Perilymphatic Fistula: an Approach to Diagnosis and Management That

Home , Round window, Stapedectomy

ORIGINAL ARTICLE International Tinnitus Journal. 2012;17(1):61-66. Perilymphatic Fistula: an approach to diagnosis and management that provides surer diagnosis and provides medical and surgical management options: report of six illustrative recent cases

Nitin Bhatia Joel F Lehrer1

Abstract

Perilymphatic Fistula (PLF) remains a controversial topic and its management is challenging. Various etiologic events have been proposed and spontaneous PLF has been described 1. Diagnostic methods and strategies have been reported in the literature but no standard algorithm exists. The most accepted diagnostic method remains intra- -operative confirmation of perilymph leak at oval window or round window. However, concerns have been raised regarding excessive surgical intervention given the lack of pre-operative confirmatory tests. We have developed a diagnostic strategy that has been successful in our hands. The aim of this study is to present six patients with PLF who underwent surgical repair. We describe our approach in the evaluation and management of these patients. We discuss the diagnostic tests and the operative technique used in management of these patients and we provide a review of the literature in support of our approach.

Keywords: dizziness, hearing loss, sensorineural, vertigo, vestibular diseases, vestibular function tests.

1Otolaryngology-Head and Neck Surgery - Holy Name Hospital - Teaneck - United States. E-mail: [email protected] Send correspondence to: Northern Jersey ENT Associates, 1 Degraw Avenue, Teaneck, NJ, USA. 07666. Paper submitted to the RBCMS-SGP (Publishing Management System) on June 19, 2012; and accepted on August 16, 2012. cod. 96.

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17(1).indb 58 02/01/2013 13:41:05 INTRODUCTION A complete audiometric evaluation is then performed, as is the suprathreshold stimulus stapedial Perilymphatic fistula (PLF) has become an testing and fistula testing. Suprathreshold stimulus integral part of modern Neurotology. PLF is an abnormal stapedial testing was performed with stimulation being communication between the perilymphatic space and presented to the contralateral ear at 0.5k, 1k, and 2k middle ear space. PLF most commonly occurs in the Hz at 110 dB. Evaluation of symmetry of response, and round window niche and/or the oval window at the abnormalities in latency, amplitude and slope of the fissula ante fenestram. Various causes of PLF have response are performed20. Fistula testing was performed been described including stapedectomy, barotrauma, with an impedance bridge using positive and negative physical exertion, head and neck trauma, congenital pressure, sweeping the pressure from +400 dapa to 2-4 and idiopathic . However, the diagnosis of PLF remains -400 dapa three times in each of four test situations, the a subject of controversy due to the variability in patient patient sitting eyes open, the patient standing eyes open, presentation and difficulty in pre-operative evaluation. the patient sitting eyes closed, and the patient standing Multiple tests have been described but no definitive eyes closed13. The audiologist performs this test, looking diagnostic tool is available at present to confirm presence for nystagmus or a sway or complaints of dizziness, of PLF preoperatively. Thus there are no firm guidelines lightheadedness, disorientation or nausea. for medical and surgical intervention. Because PLF is a ENG has not shown to be helpful in diagnosing controversial diagnosis, it is crucial to discuss it in our PLF but it was performed in all patients with suspected literature and perhaps come to a consensus. In this PLF to evaluate for the presence of vestibular function. paper, we describe 5 patients with post-traumatic PLF, Vestibular function was intact in all of our cases. and one patient with spontaneous PLF. We describe The Lasix dehydration test, as previously described our algorithm in diagnosing PLF and review the literature in the literature, was administered to patients with supporting our approach. Additionally, we describe our suspected PLF5. Patients with sulfa allergy underwent surgical technique in treatment of PLF. the Glycerin test6. Intramuscular injection of 60mg of Lasix was administered in the left buttock. Pure tone MATERIALS AND METHOD thresholds, speech reception thresholds, fistula testing, 21 Evaluation of a patient with suspected PLF begins and Quix and Romberg testing were performed before with a thorough history including a detailed history the injection and every 30 minutes subsequently for 2 regarding the mechanism of trauma. A standard ear, hours and changes in pure tone responses, speech nose, throat and neck examination is performed. A discrimination and speech reception thresholds, focused neuro-otological examination is performed fistula testing, and Quix and Romberg balance testing including cranial nerve examination, examination of were observed. If the findings improved, it indicated spontaneous and positional nystagmus, and balance presence of Endolymphatic Hydrops which confirmed 21 examination that includes Romberg and Quix testing presence of PLF . Endolymphatic Hydrops also occurs (Table 1). in Ménière’s disease. However, patient’s history and the definitive episodes of spinning vertigo differentiate the Endolymphatic Hydrops due to Ménière’s disease Table 1. Evaluation of PLF. from PLF. According to the 1995 American Academy of History Otolaryngology - Head and Neck Surgery guidelines Physical Examination describing probable, possible, definite, and certain Complete Head and Neck Examination Ménière’s disease, one of the required criteria is to rule out other possible causes of patient’s symptoms, one of Cranial Nerve Examination which can be PLF23. Spontaneous and Positional Nystagmus During the Lasix dehydration test, patients may Balance Examination - Romberg and Quix test show improvement, worsening, or have no change in Audiological Evaluation their audiometric and physical examination findings. Pure tone (Figure 1) If the symptoms worsen, perilymphatic 24 Tympanogram hypertension may be suspected . If there is no change, it indicates no evidence of inner ear fluid disorder. If the Suprathreshold Stimulus Stapedial Testing patient’s symptoms and physical exam findings improve Fistula Testing during the Lasix test, management options include ENG medical therapy with oral Lasix or surgical treatment with Lasix Dehydration Test (or Glycerin) middle ear exploration.

