MENIER’S SYNDROME = ENDOLYMPHATIC HYDROPS
1861 PROSPER MENIER WROTE 4 PAPERS ON SUBJECT (1ST TO LINK SYMPTOMS TO AN INNER EAR ETIOLOGY)
DIVIDED INTO
1) MENIER’S Dz = IDIOPATHIC HYDROPS
2) SECONDARY(CAUSED) ENDOLYMPHATIC HYDROPS
DEGENERATIVE,INFLAMMATORY(AUTOIMMUNE–ALLERGY–COGAN’S), INFECTIONS(VIRAL, SYPHILIS,CHRONIC OM), PLF, METABOLIC(HYPOTHYROID),?MIGRAINE/ALLERGY, SYMPATHETIC (COMPENSTATION FROM CONTRA PATH), TRAUMA (USUALLY DELAYED)
DIFF DX: PLF, MS, MIGRIANE, VESTIBULAR SCHWANNOMA, ISCHEMIA—–ALL EXPERTS AGREE THE HISTORY IS REALLY THE KEY TO MAKING THE DIAGNOSIS OVER ALL THE ANCILLARY TESTS
MENIER’S Dz CAN FURTHER BE DIVIDED INTO ISOLATED VESTIBULAR HYDROPS (VESTIBULAR MENIER’S) AND ISOLATED COCHLEAR HYDROPS (COCHLEAR MENIER’S)—-PROBABLY BY THE ONE-WAY VALVE OF BAST (DIVIDES THE UTRICULAR DUCT FROM THE SACCULAR DUCT)—DIVIDES THE LATER DEVELPING PARS INFERIORIS (COCHLEA AND SACCULE) FORM THE PARS SUP.
LONGITUDINAL THEORY OF ENDOLYMPH FLOW—MADE FORM THE DARK CELLS AND THE STRIA VASCULARIS—–ENDOLYMPHATIC DUCT AND SAC PRIMARILY RESPONSIBLE FOR RESORBTION
SOME EVIDENCE TO SUGGEST THAT ENDOLYMPHATIC HYDROPS IS NOT THE CAUSE BUT ANOTHER MANIFESTATION OF THE SYNDROME—-SPIRAL LIGAMENT (REGULATES FLUID AND ION BALANCE) MAY BE PARTIALLY REPONSIBLE—-
SCHUETNECK’S THEORY OF INTERMITTENT RUPTURE AND REPAIR IS MOST DOGMATIC—K+ TOXIC ENDOLYMPH ESCAPES INTO PERILYPH AND CAUSES ATTACK—THE ELS (SEAT OF THE IMMUNE SYSTEM IN THE INNER EAR) IS ALSO A SECRETORY ORGAN AND VARIOUS ABNL CAN LEAD TO HYDROPS—SOME DATA SUGGESTING MENEIR’S TO BE ON ONE END OF THE SPECTRUM OF AUTOIMMUNE INNER EAR Dz
NOT MUCH PROGRESS MADE IN THE PAST CENTURY AND EVERY THERAPY WORKS 2/3 OF THE TIME
PTS WITH MENEIR’S HAVE ENDOLYMPHATIC HYDROPS BUT NOT ALL PTS WITH HYDROPS HAVE MENEIR’S Dz
SYNDROME: EPISODIC VERTIGO—10-MIN – 24 HRS (30 MIN – 3 HRS = MEAN), IS PRIMARY COMPLAINT 60% OF TIME, CAN HAVE FALSE + PLF TEST(HENNEBERTS SIGN)–30% DUE TO DISTENTION AND FIBROSIS OF THE SACCULE TO THE UNDERSURFACE OF THE STAPES FOOTPLATE(THUS NEG PRESSURE IS MORE OUTSTANDING), HEADSHAKE +—UNILAT VESTIBULAR HYPOFUNCTION, FALSE + TULLIOS PHENOM(VERTIGO WITH AUDITORY STIM), LERMOYEZ’S SYNDROME(RESTORATION OF HEARING AFTER VERTIGO), CRISIS OF TUMARKIN (UTICULAR CRISIS-DROP ATTACKS)—POSSIBLY MICRORUPTURE LEADS TO SUDDEN STIM OF THE OTOLITHIC ORGANS AND LOSS OF LEG TONE WITHOU LOC, INCREASED SP/AP RATIO ON ECOG, POSITIVE GLYCEROL TEST——-EVENTUALLY CHRONIC DYSEQUILIBRIUM CAN MANIFEST AS THE “BURNED OUT/APOPLECTIC”  PHASE OF MENEIRS—-WILL OFTEN HAVE POSITIONAL DZ AS WELL AND BE CHRONICALLY DEBILITATED—-DO NOT BE MISLEAD
