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24. Ichijo H. Cupulolithiasis of the posterior semicircular canal. Am J Otolaryngol. 2013;34(5):458–63.

25. Asprella LG.Diagnostic and treatment strategy of lateral semicircular canal canalolithiasis. Acta Otorhinolaryngol Ital. 2005;25(5):277–83.

26. Choung YH, Shin YR, Kahng H, Park K, Choi SJ. ‘Bow and lean test’ to determine the affected ear of horizontal canal benign paroxysmal positional vertigo. Laryngoscope. 2006;116(10):1776–81.

27. Hiruma K, Numata T.Positional nystagmus showing neutral points. ORL. 2004;66(1):46–50.

28. Bisdorff AR, Debatisse D.Localizing signs in positional vertigo due to lateral canal cupulolithiasis. Neurology. 2001;57(6):1085–8.

29. Ko KM, Song MH, Kim JH, Shim DB.Persistent spontaneous nystagmus following a canalith repositioning procedure in horizontal semicircular canal benign paroxysmal positional vertigo. JAMA Otolaryngol Head Neck Surg. 2014;140(3):250–2.

30. Califano L, Vassallo A, Melillo MG, Mazzone S, Salaa F.Direction-xed paroxysmal nystagmus lateral canal benign paroxysmal positioning vertigo (BPPV): another form of lateral canalolithiasis. Acta Otorhinolaryngol Ital. 2013;33(4):254–60.

31. Vannucchi P, Pecci R.About nystagmus transformation in a case of apogeotropic lateral semicircular canal benign paroxysmal positional vertigo. Int J Otolaryngol. 2011;2011:687921.

32. Yacovino DA, Hain TC, Gualtieri F.New therapeutic maneuver for anterior canal benign paroxysmal positional vertigo. J Neurol. 2009;256(11):1851–5.

33. Fetter M, Haslwanter T, Bork M, Dichgans J.New insights into positional alcohol nystagmus using three-dimensional eye-movement analysis. Ann Neurol. 1999;45(2):216–23.

34. Kim CH, Choi JM, Jung HV, Park HJ, Shin JE.Sudden sensorineural hearing loss with simultaneous positional vertigo showing persistent geotropic direction-changing positional nystagmus. Otol Neurotol. 2014;35(9):1626–32.

35. Imai T, Matsuda K, Takeda N, Uno A, Kitahara T, Horii A, etal. Light cupula: the pathophysiological basis of persistent geotropic positional nystagmus. BMJ Open. 2015;5(1):e006607.

36. Fife TD, Iverson DJ, Lempert T, Furman JM, Baloh RW, Tusa RJ, etal. Practice parameter: therapies for benign paroxysmal positional vertigo (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2008;70(22):2067–74.

37. Foster CA, Foster CA, Ponnapan A, Zaccaro K, Strong D.A comparison of two home exercises for benign positional vertigo: half somersault versus Epley maneuver. Audiol Neurotol Extra. 2012;2(1):16–23.

38. Oron Y, Cohen-Atsmoni S, Len A, Roth Y.Treatment of horizontal canal BPPV: pathophysiology, available maneuvers, and recommended treatment. Laryngoscope. 2015;125(8):1959–64.

39. Lempert T, Tiel-Wilck K.A positional maneuver for treatment of horizontal-canal benign positional vertigo. Laryngoscope. 1996;106(4):476–8.

40. Asprella Libonati G, Gagliardi G, Cifarelli D, Larotonda G. “Step by step” treatment of lateral semicircular canal canalolithiasis under videonystagmoscopic examination. Acta Otorhinolaryngol Ital. 2003;23(1):10–5.

41. Kim J-S, Oh S-Y, Lee S-H, Kang J-H, Kim DU, Jeong S-H, et al. Randomized clinical trial for apogeotropic horizontal canal benign paroxysmal positional vertigo. Neurology. 2012;78(3):159–66.

42. Kim YK, Shin JE, Chung JW. The effect of canalith repositioning for anterior semicircular canal canalithiasis. ORL J Otorhinolaryngol Relat Spec. 2005;67(1):56–60.

43. Foster CA.Overcoming positional vertigo. Boulder, CO: Bull Publishing; 2019. p.200.

44. Agrawal SK, Parnes LS. Human experience with canal plugging. Ann N Y Acad Sci. 2001;942:300–5.

45. Brantberg K, Bergenius J.Treatment of anterior benign paroxysmal positional vertigo by canal plugging: a case report. Acta Otolaryngol. 2002;122(1):28–30.

