SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

TREATMENT AND MANAGEMENT OF PERILYMPHATIC FISTULA

Dudley J. Weider, M.D., F.A.C.S.

This article reports on 45 cases of spon­taneously occu r r i ng per i lymphat ic fistula with 8 p a t i e n t s p r e s e n t i n g with b i la te ra l involvement , 7 with invo lvement of b o t h oval a n d r o u n d window in the same ear, and 7 with fistulas believed to be of congen­i ta l o r i g i n . S e v e n t e e n p a t i e n t s ( 3 8 % ) r e q u i r e d revision surgery. Endolympha t ic s h u n t , l a b y r i n t h e c t o m y , b l o c k i n g t h e cochlear aqueduct , and streptomycin abla­tion were used variously for symptom control. Diagnosis, t rea tment , and m a n a g e m e n t of these patients is discussed.

Weider and Johnson in 19881 repor ted on 36 cases of perilymphatic fistula (PLF), compi led from January 1976 to July 1986. Seven cases were pos t s t apedec tomy PLFs and 29 cases were spontaneously occurring PLFs. F r o m 1986 t h r o u g h 1990, an addi­tional 16 patients were treated for surgically conf i rmed PLF. This r epo r t examines the original 29 and the addi t ional 16 cases of s p o n t a n e o u s l y o c c u r r i n g PLF ( to t a l 45 cases). T h e 1988 r epor t used four pa t ient classifications based on clinical p resenta­t ion: (1) pa t i en t s having ver t igo wi thou t hear ing loss, (2) patients having hearing loss without vertigo, (3) those with a Ménière 's disease "close equivalent," and (4) a miscel­laneous "end-organ" g roup . A fifth g roup c o n s i s t e d of s e v e n p o s t s t a p e d e c t o m y patients with PLF. Al though the symptoms of the fifth g roup of patients are similar to t h o s e of p a t i e n t s in s o m e of t h e o t h e r

groups, they should be considered a distinct g r o u p because the so-called s p o n t a n e o u s PLF occurs in individuals who have never had ear surgery. The author uses this crite­r ion because pat terns recognized th rough this type of analysis may prove helpful in treating patients in the various categories.

T h e cases discussed h e r e suggest the variety of presentations seen and the treat­men t modalities used in this diverse group of patients. Management of PLF is challeng­ing and occasionally requires great patience on the par t of bo th the treating physician and the patient . Both the science and the a r t of m e d i c i n e a re r e q u i r e d in t r ea t ing patients with this condition.

MATERIALS AND METHODS

Forty-one of the 45 patients had a PLF ident i f ied at surgical exp lo ra t ion . In the four cases in which a leak was no t actually seen , abnorma l i t i e s (hype rmob i l e s tapes footplate, excessive filmy adhesions a round the oval window [OW], stapes hypermobili-ty, or an extremely lateralized r o u n d win­dow [RW] membrane) were observed, and the windows in question were grafted with resulting resolution of symptoms. Because clinical history, surgical findings, and opera­tive results suggested that PLF was present in these four, the patients were included in the series. Thirty-four patients (38 ears) are

Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire. Reprint requests: Dr. Weider, Dartmouth-Hitchcock Medical Center, Medical Center Drive,

Lebanon, NH 03756.

Copyright © 1994 by Thieme Medical Publishers, Inc., 381 Park Avenue South, New York, NY 10016. All rights reserved. 11

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SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

from the 1988 study, and 19 (23 ears) are new cases.

Most patients had thorough metabolic, audiological, and neurological evaluations. Neuro log ic tes t ing i n c l u d e d d e h y d r a t i o n test ing for evaluat ion of coch lea r reserve a n d i m p r o v e m e n t in e q u i l i b r i u m . T h e agent most frequently used was intramuscu­lar furosemide (Lasix; 40 to 60 m g ) . Oral f u r o s e m i d e has n o t b e e n effective. T h e a u t h o r has also observed improvemen t in h e a r i n g t h r e sho ld s a n d equ i l i b r ium with oral glycerin, oral urea , and intravenously a d m i n i s t e r e d R e n o g r a f i n (50 cc R e n o -grafin-60). For pat ients who had cons tan t mild instability, neurological testing empha­sized performance of the Quix test and the eyes-closed turning test (ECTT) before, and 1 hou r after, the intramuscular furosemide was g i v e n . T h e a u t h o r c o n s i d e r s a n improved p e r f o r m a n c e on the Quix test3

a n d / o r ECTT4 after the adminis t ra t ion of f u r o s e m i d e (of g lycer in , u r e a , o r R e n o ­graf in) h ighly s igni f icant . P o s t o p e r a t i v e i m p r o v e m e n t was seen in 100% of such p a t i e n t s . All tests we re n o t d o n e for all patients. When adequate historic data and suf­ficient diagnostic information were collect­ed, middle-ear explorat ion was under taken .

