Vertigo [e-rvs.org]

• Dizziness

  Two-thirds had experienced dizziness. The fistula was limited to the lateral semicircular canal in 83 cases and involved the labyrinth more extensively in 14 instances. [ncbi.nlm.nih.gov]

  Dizziness and Vertigo When you're dizzy, you may feel lightheaded, woozy, or disoriented. If you feel like you or the room are spinning, you have vertigo. These feelings may make you lose your balance. Dizziness can have many different causes. [icdlist.com]

  These exercises can help to minimize your dizziness symptoms and may help improve your overall balance. Tips for avoiding dizziness There are some things that you can do to avoid becoming dizzy. [edisonstanfordhearing.com]

• Nystagmus

  Nystagmus could be commonly vertical c. Nystagmus usually is non fatiguing in nature d. [drtbalu.co.in]

  The postoperative vertigo with nystagmus is more frequent in larger fistulas. [ingentaconnect.com]

  On the window fistula the bone conduction decreased in all frequencies, as in Table 2. 4) About Nystagmus. Spontaneous nystagmus was observed in half of all cases, and beat towards the affected side. [oldfiles.bjorl.org]

  Fistula test: The basic of this test is to induce Nystagmus by producing pressure changes in the external canal which are then transmitted to the labyrinth. Stimulation of the labyrinth results in nystagmus and vertigo. [aippg.net]

  In the remaining 6 ears, pressure loading of the ear canal induced the sensation of vertigo without accompanying nystagmus. [ncbi.nlm.nih.gov]

The diagnosis may be difficult to attain due to striking similarities to BPPV and Meniere's disease, but in all patients who suffer from chronic otitis media and experience vestibulocochlear symptoms, a strong clinical suspicion must be present. A thorough patient history and a meticulous physical examination should be supported by audiology, vestibulometric studies, pressure test and CT of the temporal bone in order to differentiate between various clinical entities [1] [7].

Surgical treatment is the mainstay in these patients and current approaches include canal wall down mastoidectomy and either total or incomplete removal of cholesteatoma matrix [1] [3]. Surgery may be avoided, however, when a significant risk for postoperative complications (hearing deterioration or even deafness) exists, more commonly in the setting of extensive fistulas [3] [4]. For this reason, a careful preoperative evaluation is necessary to conclude what is the optimal therapeutic approach.

Several factors determine the outcome of labyrinthine fistula, including its size, location, severity of symptoms and experience of the surgeon [8], as hearing impairment may be a complication of treatment. Additionally, the type of fistula can determine the outcome - bony fistula affects only the bony labyrinth, while extensive fistula causes erosion of both bony and membranous labyrinths [4]. For these reasons, early recognition and treatment are mandatory in reducing the risk of adverse events.

Labyrinthine fistula almost always stems as a complication of chronic otitis media with cholesteatoma [3] [5] [7].

Labyrinthine fistulas are diagnosed in approximately 5-10% of patients who develop chronic otitis media with cholesteatoma [5], whereas others report a somewhat broader range - 3.6 to 13.9% [1]. The median age of patients was described to be 50 years in isolated reports [2], but neither gender nor age predilections have been determined so far.

Presumably, the chronic inflammatory state in chronic otitis media and the expanding cholesteatoma cause progressive erosion and loss of endochondral bone that protects the vestibulocochlear system [3]. When the bone barrier is breached, the leakage of perilymph from the inner ear into the middle ear occurs and disrupts the normal function of the labyrinth and tympanic membrane [1].

Early recognition of chronic otitis media and its treatment are by far the most important preventive strategies in reducing the risk of labyrinthine fistula development.

Labyrinthine fistula is a condition in which an abnormal connection between the perilymph-filled inner ear and air-filled middle ear exists, most likely as a complication of chronic otitis media with cholesteatoma [1] [2]. The fistula is created due to erosive loss of bone overlying the labyrinth and leakage of perilymph into the middle ear, giving symptoms such as vertigo and sensorineural hearing loss [2] [3]. About 5-10% of chronic otitis media with cholesteatoma patients develop a labyrinthine fistula and the most common location is the lateral semicircular canal [3] [4]. The diagnosis mandates a thorough diagnostic workup, as the condition may mimic benign paroxysmal positional vertigo (BPPV) and Meniere's disease [1]. Treatment principles rely on surgical correction of this inner ear pathology, with canal wall down mastoidectomy and removal of cholesteatoma matrix serving as possible approaches [5] [6] [7]. Complications such as deafness may arise post-operatively, however, which is why careful preoperative assessment by experienced surgeons is necessary to determine the optimal approach to labyrinthine fistula treatment.

Labyrinthine fistula is a condition in which chronic infection of the middle ear (otitis media) accompanied by cholesteatoma (an abnormal skin growth in the middle ear) creates a connection between the middle ear and the inner ear by destroying the bone overlying the inner ear. Damage to the labyrinth, the main structure of the inner ear, occurs as a result of these changes, causing leakage of perilymph from the inner ear into the middle ear, manifesting with symptoms such as dizziness and varying degrees of hearing loss. To make the diagnosis, it is necessary to perform a detailed physical examination and several tests to differentiate this condition from other that present with similar symptoms. Treatment requires surgical correction of the damage caused by chronic infection of the middle ear and cholesteatoma, but factors such as the size and location of the fistula and the severity of bone damage determine the optimal surgical strategy.

1. Garov EV, Antonian RG, Sheremet AS, Garova EE. The retrospective clinical study of labyrinthine fistula associated with chronic suppurative otitis media and their treatment [Article in Russian]. Vestn Otorinolaringol. 2012;(5):20-26.
2. Shim DB, Ko KM, Song MH, Song CE. A Case of Labyrinthine Fistula by Cholesteatoma Mimicking Lateral Canal Benign Paroxysmal Positional Vertigo. Korean J Audiol. 2014;18(3):153-157.
3. Ueda Y, Kurita T, Matsuda Y, Ito S, Nakashima T. Surgical treatment of labyrinthine fistula in patients with cholesteatoma. J Laryngol Otol Suppl. 2009;31:64-67.
4. Gersdorff MC, Nouwen J, Decat M, Degols JC, Bosch P. Labyrinthine fistula after cholesteatomatous chronic otitis media. Am J Otol. 2000;21(1):32-35.
5. Ghiasi S. Labyrinthine fistula in chronic otitis media with cholesteatoma. J Pak Med Assoc. 2011;61(4):352-355.
6. Quaranta N, Liuzzi C, Zizzi S, Dicorato A, Quaranta A. Surgical treatment of labyrinthine fistula in cholesteatoma surgery. Otolaryngol Head Neck Surg. 2009;140(3):406-411.
7. Busaba NY. Clinical presentation and management of labyrinthine fistula caused by chronic otitis media. Ann Otol Rhinol Laryngol. 1999;108(5):435-439.
8. Chen Z, Dongzhen, Wu Y, et al. Surgical treatment of labyrinthine fistula caused by cholesteatoma with semicircular canal occlusion. Acta Otolaryngol. 2010;130(1):75-78.