C. A. Foster

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46. Uetsuka S, Kitahara T, Horii A, Imai T, Uno A, Okazaki S, etal. Transient low-tone air-bone gaps during convalescence immediately after canal plugging surgery for BPPV.Auris Nasus Larynx. 2012;39(4):356–60.

47. Hunt WT, Zimmermann EF, Hilton MP. Modications of the Epley (canalith repositioning) manoeuvre for posterior canal benign paroxysmal positional vertigo (BPPV). Cochrane Database Syst Rev. 2012;2012(4):CD008675.

48. Faralli M, Longari F, Ricci G, Marinetti D, Frenguelli A.Mastoid oscillation in the treatment of the apogeotropic variant of benign paroxysmal positional vertigo of the lateral semicircular canal. Mediterr J Otol. 2008;4:152–6.

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Chapter 8

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Traumatic Causes ofVertigo

Christopherde Souza, Rosemariede Souza, AishanPatil, AdipShetty, VimalSomeshwar, andManishSrivastav

Perilymph Fistulae

Christopherde Souza, Rosemariede Souza, AishanPatil and AdipShetty

Perilymphatic stula (PLF) can be dened as an abnormal communication between the inner ear (the cochlear and the vestibule) and the middle ear cavity, mastoid, or intracranial cavity.

Tests to currently diagnose PLFs have a signicant limitations, lacking the sensitivity and specicity that is needed to provide an accurate and consistent diagnosis. Major improvements in radiological imaging modalities and emerging technologies including the use of biomarkers have recently shown immense promise to help diagnose PLF.PLFs cause cochlear and vestibular symptoms which can cause signicant disability.

PLFs can be divided into two groups. Those patients in whom a cause or an antecedent event can be identied and those in whom a possible cause cannot be identied.

C. de Souza (*) Lilavati Hospital, Mumbai, India

R. de Souza Department of Internal Medicine, BYL Nair Hospital, Mumbai, India

A. Patil Vascular Surgery, Borders General Hospital, Melrose, Scotland, UK

A. Shetty Rajawadi Hospital, Mumbai, India

V. Someshwar · M. Srivastav KD Ambani Hospital, Mumbai, India

https://doi.org/10.1007/978-3-031-40524-2_8

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Surgical techniques for stapes surgery, especially the need for a reliable seal over the neo fenestra, have evolved considerably through the years. PLFs present as a complication in approximately in 1% of stapedotomy procedures. The incidence of PLF increases signicantly in those individuals requiring revision stapedectomies. It is found that one third of patients undergoing revision stapedectomy will likely develop PLF.

C. de Souza et al.

Perilymphatic Fistula Post Stapedectomy

A perilymph stula is one of the common causes of sensorineural hearing loss following stapedectomy. It is thought that perilymph stulae occur because of inadequate closure of the fenestra at the footplate, too long of a prosthesis, or possible increased perilymph pressure. The diagnosis and treatment of this condition is vital, because it has the potential to cause irreversible profound sensorineural hearing loss, as well as puts the patient at risk of developing meningitis. Newlands [1] reported on a patient who developed acute otitis media, labyrinthitis, and meningitis 16months following stapedectomy. Poststapedectomy PLF carries an increased risk of labyrinthitis and meningitis. Should labyrinthitis occur with or without meningitis, stula repair must be undertaken as soon as the infection has been treated [2].

Perilymph stulae have been observed with all types of stapes surgeries and with all techniques. Even as early as 1961, Lewis [3] described a perilymphatic stula, observing that the polyethylene strut used was responsible for perilymph leakage.

Signs andSymptoms of Perilymphatic Fistula

The most common symptoms are

1. Fluctuating sensorineural hearing loss

2. Roaring tinnitus

3. Vertigo

4. Fullness in the ear

While these symptoms are also associated with endolymphatic hydrops, the key to making a diagnosis of perilymphatic stula is the proximity to stapedectomy surgery. If it occurs immediately following surgery, the diagnosis of perilymphatic surgery is obvious. This is termed as “primary” or “early” perilymphatic stula. If instead symptoms occur long after stapedectomy, then establishing a diagnosis of perilymphatic stula may not be as straightforward. This stula is termed “delayed” or “secondary” perilymphatic stula. (For criteria to diagnose PLF see Table8.1).