At the time of surgery, the pat ient was preinjected with 2.0% lidocaine HC1 (Xylo-caine) with ep inephr ine 1:100,000 while in the T r e n d e l e n b u r g posi t ion to encou rage passive increase in spinal fluid pressure and p e r i l y m p h a t i c f luid leak. G r e a t ca re was taken to opera te on a "bloodless field" so that the surgeon 's first view of the middle ear was no t obscured by blood or irrigation fluids. Careful observation of bo th windows was done immediately. Cure t tement of the scutum was done if necessary. Any anatomic abnormal i t i es (e.g., ossicular deformit ies , stapes hypermobil i ty, abno rma l RW mem­b r a n e posi t ion, a n d a b n o r m a l adhes ions) were n o t e d a n d la ter d i a g r a m m e d . Both windows were observed for a min imum of 5 minutes , unless there was an obvious leak. In t h e case of a slow l eak , t h e f lu id o r reg ion of consistently shifting l ight reflex was d r i e d a n d o b s e r v e d . If a w a k e , t h e p a t i e n t was asked to p e r f o r m a Valsalva m a n e u v e r . Occas iona l ly a c o l l e a g u e was

called to confirm a quest ionable PLF. Since July 1986, near ly all exp lo ra t ions for sus­pected PLF have been videotaped.

When the PLF is identified, the mucosa a r o u n d t h e O W o r RW is a b r a d e d , any adhesions lysed, and the windows covered with fat, p e r i c h o n d r i u m , or loose a reo la r tissue. T h e au tho r has used all tissues suc­cessfully, bu t favors the loose areolar tissue because it conforms well to an irregular sur­face. A cryoprec ip i ta te (au to logous) two-par t glue is used to aid in securing the graft m a t e r i a l . B l o o d for c r y o p r e c i p i t a t e is o b t a i n e d f r o m t h e p a t i e n t severa l days before surgery. When the area in question has been prepared , a pudd le of cryoprecipi­tate is created in a window area and the tis­sue is d i p p e d in bovine t h r o m b i n d i lu ted with calcium chloride 1:1. When the tissue is placed in the window area, the two solu­tions mix to form a "glue" that helps hold the tissue in place. If a leak is observed in one window, both OW and RW are typically covered so tha t r eexp lo ra t i on to repa i r a remaining window, as has h a p p e n e d on two occasions, will no t be necessary. Hear ing is genera l ly n o t significantly affected w h e n b o t h windows are covered. Both windows are also covered if a fistula is strongly sus­pected a l though no t visualized at the time of surgery because n o t all PLFs leak con­stantly. A n u m b e r of pat ients with uncon­f i r m e d P L F h a v e h a d t h e i r s y m p t o m s resolve after this was done . Lastly, Gelfoam is placed over the soft-tissue graft followed by Gelfilm p l aced be tween the tympan ic m e m b r a n e and the incus to prevent adhe­sions from forming.

OTHER PROCEDURES

In cases where revision is necessary, a h a n d - h e l d a r g o n laser is u s e d to lyse all adhesions, thereby creating a relatively dry field. If the stapes is hype rmobi l e or sub-luxed (tilted with the anter ior end elevated above the n o r m a l level a n d the pos te r ior e n d at or slightly below its n o r m a l level), the stapedius t endon is cut.5

In two revision cases, a l u m b a r spine decompression drain was placed at the time 12

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TREATMENT AND MANAGEMENT—WEIDER

of surgery and allowed to remain in place for 5 days to r e d u c e ce r eb rosp ina l fluid (CSF) pressure and prevent the graft from becoming displaced in the immediate post­opera t ive p e r i o d . Both of these pa t i en t s were being treated for fluctuating and dete­r io ra t ing hea r ing in an only-hearing ear. This procedure worked for both cases and stabilization was achieved for an ex tended p e r i o d of t ime . In each of four revision cases, the pa t ien t ' s own b lood was drawn and t hen p laced in the midd le ear in an a t t empt to create a b lood clot that would p r o v i d e a sea l . 6 T h i s was d o n e for two patients by way of an exploratory tympan­otomy and for two patients transtympanical-ly in the office.

An endolymphatic shunt (ELS) may be done if elements of hydrops are present.7,8

When doing an ELS for Meniere 's disease, the middle ear is explored for possible PLF and, if there is any question of its presence, both windows are grafted.

In cases of repeated failure to control ver t igo, vest ibular nerve section was per­formed on two pat ients and streptomycin ablation was done in two cases. Other pro­cedures included labyrinthectomy done in one pa t ien t ' s dead ear believed to be the site of a r e c u r r e n t PLF, a n d r e p a i r of a widely patent cochlear aqueduct in another patient.

RESULTS

T a b l e 1 p r e s e n t s d a t a o n t h e 45 patients in this study divided into the four groups previously ment ioned . Table 2 lists etiologies of PLF in these patients. Tables 3, 4, a n d 5 p r e s e n t fu r ther da ta for several subgroups of patients considered to be of special interest.