FLUCTUATING SNHL—-PROGRESSIVE, PRIMARILY LOW FREQUENCY, PEAKS AT 2000 Hz, SDS INITIALLY WELL PRESERVED, NO TONE DECAY, TYPE II BEKESY SCORE, INCREASED SISI SCORE, DIPLAUCUSIS BINAURIS DYSHARMONICA(HIGHER/ROUGHER PITCH HEARD IN AFFECTED EAR BEFORE OR AFTER AN ATTACK USING A 512 HZ TUNING FORK TO TEST AC IN EACH EAR)—-OVER TIME FLAT SNHL DEVELOPES AND SDS DROPS
TINNITUS—-ROARING MID PITCHED, MORE LIKE A NOISE (WATERFALL, ELECTRIC TOWERE ), OFTEN FLUCTUATING WITH HEARING LOSS–OFTEN PARALLELS THE TONE OF THE HL—-THUS THE “BOUNDRY” THEORY BETWEEN ACTIVE AND INACTIVE SEGMENTS OF THE COCHLEA MIGHT BE THE MECHANISM
AURAL FULLNESS/PRESSURE—-IPSILAT, FLUCTUATING—OFTEN THE COMMON DENOMINATOR—-PROBABLY RELATED TO SENSORY INNERVATION OF THE DURA SURROUNDING THE ELS—-POSSIBLY THE MIDDLE EAR (RW) AFFERENTS
ATTACKS–AVG 8/YEAR FOR THE FIRST 20 THEN DROPS OFF TO 5/YR
20-40% BILAT—-TENDS TO INCREASE WITH TIME(DO NOT BE TO ANXIOUS TO DO A DESTRUCTIVE PROCEDURE)–15 YEARS=40% B, ONE STUDY SHOWED 80% B AT 50 YEARS?  NEVER EVEN CONSIDER AN ABLATIVE PROCEDURE UNLESS PT SUFFERS DEBILITATING VERTIGO FREQUENTLY
AGE 30-50
SLIGHT FEMALE PREPONDERANCE (USED TO BE MALE)
10-15% + FH
RARE IN CHILDREN (3% IN CHILDREN > 5 YRS OLD)
AFFECT OF PREGNANCY—PROBABLY AN INCREASED RISK WITH THE BODIES FLUID STATUS CHANGES
60% COMPLETE REMISSION
EVEN 57% OF “INTRACTIBLE” VERTIGO RESOLVE WITH TIME
HIGH HSV IgG TITERS HAVE BEEN IDENTIFIED—-?TRIAL OF VALTREX?
Dx: Hx, PE, AUDIO, ROUTINE SNHL LABS, GLYCEROL TEST (CAN ONLY DO IF PT HAS SIG SNHL!): 1.2 ML OF 95% SOL/KG—AUDIO 1, 2, AND 3 HOURS POST INGESTION—Sx IMPROVE IN 1 HOUR WITH MAX IMPROVEMENT IN 2-3 HOURS (>60% HYDROPIC PTS IMPROVE > 10 db PURE TONES BETWEEN 250-1000 DB AND 12 – 16 % IN SDS), ECOG DEMONSTRATES ENHANCEMENT OF THE SP/AP RATIO GREATER THAN O.33- O.4, ALSO AN INCREASED AP DIFFERENCE (95% SENSITIVE FOR EARLY MENIERS), ENG MAY BE CLOSE TO NL BETWEEN ATTACKS
ECOG—STIMULUS IS AUDITORY CLICKS—SENSE ELECTRIC POTENTIALS—GET DETECTION  ELECTODE AS CLOSE TO RW AS POSSIBLE (THROUGH THE TM), AP FORM THE TYPE I OCH, SP FROM THE BASILAR MEMBRANE DEFLECTING INTO THE SCALA TYMPANI
ECOG CAN BE USED INTRAOP FOR MONITORING, PRE OP FOR COCHLEAR IMPLANT!
Rx:   SODIUM RESTRICTION (<1.5 G/DAY—THE SINGLE MOST EFFECTIVE THERAPY)—-VERY DIFFICULT TO ACHEIVE—SEND TO A DIETICIAN, DIURETIC THERAPY MAXIDE OR DYAZIDE (HCTZ AND TRIAMPTERENE) 1 PO Q D—MAY NEED TO WATCH K+, DIAMOX (CA INHIBITORS) 250 MG QD–ADVANCE TO 250 MG QID–NEPTAZINE 50-150 MG Q D—START SLOW–CAUSES (LIP) NUMBNESS AND (FINGER) PARESTHESIAS-TINGLING–IS K+ SPARRING–CROSS ALLERGY POTENTIAL WITH SULFA DRUGS–CAUSES A MET ACIDOSIS (AVOID IN COPD PTS)—ALSO MAY LEAD TO FORMATION OF KIDNEY STONES IN 2 % OF PTS WHO USE FOR 3-6 MONTHS–CHECK RENAL FUNCTION AND US KIDNEYS
VITAMINS AND STEROIDS—NOT PROVEN BUT WORTH A TRY (GINKO-BILOBA/GINSENG ECT…..)