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Table 8.1

Criteria to diagnose a denite PLF

(a) Fluctuating or nonuctuating hearing loss (b) Tinnitus (usually described as being “roaring”) (c) Vertigo (d) Aural fullness

1. (a) External trauma to the ear, blow to the head, exposure to an explosive blast. Or (b) Barotrauma caused by events like, e.g., nose-blowing, sneezing, skydiving, underwater

diving, straining, or lifting of heavy weights.

2. Direct trauma to the inner ear caused by Q-tip injury, stapedotomy operation, temporal bone fracture (a) Laboratory testing for a perilymph biomarker with high sensitivity and specicity.

Incidence

Perilymph stulae account for up to 9%–10% of stapedectomy failures [4–6]. However, other analyses have documented a far lower incidence. Feldman and Schuknecht [7] analyzed 154 revision stapedectomies and reported that perilymphatic stulae were found in just ve cases. The incidence of perilymphatic stulae as a cause of failure in stapedectomy is approximately the same for both short term and long term follow up [6].

“Early” (Primary) Perilymph Fistula

Stapedectomy by necessity involves the creation of a stula for the insertion of the prosthesis. The primary perilymph stula occurs when the stula created at the time of surgery persists and fails to seal off the vestibule from the middle ear. This can occur immediately following surgery, and symptoms can persist for days and weeks following surgery.

Signs andSymptoms

The typical symptoms consist of hearing loss, tinnitus, and vertigo. Since these symptoms are also typical of endolymphatic hydrops, a perilymphatic stula can be confused with endolymphatic hydrops.

The most common symptom in some series is vertigo, while in others the most common symptom is a drop or a uctuation in hearing [8]. Harrison et al. [9] reviewed 46 cases of poststapedectomy perilymphatic stulae and found hearing loss or uctuating hearing loss to be the most common symptom in 87% of cases. Moon [10] examined 49 cases of poststapedectomy perilymphatic stulae and found that 71% of cases with primary stulae, and 78% of cases with secondary

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perilymphatic stulae presented with hearing loss as their chief complaint. Moon also reported that a pure sensorineural hearing loss is the most common type of loss in both primary and secondary stulae. Rarely, mixed hearing losses and pure conductive hearing losses can occur from a perilymphatic stula. Goodhill [11] noted that a perilymph stula may present only as a uctuating or persistent conductive hearing loss. He postulated that the ratio of the transducer effect of the prosthesis to the perilymph spillage effect is less than ideal in such patients, thus accounting for their symptoms.

Vertigo may result from semicircular canal dysfunction leading to rotatory vertigo, or may have a utricular origin which results in a sense of falling. Harrison etal. [9] found that 35% of their patients complained of vertigo, while 39% had a sensation of imbalance. Moon [10] found that 77% of primary stulae and 61% of secondary stulae caused disequilibrium.

Tinnitus was found in 28% of patients as reported by Harrison etal. [9]. Moon [10] found tinnitus to be present in 45% of patients who had a perilymph stula.

The signs and symptoms vary with the size of the stula. Large stulae result in rapid hearing loss, tinnitus, and vertigo. Morrison [12] noted that such large stulae accompanied technical difculties by the operating surgeon at the time of stapedectomy. Fluctuation in hearing loss is unlikely to be a feature in such a situation. In early perilymph stulae, when the leak is small, the hearing loss may initially appear as a conductive hearing loss; then has a sensorineural component; then progressing to a total sensorineural hearing loss. When such a stula is repaired late, the hearing does not improve, though vertigo may resolve. When a small stula is present, the only evidence of the stula may be failure to achieve adequate closure of the air– bone gap, with mild uctuation in hearing and a small decreases in speech discrimination scores.

C. de Souza et al.

Cause of Primary Perilymph Fistula

Improperly sealed oval window fenestra are the likely cause of such stulae. Goodhill [11] stated that if a mucosal seal does not hermetically seal off the vestibule from the middle ear at the time of surgery, then the chances of such a stula forming are high. Failure to reect the mucoperiosteal aps may allow the lacerated edges of tissue to extend down into the vestibule and prevent formation of a new endosteal membrane at the level of the oval window. A prosthesis that is too long may also prevent the stula from sealing off.