VERTIGO WITHOUT HEARING LOSS

T h e r e a re 10 pa t i en t s in this g r o u p (Table 1). These 10 represent 22% of the cases. Five are male a n d five are female, with an age range from 8 to 44 years and an average age of 34 years. Seven of the 10

reported a precipitating event at the onset of vertigo. Three patients who could not date the onset of symptoms to an event could ini t iate ver t iginous feelings or increase their feeling of instability with physical stress (lifting, bending, hard physi­cal work). Nine of the 10 related vertigi­nous episodes directly to physical labor. Events initiating vertigo for the first time included log splitting, scuba diving, lifting, head trauma, vomiting, and skiing at high altitude. Seven felt subjectively better with­in 1 hour of being given dehydra t ing agents (furosemide or glycerin). Reno-grafin was also of value in immediately alle­viating vertigo in one patient. Effectiveness of the test depended on the presence of a feeling of chronic mild instability, fre­quently present in these patients. Despite absence of significant hearing loss, the symptomatic ear is usually, although not always, correctly diagnosed in patients with vertigo. Tinnitus, dullness or pressure, occasional deep-ear pain (usually tran­sient), an abnormal Quix or ECTT, and abnormal summating potentials on electro-cochleography9 all help in identifying the symptomatic ear. In cases of possible bilat­eral PLFs (usually of congenital or traumat­ic etiology), there may be a diagnostic dilemma. After one ear is repaired and par­tial recovery is effected, reevaluation and subsequent explorations of the other ear are done. Of the 10 patients who had verti­go without hearing loss, only 1 had bilater­al PLF. This 30-year-old woman (patient 6, Table 4) experienced mild chronic instabil­ity without nausea since age 14 years. Electronystagmography (ENG) testing re­vealed bilateral hyperactive caloric respons­es, an abnormally increased summating potential in her left ear on electrococh-leography, and a positive response to furosemide. Her symptoms invariably worsened premenstrually. At surgery, she had what seemed to be an obvious OW leak, which was repaired with improvement that was sustained for about 5 weeks. She subsequently had a relapse of her symp­toms. During surgical exploration of the right ear, the presence of PLF was ques­tionable. Repair resulted in temporary 13

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SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

TABLE 1. Related Data for 45 Patients with Perilymphatic Fistula

Total Male Female Left ear Right ear Bilateral ear Age range (yr) Average age (yr)

Etiology Precipi tat ing event Congeni ta l Id iopathic

Symptoms Fluctuat ing hea r ing Imbalance Tinni tus Aural pressure Aural pain Symptoms increased

by physical stress

Neurologic tests Lasix Glyce r in /u rea Renografin Eyes-closed t u r n i n g /

Quix tests

Surgical findings Oval window R o u n d window Both windows

N u m b e r of r ecur rences O n e Two T h r e e Four

Adjunct t r ea tmen t modali t ies

Endolymphat ic shun t Streptomycin Labyrinthectomy Repair of cochlear

aqueduc t

Results Hea r ing improved Hea r ing stable Hea r ing worse Balance improved Balance un improved Tinni tus improved Tinni tus u n c h a n g e d

Vertigo without

Hearing Loss 10

5 5 5 5 1

8-44 34

7 1 2

0 10

2 2 3

9

3 3 3

6

7 0 2

4 2 0 0

4 0 0

0

0 0 0 9 1 1 0

Hearing Loss

without Vertigo

5 5 0 4 2 1

4 -55 25

4 1 0

2 0 0 0 0

2

0 3 0

0

4 0 1

1 0 0 0

0 0 0

0

3 1 1 0 0 0 0

Ménière's Disease Close Equivalent

18 6

12 7

13 3

9-67 41

8 3 7

17 18 11

7 2

10

5 5 0

2

15 0 2

2 0 3 0

8 1 0

0

6 9 3

16 0 9 3

Miscellaneous End Organ

12 6 6 9 7 3

15-58 38

6 2 4

12 12

4 1 1

9

2 3 1

2

12 1 2

0 1 3 1

1 1 1

1

3 8 0

12 0 4 1

benign positional vertigo, which occurred when the patient lay on the operated side, and no relief of preoperative symptoms. Because of the continued increase in sum-mating potential in the left ear, an ELS was done as well as regrafting of the OW on that

side with good relief of symptoms for 6 weeks. The patient's hearing improved to normal, but she later had another relapse. She is currently being reevaluated and a possible diagnosis of vascular loop syn­drome is considered.10

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TREATMENT AND MANAGEMENT—WEIDER

TABLE 2. Etiologic Factors in 45 Patients with Perilymphatic Fistula

Etiology

Precipitat ing event (n = 26) Scuba diving Baro t rauma Nose blowing Vomit ing Bowel movement Physical l i f t ing/straining Head t rauma Childbir th

Congeni tal origin Unknown origin Total

Number of Patients

3 2 3 3 1 7 5 2 7

12 45

Of the remaining nine patients in this group, eight are symptom free and the other patient has near-complete resolution of symptoms. One patient continues to do heavy lifting in the course of his employ­ment as a woodworker and after two PLF repairs and one ELS is able to tolerate occa­sional low-level vertigo and instability with the aid of occasional meclizine. One patient feels less aural pressure if a ventilation tube is in place. This patient had a relapse and subsequently an ELS was done and the orig­inal leak was resealed. Another patient was initially misdiagnosed, but improved dra­matically after an ELS was done for pre­sumed Ménière's disease. She suffered a severe relapse after flying in a nonpressur-ized airplane, but has been totally asympto­matic after subsequent OW and RW repairs. Six other patients experienced complete and lasting relief of their symptoms after a single repair in which leaks, found during middle-ear exploration, were sealed. Four of these were OW leaks, while in two cases (patients 2 and 3, Table 5) there were leaks from both windows.