CAFFEINE, NICOTINE, MSG, SUGAR, STRESS, AND ETOH RESTRICTION
VESTIBULAR REHAB—ALWAYS A GOOD IDEA
VESTIBULAR SUPPRESSANTS—COMBATS YOUR COMPENSATION AND REHAB
TRY SL ATIVAN TO ABORT ATTACK!!!(MUST BE BRAND NAME)
SCOPOLAMINE (BANNED FOR PSYCOSIS), DRAMAMINE (DIMENHYDRINATE), MECLIZINE (ANTIVERT) 25 MG PO Q 6 HOURS PRN (ANTIHISTAMINE), CINNARIZINE (CA++ CHANNEL AND H1 HISTAMINE BLOCKER–NOT AVAILABLE IN US), VASODILATORS–HISTAMINE (ANALOGUE) INJ S.Q. OR I.V., BENZOS: GABA MODULATORS–VALIUM 5 MG PO TID/ 1-5 MG IV Q 1 HOUR PRN, CLONAZEPAM 0.25-0.5 MG PO BID-QID
IMMUNOTHERAPY—-STEROIDS—-TAUTED BY DEREBERRY TO BE OF BENEFIT IN MANY PTS—-AT LEAST ASK YOUR PTS ABOUT OTHER ALLERGIC SYMPTOMS
INTRATYMPANIC DEXAMETHASONE (RECENTLY SHOWN TO BE NO BETTER THAN PLACEBO (SILVERSTEIN)
VALTREX  1 G PO TID—FOR POSSIBLE HSV LINK
INOVAR THERAPY (FENTANYL & DROPERIDOL)—-GATES—VERY SUSPECT
ELS SHUNTING TO MASTOID, ELS EXCISION (D BRADLEY WELLING), ELS AND SIGMOID SINUS DECOMPRESSION—A BIT SPOOKY BUT AT LEAST IT IS NON-ABATIVE—ALSO SOME DATA SHOWS IT TO BE BETTER FOR THE LONG TERM HEARING THAN VESTIBULAR NERVE SECTION AS YOU MAY ACTUALLY BE TREATING THE DZ INSTEAD OF TRYING TO MASK IT
SURGERY ORIGINALLY INVENTED BY PORTMAN WHO LIKENED THE DZ TO GLAUCOMA
ABLATIVE THERAPIES—-LAST RESORT (TO USE ONLY IN DEBILITATING Dz) AS DZ MAY TAKE THE HEARING IN BOTH EARS EVENTUALL AND YOU DO NOT WANT TO HELP IT ALONG
INTRATYMPANIC GENT—GOOD FOR PTS WITH UNILAT DZ, VERY DEBILITATING—NOT CHRONICALLY DIZZY—SEEMS TO ENTER INNER EAR BY THE RW OR ANNULAR LIG.  AND DAMAGES THE DARK CELLS OF THE CRISTAE AND SEMICIRCULAR CANALS— ALSO DAMAGES TYPE I VESTIBULAR HAIR CELLS AND STRIA VASCULARIS (MAY CONTROL HYDROPS AND ACTUALLY IMPROVE HEARING IN SOME PEOPLE.)—MAY ACTUALLY DRILL OUT THE RW NICHE TO FACILITATE THIS Rx—PUT IN SPECIAL PET
COUNSEL PTS—ONLY CONSIDER THIS TREATMENT IN PTS IN WHOM YOU ARE CONSIDERING A NERVE SECTION OR LABYRINTHECTOMY-
PHENOL BLOCK—MYRINGOTOMY—-INFUSION OF BODY TEMP STOCK SOLUTION (40MG/ML)—WARN PTS (PH 5.4)—HAVE LAY WITH EAR UP FOR 30 MIN (AVOID SWALLOWING)—-GO HOME—WARN PTS ABOUT VERTIGO IN 2-4 DAYS—-WAIT A MONTH BEFOR REPEATING
MONITOR WITH  SERIAL AUDIOGRAMS
SCHUKNECHT–INTRATYMPANIC STREPTOMYCIN (ASSOC SNHL)
SYSTEMIC STREPTOMYCIN 2 G IM Q D X 2 WEEKS—DEV. OSCILOPSIA
VESTIBULAR NERVE SECTION—MAY DO ENDOSCOPICALLY—NO NEED TO RETRACT THE CEREBELLUM–BETTER VISUALIZATION—MIGHT DO WORSE WITH HEARING IN THE LONG TERM THAN ELS AS YOU ARE NOT TREATING THE DZ BUT ONLY MASKING IT—-MAY CONSIDER INTRA-TYMPANIC GENT IN THESE PTS AS WELL
COCHLEOSACCULOTOMY—ELDERLY PT WITH NO SEVICABLE HEARING)
CODY-TACK OPERATION (SACCULOTOMY–20% PROFOUND SNHL)
LABYRINTHECTOMY (THE GOLD STANDARD)
ULTRASONIC LABYRINTHECTOMY UNDER LOCAL(SWEDEN)