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17(1).indb 59 02/01/2013 13:41:05 RESULTS

The presenting complaint of all patients was persistent imbalance. Post-traumatic cases were seen from three months to five years after the trauma. None of our patients reported hearing loss as a result of the trauma. One patient, who was 97 years old, had moderate to severe hearing loss prior to the trauma. The remaining patients had normal hearing at all times. All patients had imbalance on Romberg and Quix testing and tested positive on fistula testing. The Lasix dehydration test was performed after suspecting PLF and all patients showed improvement in their balance during the test (Table 2). Oral treatment with Lasix was offered to all six patients. However, due to various factors, these patients were unable to tolerate Lasix and opted for surgical treatment. Surgery was performed between eight months and Initial treatment in all patients with suspected PLF six years from the time of the injury. Surgical interven- consists of oral Lasix. In our hands, loop diuretics, not tion included fat grafting at the round and oval windows hydrochlorothiazide, have shown success. If the patient as described above. On post-operative follow up, all could not tolerate medical therapy or if symptoms persist, patients had improvement in their symptoms and their we offer the option of surgical intervention. If a decision is examination. Five out of 6 patients had normal Romberg made to perform surgery, informed consent is obtained and Quix test. One patient had improvement in balance and the risks and benefits are explained to the patients. but not complete resolution. This patient has a suspicion All cases were performed under general anesthesia. of fistula in the contralateral ear as well, and ultimately Patients were placed in slight Trendelenberg position. did undergo surgery on the opposite ear. There were Three 2 mm size fat grafts are harvested from the ear no complications related to hearing loss, perforated lobe and are dissected into 0.5 mm segments for grafting. tympanic membrane or any other surgical complications. The grafts are securely kept in saline. The middle ear is approached via transcanal approach. The ear canal is DISCUSSION injected with 1% lidocaine with 1:60,000 epinephrine. A number 2 type endaural incision is made at the superior Patients with vestibular disorders present with a canal wall to widen the canal and better visualize the unique challenge, as they are difficult to diagnose and superior tympanic membrane. A Shea self-retaining treat. Definitive tests and imaging are useful in only a retractor is placed in the ear canal and a tympanotomy minority of patients. PLF is one of the most challenging flap is lifted up to the annulus. Prior to entering the middle and controversial diagnosis among vestibular disorders. ear space, special attention is paid towards adequate Unfortunately, because this is a controversial diagnosis, hemostasis to avoid any blood accumulating in the people have omitted this diagnosis in their differential middle ear. The annular ligament is then lifted anteriorly diagnosis of vestibular disorders. But there are patients and the middle ear is entered. The round window and who are suffering from PLF and it should be addressed. the oval window are inspected to assess any leakage of It is our opinion that in PLF patients, it is the chronic clear fluid. Occasionally a bony overhang is noted over distortion in endolymph and perilymph that results in the the round window, which is removed using a micro drill. loss of the normal perception of gravity. Any fluid noted at the round window or the oval window While spontaneous PLF and congenital PLF have is gently suctioned with a number 24 otologic suction been described, post traumatic, post-stapedectomy and and reaccumulation is confirmed, as described by Kohut, barotrauma are well-documented causes of PLF, being et al. to confirm the diagnosis of PLF13. The mucosa is described in the literature. Traumatic PLF was initially then denuded using a curved otologic pick and 3-4 0.5 described as a result of sudden increase in ICP7. Up to mm fat graft are placed at each site and are covered with 50% of post-traumatic PLF have been reported to be gel foam. The flap is then replaced and the ear canal is present bilaterally8. Firber-Viart et al. described accidents packed with quarter inch packing impregnated in Poly- where injuries secondary to airbags cause PLF9. Goodhill sporin ointment over strips of gel film. presented his series where he indicated that the trauma