In three of the four pat ients who required reoperation, relapse was clearly related to physical stress (heavy lifting in two cases and barotrauma in one case). The remaining case of an adult woman (patient 6, Table 4) is still under evaluation. She may have relapsed in response to an increase in CSF pressure, but the relation­ship to stress is not obvious. Three of the four patients relapsed within 4 months of

original repair and one about 12 months after original repair.

It seems increasingly important to rec­ommend emphatically a period of extended near-total bedrest for as long as 6 weeks to permit a good seal of soft tissue to bone in patients who may have a relatively open cochlear aqueduct.8,9 For patients whose jobs require that they do either heavy lifting or experience barometric pressure changes, a change of occupation may be advisable.

Experiences with three patients in this group seem to indicate that endolymphatic sac decompression can be an effective treat­ment in some cases of PLF.1,7,8 It is interest­ing to note that the symptom of tinnitus was mentioned as a major complaint by only two of the patients in this group.

HEARING LOSS WITHOUT VERTIGO

This is the smallest of the four groups, containing only five patients, or 11% of the total (Table 1). All five are male; they range in age from 4 to 55 years, with an average age of 25 years. Four patients in this group could date the onset of symptoms to a par­ticular event, while one patient had a PLF that was almost certainly of a congenital and hereditary nature because both he and his mother had bilateral OW PLF. All patients in this group had OW leaks at sur­gical exploration. The 33-year-old male scuba diver (patient 1, Table 5) had leaks from both windows. Three of the five patients demonstrated hearing improve­ment after administration of glycerin.

Two of the five patients could accentu­ate their hearing loss by physical stress: one by intense physical exertion (U.S. Ski Team member) and another by nose blowing.

TABLE 3. Primary Symptom in Patients with Perilymphatic Fistula of Unknown Origin

Symptom Vertigo or imbalance increased

with physical stress Constant mild instability Daily fluctuation in hear ing Classic Ménière ' s disease

Number of Patients (n = 13)

(29%) 8

1 1 3 15

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SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

TABLE 4. Patients with Bilateral Perilymphatic Fistulas

Patient 1

2*

3*

4†

5†

6

7

8

Age (yr) Sex

15 F

19 M

33 F

67 F

55 F

30 F

39 F

36 M

Etiology Congenital

Congenital

Congenital

Congenital

Congenital

Congenital

Childbirth

Nose blowing

Hearing Bilateral (-)

Bilateral (-)

Bilateral (-)

Bilateral (-)

Bilateral (-)

Bilateral (-)

Bilateral (-)

Left (-) Right dead

Vertigo Yes

N o

N o

Frequent mild imbalance Frequent mild imbalance Chronic mild imbalance Frequent episodes Yes

Finding Bilateral OW

Bilateral OW

Bilateral OW

Bilateral OW

Left OW

Bilateral OW

Bilateral OW

Bilateral OW

(-) = depressed or fluctuating hearing; OW = oval window perilymphatic fistula. *Mother and son.

†Sisters.

After maintaining his preoperative and postoperative level of hearing for 10 years, a 13-year-old male experienced a decline in hearing during a 6-month period while working in a logging camp designed to help teenaged boys with behavior problems. Heavy labor may have contributed to his rapid decline in hearing, which occurred without any symptom of instability. A 19-year-old male (patient 2, Table 4), the pre­viously mentioned patient with bilateral congenital OW PLFs, also exper ienced some bilateral high-tone symmetrical hear­ing decline. His left ear was reexplored with negative findings. Despite continued heavy work, his hearing has remained stable at a somewhat reduced level for 3 years.

All five patients in this group experi­enced arrest of their hearing loss and cessa­tion of fluctuation, with the exception of a patient who resumed strenuous exercise within 2 weeks of surgery despite suggestions to the contrary. Three of the five experi­enced postoperative hearing improvement. The 33-year-old scuba diver (patient 1, Table 5) regained normal hearing within 5 days of surgical repair. This patient may not technically belong in this group because he experienced a brief period of mild vertigo

after his diving accident. After experiencing difficulty in decompression, he felt a sud­den "pop" and noticed decreased hearing in his left ear. When he emerged from the water, he experienced transient mild verti­go, which subsided within 30 minutes , although an obvious hearing loss persisted. He experienced no vertigo during his air flight home. A pronounced low-frequency hearing loss was diagnosed (250 Hz = 50 dB; 500 Hz = 40 dB; 1000 Hz = 30 dB; 2000 Hz = 20 dB; 4000 Hz = 20 dB; 8000 Hz = 10 dB; discrimination = 84%). Surgical explo­ra t ion demons t r a t ed a major RW leak (amber-colored fluid) and a much smaller OW leak. Both windows were repa i red using earlobe fat with immediate and last­ing improvement in hearing, which was confirmed by follow-up 21 months later. He has been advised against further scuba diving.