Numerous studies [13–15] have shown that the use of gelatin sponge (gelfoam) as a seal for the oval window fenestra is associated with a high incidence of perilymph stula. Sheehy and Perkins [13] compared gelatin sponge, fat and fascia as seals for the fenestra and found that the incidence of perilymph stula was 3.5% when gelatin sponge was used, 1.9% when fat was used, and 0.6% when fascia was used. Lippy and Schuring [16] compared the incidence of perilymph stula formation with the use of gelatin sponge versus that of the Robinson vein graft prosthesis.

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They reported a signicant difference: 50% for gelatin sponge and 4% for tissue seals. Gelatin sponge is thus inadequate as an oval window seal because:

1. It may be resorbed before the neomembrane has formed.

2. The gelatin sponge will get softened by the perilymph, and the prosthesis will

penetrate through the gelatin sponge causing a perilymph stula.

3. The neomembrane that forms with a gelatin sponge is very thin and gets perfo-

rated repeatedly by the prosthesis leading to the formation of a perilymph stula.

Linthicum [17] in his report provided evidence that gelatin sponge when used as an oval window seal is more likely to cause perilymph stula. Causse etal. [18] recommended using a tissue seal over the oval window fenestra to prevent perilymph stula formation.

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Late (Delayed or Secondary) Perilymphatic Fistula

Such a stula can occur long after a successful stapedectomy. Morrison [12] states that this is often the cause of sensorineural deafness that sometimes occurs long after a successful stapedectomy. Most authorities state that the longer stapedectomy patients are followed up, the greater the chances of perilymphatic stula occurring. He found that in 50% of late perilymphatic stulae, no obvious cause could be discerned (in the other patients, mountaineering, lifting heavy objects, coughing, sneezing, pressure changes [barotrauma] from ying, and head injuries were suspected as the cause). Otitis media following stapedectomy is not likely to cause a perilymph stula.

How Does aPerilymph Fistula Form?

Glasscock [19] noted that the cochlear aqueduct is patent in a majority of human adults. It puts the cerebrospinal uid (CSF) in potential communication with the perilymph. Pressure in the cerebrospinal uid is transmitted directly to the perilymph. Shea [20] noted that CSF pressure in the lumbar spine is 150 mmH2O and that the pressure in the cistern through the cochlear aqueduct can be as high as 350 mmH2O (millimeter of water). In the event of the Eustachian tube getting blocked, negative pressure in the middle ear builds up and can reach minus 600 mmH2O.Thus, a potential gradient of 950 mmH2O is created, which has the potential to push the perilymph out of the vestibule. This can prevent the seal over the fenestra from healing, resulting in a perilymph stula.

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C. de Souza et al.

Clinical Findings

Clinical examination of the ear may appear normal in cases of even active PLF.Occasionally the tympanic membrane will be retracted indicative of underlying Eustachian tube dysfunction.

Audiometric Evaluation

Pure tone audiometry in the setting of a PLF typically reveals sensorineural hearing loss in the low frequencies followed by a at sensorineural hearing loss that uctuates. Recruitment will be present in the initial stages and short increment sensitivity index (SISI) scores are often above 75%. In the early stages, speech discrimination scores uctuate with the pure tone thresholds; later on they may lower disproportionately. A variable conductive hearing loss may be seen.

Vestibular Tests [21]

Hallpike Caloric Tests

Canal paresis or a hypoactive response is a likely nding in cases of PLF.However, because there is normally a high incidence of diminished caloric response after stapedectomy, it may become difcult to assess the true value of its signicance in using it to diagnose the presence of a perilymph stula.

Electronystagmography (ENG) or Videonystagmography (VNG)

ENG/VNG is relatively insensitive to the presence of a perilymphatic stula. While it may reveal a direction xed positional nystagmus, this nding in itself is not diagnostic of a perilymph stula.