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17(1).indb 60 02/01/2013 13:41:05 - - Comments Abdominal with Pain oral Lasix to get Wants pregnant; Lasix con traindicated Severe we akness with oral Lasix Excessive urination with oral Lasix Excessive fatigue with oral Lasix Could not tolerate oral Lasix after 1 year - - Post -operative Normal Quix and Romberg Normal Quix and Romberg Normal Quix and Romberg Partial Improve ment Normal Quix and Romberg Normal Quix and Romberg Intervention RW OW, Repair RW OW, Repair RW OW, Repair RW OW, Repair RW OW, Repair RW OW, Repair Lasix Test Improvement Improvement Improvement Improvement Improvement Improvement Fistula Test Left > Right Left = Right Left > Right Right > Left Left > Right Left Only Audiogram Normal Normal Moderate SNHL Normal Normal Normal Spontaneous Nystagmus Absent Absent Absent Absent Absent Absent Positional Positional Nystagmus Absent, buy dizzy on sitting up Absent, buy dizzy on sitting up Absent Absent Absent Absent, buy dizzy on sitting up Quix Shaky right sway Shaky Shaky falls back Shaky Romberg slight shaky slight shaky shaky shaky falls back shaky Chief Complaint Persistent Dizziness Persistent Dizziness Persistent Dizziness Persistent Dizziness Persistent Dizziness Persistent Dizziness Time to Time surgery 33 months 8 months 18 months 26 months 9 months 78 months Time to Time presentation 12 months 3 months 5 months 3 months 4 months 60 months Injury MVA MVA MVA MVA spontaneous Fall Patients Testing and Results. Testing Patients Patient 1 2 3 4 5 6 Table 2. Table