Close and ongoing follow-up coupled with intense counseling regarding avoid­ance of physical stress might have prevent­ed the precipitous deterioration in hearing observed in the 13-year-old male, a battered child who had one dead ear and one ear with fluctuating hearing when originally diagnosed at age 4 in 1982. A longer post-16

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TREATMENT AND MANAGEMENT—WEIDER

operat ive pe r iod of bedres t also probably would have allowed the 23-year-old compet­itive skier to achieve normal hearing.

It is interest ing to no te that a l though two scuba diving insults (Patients 1 and 2, Table 5) resulted in injury to both OW and RW in the affected ears one patient experi­enced vertigo without hear ing loss and the o ther hear ing loss without significant verti­go . B o t h we re t r e a t e d acu te ly (wi th in a w e e k of o c c u r r e n c e ) a n d b o t h h a v e remained symptom free.

MENIERE'S DISEASE CLOSE EQUIVALENT

This g roup (see Table 1) consisted of 18 patients (40%) whose symptoms at some time dur ing the course of their illness close­ly resembled those of pat ients with classic Men ie re ' s disease. Each had two or m o r e major episodes of vertigo lasting from 1 to 24 hours . Fluctuating hear ing loss, tinnitus, or frequent feelings of aural pressure were

also present in these patients. There were 6 males and 12 females in this group, with an age range of 9 to 67 years and an average age of 41 years.

In 8 of the 18 pa t ien ts , the onse t of symptoms was associated with a precipitat­ing event, while 3 were believed to have PLF of congenital origin and 6 were believed to have PLF of idiopathic origin. Ten of the 18 patients had a history of increased vertigo or imbalance associated with physical stress, which distinguishes t hem from the larger group of patients with classic Meniere 's dis­ease . Wi th few e x c e p t i o n s , m o s t of this g r o u p h a d v e r t i g o o r i m b a l a n c e o n an almost daily basis. Attacks tended to be mild and of short duration. Some patients expe­rienced a low-grade vertigo constantly. Most patients felt better in the morn ing than at any other time of the day. Increased activity tended to intensify their imbalance.

At the time of surgery, OW leaks were f o u n d in 15 p a t i e n t s , a n d in 2 p a t i e n t s (Patients 5 and 7, Table 5) bo th windows

TABLE 5. Patients with Oval and Round Window Involvement in the Same Ear

Patient 1

2

3

4

5†

6*

7§

Age (yr) Sex 33 M 23 F 34 M

58 M 17 F 34 M 27 F

Etiology/ Precipitating

Event Scuba diving frequencies Scuba diving

Barotrauma

Splitting wood

Cheerleading

Congenital patent cochlear aqueduct Nose blowing

Hearing (-) low 30 min only Normal

Normal

(-)

(-)

(-) Daily fluctuation (-) slight with fluctuation

Vertigo Initially

Constant

Constant severe motion intolerance Constant

Constant

None

Mild but constant

Results* H V + 0

± 0

± 0

± 0

+ 0

± 0

± 0

(-) = hearing depressed or fluctuating; ± = hearing stabilized at preoperative level; + = hearing improved to nor­mal; 0 = absence of vertigo. *Results of exploration and surgical repair of oval window PLFs. H = hearing; V = vertigo. †Three revisions were required. The patient had a relapse once after calisthenics and twice after childbirth. The relapses were separated by periods of longer than 1 year. ‡Patency of the cochlear aqueduct was proved by the introduction of a radioactive tracer into the spinal fluid. With a tympanotomy tube placed in the tympanic membrane, the tracer was collected on a cotton ball placed in the ear canal. §Vertigo did not resolve until an extremely lateralized round window membrane (not observed to be leaking) was covered at a second surgery 1 week after the oval window was repaired. 17

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SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

w e r e f o u n d to h a v e l e a k s . O n e of t h e patients with both OW and RW involvement was a 17-year-old f e m a l e w h o s e v e r t i g o began with athlet ic stress ( chee r l ead ing ) , and she suffered a relapse on three differ­e n t occasions with long pe r iods of remis­s i on b e t w e e n r e l a p s e s . E a c h r e l a p s e o c c u r r e d after physical stress (once after ca l i s thenics a n d twice after ch i ldb i r th ; a third pregnancy, with delivery by caesarean s e c t i o n , d i d n o t i n i t i a t e a r e l a p s e ) . Although both windows were involved origi­nally, only the OW was found to be involved on subsequent explorations.

Eight patients in this g roup underwent endolymphat ic sac decompress ion surgery. Two of these did no t seem to have stress-induced vertigo and were suspected of hav­ing classic Meniere ' s disease. Oval window fistulas were r e c o g n i z e d d u r i n g surgica l exploration for PLF, which the au thor now conducts before per forming an ELS in all patients who are considered to have classic M é n i è r e ' s d i sease . In t h r e e of t h e e i g h t patients in whom PLF repair was combined with endolymphatic sac decompression, no t only was vertigo cured but also a dramatic hear ing improvement was achieved.