Fistula Test

Fistula tests with a pneumatic otoscope is a simple test that can be done as part of a routine clinical exam. It involves applying positive pneumatic pressure to the ear canal under a seal. In a positive test, nystagmus will develop. However, this test has been found to be negative in one-third of the cases. However, when the stula test is combined with results from an ENG/VNG, a higher degree of accuracy for PLF diagnosis has been reported. Beales [21] studied 16 patients who had a negative

8 Traumatic Causes ofVertigo

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stula test but in whom a stula was suspected were reoperated to rule out a stula. Only one had a perilymph stula; while out of six patients with a positive ENG stula test, ve were found to have a stula. Thus, the incidence of false-positive and false-negative ndings was low enough for the authors to conclude that this test was reasonably reliable.

151

Radiological Evaluation

Kosling etal. [22] reported on the use of high-resolution CT scanning to detect the presence of perilymphatic stula. They took high-resolution CT scans (1mm slice thickness) in the axial and coronal planes They reported an air bubble (in the vestibule) at the end of the prosthesis to be an indirect sign of the presence of a perilymphatic stula. This was the nding in six patients, and all six were found to have perilymphatic stula. Thus, CT scans were found to be of value in detecting the presence of perilymphatic stula poststapedectomy.

How Can aPerilymph Fistula BePrevented?

A number of suggestions have been put forward to reduce the likelihood of developing a poststapedectomy PLF:

1. Reect the mucosa off the footplate completely before making a fenestra.

2. Some have suggested that a stapedotomy technique is less likely to result in

perilymph stula formation as compared to a partial or total stapedectomy, though denitive studies comparing these techniques with regard to the incidence of PLF has not been done.

3. A tissue graft seal over the fenestra acting as a barrier between the perilymph

(vestibule) and the prosthesis is highly recommended.

4. The prosthesis should be securely placed on the incus to prevent it from

migrating.

5. Avoid using gelatin sponge as a seal.

Additional postoperative recommendations during the rst several weeks after surgery that the patient can do to limit the development of PLF include:

1. Avoid trauma to the head.

2. Coughing and sneezing with the mouth wide open.

3. Avoid straining against a closed glottis.

4. Avoiding activities that may increase the risk of barotrauma.

5. Avoid lifting heavy weights.

6. Reporting to the surgical team immediately if symptoms such as vertigo, tinni-

tus, or hearing loss manifest themselves.

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C. de Souza et al.

Management of aPerilymph Fistula

Surgical closure of the stula is the treatment of choice. Many surgeons consider this to be a surgical emergency. It is important that the eld be kept dry to inspect the oval window under high powered microscopy to assess for the presence of perilymph. If the leak is not visible, then the patient’s head should be lowered and the internal jugular vein on the same side compressed in an effort to make the leak visible. If the patient is under general anesthesia, then the anesthesiologist can also elicit a Valsalva maneuver to see if perilymph accumulates around the oval window.

Causse etal. [23] devised a test to help detect a perilymph stula intraoperatively. They used a 1.0mm diamond burr to remove brous adhesions from the oval window (this technique can also be performed with a laser).

To conrm that a stula is present, a small piece of gelfoam is placed at the site of the suspected stula. This gelfoam is then tested on Clinitest glucose test paper. If the paper turns red, then perilymphatic uid is present, indicating the presence of a perilymphatic stula. Once the leak is detected, the stulous tract is excised and the prosthesis removed. Lasers have proven to be immensely helpful and are excellent working tools in such a situation. The mucosa over the footplate is elevated completely. A fresh soft tissue seal is placed over an adequately created fenestra and a new prosthesis is placed over the seal. The patient is advised total bed rest for 48h.

Pirodda etal. [24], in a retrospective analysis of 26 cases, reported on their ndings of their approach to the management of rapid deterioration in bone conduction thresholds following stapes surgery. Seven were treated conservatively with medication (pharmacologically with steroids steroids), and the other nineteen were treated with surgery and medication. Of the seven treated medically, improvement was seen in three, while in the other four hearing remained unchanged. In the nineteen cases managed with surgery and medication, in ve cases a perilymphatic stula was found at surgical exploration and four of these ve improved. Of those where no stula was found at surgery, four worsened, and eleven cases experienced no change. The authors concluded their study by advocating a combined medical and surgical approach to treating poststapedectomy perilymphatic stula.

Results ofTreatment ofPoststapedectomy Perilymph Fistula

Improvement of hearing, especially once sensorineural hearing loss is present, is minimal. Early repair of perilymph stulae does help symptoms such as vertigo resolve. Tinnitus may not resolve completely. Thus ideally, PFL is diagnosed early, and treated as a surgical emergency.

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