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17(1).indb 61 02/01/2013 13:41:05 need not be severe. Even whiplash injury to the neck utilized in Ménière’s disease can be helpful in diagno- can cause PLF10,11. Contrary to popular belief, only half sing PLF, as long as the patient’s history is appropriately of patients with traumatic PLF have hearing loss. considered. Chronic or persistent combinations of disequilib- Inner ear dehydration tests have been described rium, headache, adult-onset motion sickness, neuro- for diagnosis of Ménière’s disease. Since endolymphatic cognitive disruption exacerbated by exertion or physical hydrops is also present in PLF as described in the litera- activity, and hearing loss have all been described as ture, various inner ear dehydration tests have been used symptoms of PLF. In patients with these symptoms, in PLF4-6. We use the Lasix dehydration test because it findings of positive Romberg, ataxia and positional nys- is the most convenient for patients and it provides the tagmus should raise the suspicion of PLF. Healy et al. opportunity for medical therapy. Patients are themselves described episodic positional vertigo and ataxia between able to witness improvement in their imbalance during the episodes as chief complaints in their series of pa- the test. Loop diuretics are effective in our hands whereas tients with PLF12. Their criteria for middle ear exploration thiazide diuretics are not. If patients’ symptoms get worse included abnormal gait, positional nystagmus, positive on the Lasix dehydration test, a presumptive diagnosis Romberg, and positive fistula test. Hearing loss was a of perilymphatic hypertension can be made24. less common complaint in their cohort, whereas others If medical therapy is contraindicated or not to- have reported hearing loss to be the primary complaint lerated by the patient, surgical intervention is offered. in patients with PLF7,10. During the surgical procedure, certain steps are crucial Although multiple tests are available to assist a to provide the best outcome. It is important to have a physician in diagnosing PLF, controversy exists in the broad view with a large ear speculum. A superior en- utility of these tests. The use of fistula test in addition to daural incision can provide a wider surgical field before the classic physical exam findings in identifying patients entering the middle ear. Maintaining a bloodless field with PLF has been well documented12. During fistula test- prior to raising the annulus and entering in the middle ear ing, dizziness may occur in patients with PLF by applying avoids pooling of blood around the oval window or the positive and negative pressure in EAC, which results in round window niche. In order to better visualize the leak, pressure changes in inner ear, with positive fistula test- we place the patients in Trendelenberg which increases ing more likely occur in the symptomatic ear. The usual intracranial and perilymph pressure. Other techniques fistula test is performed using a pneumatic otoscope have been described including Valsalva and Internal which only provides positive pressure. However, Kohut Jugular compression to assist in visualizing the leakage et al. have found that negative pressure is a more sensi- of clear fluid from the oval and the round window. We tive test, and therefore important to perform as part of confirm the leak by suctioning and observing the reac- the fistula testing13. cumulation of fluid according to the method of Kohut13. Multiple diagnostic modalities have been described Before placing the fat grafts, we denude the mucosa in to help diagnose PLF. Moving platform fistula test has the area of the fissula ante-fenestram in the oval window not been widely used14. Transtympanic endoscopy and area and the floor of the round window niche inferior to the use of fluorescein have been described as methods the round window membrane in order to provide a raw of documenting a fistula without operation15,16. However, surface for the graft to adhere. If a leak is seen at one of injection of this dye has been found to be inaccurate in the two areas, we graft both because of the possibility the diagnosis of PLF as dye has been found in serum as of an inapparent fistula at the second window. well as perilymph. This procedure has not been reported We have discussed our evaluation and manage- in recent literature because of its lack of efficacy. Standard ment of patients with PLF. We encourage our colleagues ECOG, transtympanic ECOG, and intraoperative ECOG to include PLF in their differential diagnosis and to consi- have also been described in the diagnosis of Ménière’s der our diagnostic approach and testing in their evalua- Disease and in the evaluation of PLF but these tests also tion of these patients. Our experience is that imbalance have not been used widely17,18. In our practice, we have can be reversed long after the insult but hearing loss utilized tympanic electrodes to perform ECOG. cannot be reversed after several weeks, so that prompt Most recently, magnetic resonance imaging of attention (usually within a week) is required in cases of inner ear after intratympanic injection of gadolinium has PLF with sudden hearing loss, such as those seen in been described for evaluation of Ménière’s disease19. If a SCUBA divers. patient has a history more suggestive of PLF, an increa- sed signal on MRI, indicating hydrops secondary to PLF CONCLUSION should be considered because although Endolymphatic Hydrops is a pathologic correlate of Ménière’s disease, PLF is a condition that has multiple causes and it has also been observed in PLF. Thus, a diagnostic test can present with varying symptoms. PLF should be considered as part of the differential diagnosis in patients

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