MISCELLANEOUS END-ORGAN G R O U P

This g r o u p of pa t ien t s (see Tab le 1) differs from the p r e c e d i n g g r o u p only in t h a t n o n e of t h e p a t i e n t s in th is g r o u p repor ted a pro longed (longer than 1 hour) classic attack of vertigo. This g roup consists of 12 patients of whom 3 had bilateral PLFs (pa t ien ts 1, 3, a n d 8, Tab le 4 ) . T h e ages ranged from 15 to 58 years, with an average age of 38 years. The re were six males and six females . Six of t he 12 cou ld da te t he onset of their symptoms to an event involv­i n g p h y s i c a l s t r e s s . T w o p a t i e n t s w e r e t h o u g h t to have PLF of congeni ta l origin and four were thought to have PLF of idio­pathic origin. Nine of the 12 patients in this g r o u p e x p e r i e n c e d increase in vert igo or imbalance coincident with physical stress.

At surgery, OW PLF was found in 12 of the 15 ears explored. Two patients (patients 4 a n d 6, Tab le 5) h a d leaks in b o t h win­

dows, and there was an isolated RW leak in o n e pa t i en t , a 40-year-old female , whose precipitating event was an episode of severe vomiting.

O n e pat ient in this g roup required two revisions, t h r e e r e q u i r e d t h r e e revisions, and one requi red four revisions. Because of difficulty in con t ro l l i ng symptoms, o t h e r p r o c e d u r e s we re u s e d in s o m e of t h e s e c a s e s . O n e p a t i e n t u n d e r w e n t a n ELS surgery, o n e s t reptomycin ablat ion, one a labyrinthectomy, and one a posterior fossa r e p a i r of a p a t e n t c o c h l e a r a q u e d u c t ( p a t i e n t 5, T a b l e 5 ) . In t h e last case , a radioactive tracer, which was placed by lum­bar p u n c t u r e , was ob ta ined by way of the middle ear t h rough an indwelling ventila­tion tube.11,12

All pa t ien ts in this g r o u p are free of i m b a l a n c e a n d have s table h e a r i n g . T h e o n e e x c e p t i o n is t h e p a t i e n t w h o h a d cochlear aqueduct repair. His hear ing con­t inues to fluctuate slightly, bu t to a lesser extent than it did before his repair.

ADDITIONAL COMMENTS

Table 2 lists etiologic factors of PLF for the entire series of 45 patients. Table 3 pre­sents the 13 pat ients (29%) who were no t be l ieved to have c o n g e n i t a l PLF a n d for w h o m a p rec ip i t a t ing event cou ld n o t be de te rmined . Despite the absence of a pre­c ip i ta t ing event , o n c e the symptom com­p l e x b e c a m e e s t a b l i s h e d m o s t of t h e s e pa t i en t s man i fes ted symptoms cons i s t en t with a diagnosis of PLF, such as cons tan t mild unsteadiness and increased instability or hear ing fluctuation coincident with phys­ical stress.

Table 4 depicts the patients who were diagnosed with bilateral PLF. Of this g roup of eight patients, six were t hough t to have evidence of congeni ta l etiology. Pa t ien t 1 first began to have wildly fluctuating hear­ing in he r left ear and vertigo at age 7 years. At age 13, she lost all hear ing in he r left ear coincident with a pro longed per iod of verti­go and imbalance. Oval window PLF repair cured her vertigo bu t no t her hear ing loss. At age 15, h e r r ight-ear hea r ing began to 18

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fluctuate (low-frequency emphasis) , again coincident with vertigo. Three PLF repairs and an ELS surgery have been done. Twice, autologous blood was placed in the middle ear in an a t tempt to stabilize he r hear ing and control imbalance.

Patients 2 and 3 have relatively stable hear ing with a mild high-frequency hearing loss.13 Patients 4 and 5 are sisters who have modera te hear ing loss and good word dis­cr iminat ion ability. Both had bo the r some tinnitus and chronic mild instability. Patient 5 had both symptoms alleviated by simple PLF r epa i r . Pa t i en t 4 c o n t i n u e s to have audi tory ha l luc ina t ions and slight imbal­ance despite PLF repair.

Patient 6 has had mild instability since the age of 14 years. Her case was discussed in the earlier section, Vertigo without Hear­ing Loss.

Patient 7 first experienced hearing loss and vertigo immediately after giving birth, with the r ight ear involvement beg inn ing after the birth of her first child and her left ear symptoms beginning immediately after h e r second chi ld was b o r n 3 years later . This p a t i e n t p robab ly h a d widely p a t e n t cochlear aqueducts.

Pat ient 8 suffered hear ing loss in his on ly -hea r ing ear a n d ver t igo c o i n c i d e n t wi th an u p p e r r e s p i r a t o r y i n f e c t i o n . Symptoms comple te ly resolved with b e d rest . O n e year later , symptoms r e t u r n e d unde r identical circumstances. An OW PLF repair was performed, resulting in hearing i m p r o v e m e n t a n d ces sa t ion of v e r t i g o . W h e n s l eep a p n e a s y n d r o m e was d iag­nosed, he underwent a uvulopalatopharyn-goplas ty a n d c o r r e c t i o n of severe nasal obstruction. Subsequently, a PLF was found in his opposite ear (total hearing loss since b i r th ) a n d two repa i r s were d o n e in the originally involved hear ing ear. Ultimately, streptomycin ablation was used to control symptoms of vertigo. Hearing in the left ear is still excellent. The finding of a PLF in the nonhear ing ear corroborates speculation by Simmons that patients with an only-hearing ear may be at increased risk for developing PLF.14

It has been speculated1 5 that physical stress may trigger elevation in spinal fluid

pressure to the extent that the fibrous mate­rial found in most adult cochlear aqueducts suddenly breaks down, allowing free flow of spinal fluid and causing subsequent rup ture of any o the r sufficiently weak par t of the inner ear (i.e., OW, RW, fissula ante fenes-tram) or other vulnerable places in the otic capsule. Once such an event has occurred, the pa t ien t might remain pe rmanen t ly at increased risk for relapse.

Table 5 describes the six patients who h a d i n v o l v e m e n t of b o t h w i n d o w s . I t appears that RW involvement occurs only when fairly extreme implosive or explosive forces are p rec ip i t a t ing factors, or when there is a preexist ing ana tomic condi t ion that puts the individual at risk, such as a widely patent cochlear aqueduct (patient 6, Tab le 5) or a lateral ly p l aced RW m e m ­brane (patient 7, Table 5).

P a t i e n t s 1 a n d 2 were s cuba d ivers a t t empt ing unsuccessfully to decompres s their middle ears dur ing descent. Sudden ext reme negative middle-ear pressure cou­p led with i n c r e a s e d e x t r a c o r p o r e a l a n d possibly secondar i ly inc reased CSF pres­sure p robab ly caused t ea r ing of the RW membrane .

Patients 3, 4, and 5 experienced fairly e x t r e m e physical stress l e a d i n g to t h e i r injuries. Patient 6 had an extremely lateral RW m e m b r a n e . She was involved in fre­quen t calisthenics before the onset of he r symptoms, bu t was forced to d i scon t inue this activity because of stress-induced exac­erbation of vertigo.

CONCLUSIONS

DIAGNOSIS

A p a t i e n t with PLF may e x p e r i e n c e dizziness, fluctuating or deteriorating hear­ing, aural pressure or fullness, tinnitus, and, occasionally, ear pain. These symptoms can occur alone or in combination. The condi­tion can mimic o the r inner-ear processes such as endolymphatic hydrops, the condi­tion with which it is most commonly com­p a r e d a n d with which , in some cases, it p r o b a b l y c o e x i s t s . S o m e i n v e s t i g a t o r s 19

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SEMINARS IN HEARING—VOLUME 15, NUMBER 1 FEBRUARY 1994

believe that the existence of PLF may lead to secondary endolymphat ic hydrops , a n d others believe that the repair of a PLF may lead to a secondary hydrops . Because this a u t h o r f o u n d O W PLF in t h r e e p a t i e n t s believed to have classic Méniè re ' s disease (see the Results section), patients in whom endolymphat ic sac decompression is being under taken are routinely explored.

PLF can often be traced to a precipitat­ing even t caus ing implosive o r explosive forces on the OW or RW, as is the case in 58% of the patients in this series. A signifi­cant n u m b e r of patients (42% in this series) are unable to identify such an event. Some of the patients have PLF of congenital ori­gin (7 p a t i e n t s o r 15 .5% of this s e r i e s ) , while o the r s have PLF of u n k n o w n cause (13 patients or 29% of this series). Regard­less of et iology, t he symptom of c h r o n i c mild instability that usually worsens with an increase in physical activity (bend ing , lift­ing, etc.) is the most c o m m o n symptom in these patients. When this symptom is dimin­i s h e d o r e l i m i n a t e d by t h e i n t r a v e n o u s a d m i n i s t r a t i o n of f u r o s e m i d e o r R e n o -grafin, or by oral glycerin or oral urea, the diagnosis of PLF is probable . An abnormal Quix test or ECTT may also be reversed by administration of these agents.

TREATMENT

Pa t i en t s wi th severe h e a r i n g loss o r severe vertigo whose symptoms are precipi­tated by a significant implosive or explosive event are probably best t reated by surgical intervention as soon as possible. Hear ing is f r equen t ly r e s t o r e d to n o r m a l levels (as seen in Patients 1 and 7, Table 5). A short per iod of complete bed rest no t to exceed 7 days may also be effective; however, delay­ing surgery may permi t cochlear deteriora­t ion if a PLF seal does n o t occur . In this

a u t h o r ' s e x p e r i e n c e , t h e resul t s of early intervention have been excellent.

Fat, fascia, loose a reo la r t issue, per i ­chondr ium, a n d b lood can all be used effec­tively in PLF repair. This au thor has used all tissue types, bu t favors loose areolar tissue because it conforms well to an irregular sur­face. Auto logous tissue glue (cryoprecipi-tate and fibrinogen) aids in the repair. T h e tissue is packed into place with addi t ional Gelfoam with a layer of Gelfilm between the t y m p a n i c m e m b r a n e a n d t h e i n c u s . Scarification of the area to be grafted is usu­ally d o n e , a l t h o u g h g o o d resul ts may be achieved by covering the area with autolo­gous t issue a n d a l lowing t h e u n d e r l y i n g mucosa to grow together and seal.16 Possibly more impor tan t than the technique used is the insistence on a pro longed recovery peri­od with n o significant lifting, bend ing , or s training for as long as 6 weeks. In nearly eve ry i n s t a n c e of r e c u r r e n t P L F , t h e pa t ients r e p o r t lifting or s t ra ining coinci­d e n t with the re tu rn of symptoms.

Alternative p rocedu re s may be neces­sary in some cases. In addi t ion to otologic t reatments (endolymphatic sac decompres­sion, labyr in thec tomy, coch lea r a q u e d u c t repair, vestibular nerve section), upper-airway m a n a g e m e n t ( submucosa l resec t ion , uvu-lopalatopharyngoplasty) may be requi red to rel ieve severe nasal o b s t r u c t i o n o r s leep apnea . Sleep apnea can lead to decreased oxygen saturation and increased serum car­bon dioxide. This has the potential to cause increased CSF pressure . Addit ional ly, the struggle to brea the dur ing apnea spells and daytime nose blowing can cause implosive and explosive forces on the inner ears. For patients with PLF who become pregnant , a caesa rean sect ion is advisable. Caesa rean was advised to two patients who had under­g o n e r e v i s i o n f i s t u l a s u r g e r y ; n e i t h e r pa t ien t h a d a relapse co inc ident with that delivery.

REFERENCES

1. Weider, D.J., & Johnson , G.D. (1988). Perilymphatic fistula: A New Hampshire experience. American Journal of Otology, 9, 184-196.

2. Balkany, T.J., DeBlanc, G.B., & Weider, D.J. (1976). Reversible sudden deafness and ver­tigo. Eye, Ear, Nose, and Throat Monographs, 55, 148-151. 20

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TREATMENT AND MANAGEMENT—WEIDER

3. Lehrer , J.F., Poole, D.C., & Sigal, B. (1980). Use of the glycerin test in the diagnosis of p o s t - t r a u m a t i c p e r i l y m p h a t i c f i s tu l a s . American Journal of Otolaryngology, 1, 207-210.

4. Singleton, G.T. (1986). Diagnosis and treat­m e n t of p e r i l y m p h fistulas with h e a r i n g loss. Otlaryngology and Head and Neck Surgery, 94, 426-429.

5. Tonkin, J. Personal communicat ion. 6. Causse, J.B. Personal communicat ion. 7. S e l t z e r , S., & M c C a b e , B.F. ( 1 9 8 6 ) .

Pe r i l ymph fistula: T h e Iowa e x p e r i e n c e . Laryngo&ope, 96, 37-49.

8. Lehrer , J.F., Quraishi, A.U., & Poole, D.C. ( 1 9 8 7 ) . T h e r o l e of e n d o l y m p h a t i c sac surgery in the m a n a g e m e n t of secondary e n d o l y m p h a t i c h y d r o p s a s soc ia t ed with perilymphatic fistulas: Preliminary observa­tions. American Journal of Otology, 8, 93-95.

9. Meyerhoff, W.L., & Yellin M.W. (1990) . Summating potent ia l /ac t ion potential ratio in p e r i l y m p h fistula. Otolaryngology and

Head and Neck Surgery, 102, 678-682. 10. McCabe, B.F., & Gantz, B.J. (1989). Va&ular

loop as a cause of incapacitating dizziness. American Journal of Otology, 10, 117-120.

11. F a r r i o r , J . B . , & E n d i c o t t , J . N . ( 1 9 7 1 ) . Congenital mixed deafness: Cerebrospinal fluid otorrhea: Ablation of the aqueduct of the cochlea. Laryngo&ope, 81, 684-699.

12. Wodyka , J . ( 1 9 7 8 ) . S tud ie s o n c o c h l e a r a q u e d u c t p a t e n c y . Annals of Otology, Rhinology, and Laryngology; 87, 22-28.

13. W e i d e r , D.J. , & M u s i e k , F .E . ( 1 9 8 4 ) . Bilateral congenital oval window microfistu¬ lae in a mo the r and son. Laryngo&ope, 94, 1455-1458.

14. S i m m o n s , F .B . , & Pe t rof f , M.A., & Winzelberg, J. (1986). Two emerging peri­l y m p h f is tula " s y n d r o m e s " in c h i l d r e n . Laryngo&ope, 96, 498-501.

15. P o t t e r , C.R., & C o n n e r , G . H . ( 1 9 8 3 ) . Hydrops following perilymph fistula repair. Laryngo&ope, 93, 810-812.

16. Lesinski, S. G. Personal communicat ion.

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