OTOSCLEROSIS–PROBABLY BETTER CALLED “OTOSPONGIOSIS AN OSSEOUS DYSCRASIA OF THE (MESENCHYMALLY DERIVED/ENDOCHONDRAL OSSIFICATION)OTIC CAPSULE (ONLY FOUND IN THE HUMAN —ONLY FOUND IN THE T-BONE)—MEMBRANOUS LABYRINTH=ECTODERMALLY DERIVED
AUTO D—LOCALIZED TO CHROMOSOME 15
50-70% +FH(AUTO D WITH 25-40% PENETRANCE)?AUTO r—20% RISK OF VERTICLE TRANSMISSION IF 1 PARENT AFFECTED
PRIMARY CAUSE OF CHL IN PTS AGE 15-50(11-30)/30-39
1% OF CHL IN WHITE POPULATION—REPORTEDLY HIGHEST PREVELENCE IN THE EAST INDIAN POP.
CAUCASION > OTHER
FEMALE 2:1
EXACERBATED BY PREGNANCY—–OFTEN COINCIDES WITH THE ONSET OF SUBJECTIVE HL—25% RISK OF INCREASED HL WITH EACH PREGNANCY
75-90% B
EPIDEMIOLOGICALLY LINKED TO HIGH TITERS OF IgG AGAINST MEASELS (PARAMYXO) VIRUS—PCR DATA CONFIRMS
CLASSIFIED AS ACTIVE(SPONGIOTIC)/EARLY OR INACTIVE(SCLEROTIC)/LATE
Sx: GRADUAL NON FLUCTUANT CHL
75% LOW PITCHED ROARING TINNITUS–DUE TO COCHLEAR OTOSCLEROSIS
25% VESTIBULAR Sx–VERTIGO, POSTURAL INSTABILITY—-DUE TO ACTUAL DEGENERATION OF SCARPAS GANGLION
MUST BE SURE Sx ARE NOT FLUCTUANT—R/O HYDROPS!
DIFF Dx = OSTEOGENESIS IMPERFECTA TARDA (VAN DER HOEVE-DE KLEYN SYNDROME) AND PAGET’S Dz (OSTEITIS DEFORMANS) AND TYMPANOSCLEROSIS, OME, CONG FOOT PLATE FIXATION (EARLIER, MALE>FEMALE, NON-PROGRESSIVE, NO EMBRYONIC ANNULUS, HIGH INCIDENCE OF PATENT COCHLEAR AQUEDUCT–PERILYMPH GUSHER, OSSICULAR FIXATION OR DISCONTINUITY, CONG. CHOLESTEATOMA, CONG FOOTPLATE FIXATION, ABNL PERILYMPH PRESSURE—PUSHING OUT ON THE FOOTPLATE
MAY WANT TO GET A CT SCAN IN A NON CLASICAL CASE TO R/O PATENT COCHLEAR AQUEDUCT
SURGERY NOT TOO SUCCESSFUL IN PAGETS
Dx:STROMELYSIN mRNA=GENETIC MARKER
“HALO” OR “RING SIGN” AROUND THE OTIC CAPSULE ON HIGH DENSITY CT SCAN
As TYMPANOGRAM—-IF CHL > 50 Db AND Ad TYMP CONSIDER OSSICULAR DISCONTINUITY
PARACUSIS VON WILLISI—IMPROVED HEARING WITH CONSTANT BACK GROUND NOISE—-”LADY AND HER DRUMMER”–SEEN WITH ANY CHL (20-80%)
HISTOLOGY–10% OF CAUCASIONS HAVE HISTOLOGIC EVIDENCE OF OTOSCLEROSIS
10-12% OF THESE—-1% OF CAUCASIONS HAVE CLINICAL EVIDENCE OF Dz
PRIMARILY ON ANT/INF BORDER OF OW-(SAME LOCAL OF PLF)–FISSULA ANT FENESTRUM(90%)–(VS FOSSULA POST FENESTRUM)—-APPEARS BLUE(BASOPHILIC) ON H&E STRAIN— “BLUE MANTLE OF MANASSE”
“SCHWARTZE’S SIGN”-1873—RED HUGHE OF HYPERVASCULARITY OVER THE PROMONTORY NEAR THE ANT OW (10% INCIDENCE)–PROBABLY REPRESENTS EARLY/ACTIVE/SPONGIOTIC Dz–SOME SAY TO Rx WITH FLOURIDE INSTEAD OF OPERATING
30% RW INVOLVEMENT>COCHLEA OR LABYRINTH>FOOT PLATE ALONE, 1% (BISQUIT FOOT PLATE)—-MUST ALWAYS CHECK A RW REFLEX FOLLOWING STAPES SURGERY!!
POSSIBLE MALLEUS FIXATION (1.6%)
CARHART’S NOTCH = A DEPRESSION IN THE BONE CONDUCTION THRESHOLD GREATEST FROM 1000-2000 Hz—–AN ARTIFACT OF ANKYLOSIS—REALLY A 15-20dB DROP AT 2000Hz—–COCHLEAR RESERVE = A FALSE SNHL GREATEST AT 2000 Hz—MUST DIFF FROM COCHLEAR OTOSCLEROSIS—–CARHART EFFECT = NOTCH IMPROVEMENT AFTER SURGERY
YOU GET A REVERSE CARHART PHENOM WITH I-S JOINT EROSION—THE SENSORY HEARING ELEVATES TO THE BONE COMPONENT AT ABOUT 2000 HZ
CHL WITH EXCELLENT SDS–USED TO SAY 2 NEG FORKS TO OPERATE, NL TM, NL MOBILITY
USUALLY CHL BEGINS IN LOW FREQ
GENERALLY NO RECRUITMENT
ON/OFF EFFECT = ABNL DECREASE IN IMPEDENCE(COMPLIANCE=PV CURVE) OF STAPEDIAL REFLEX NOTED AT ONSET AND OFFSET OF SIGNAL DUE TO EARLY FOOTPLATE FIXATION=PATHOGNEUMONIC
DEPRESSED RESPONSE TO CALORICS ON ENG
COCHLEAR OTOSCLEROSIS
‘67 SHAMBAUGH—-NOT UNCOMMON, AUDIO WITH FLAT OR COOKIE BITE CURVE SNHL WITH EXCELLENT DISCRIM—-PROBABLY DUE TO LOCAL AVM’S—-ISCHEMIA DUE TO ABNL BONE METABOLISM—-SEEM TO LOOSE HEARING IN
FREQUENCIES THAT ARE JUXTAPOSED TOAFFECTED REGIONS OF THE MODIOLUS–?CAUSES SUBADJACENT RESORBTION AND ATROPHY OF THE SPIRAL LIGAMENT AND STRIA VASCULARIS—-DOES NOT SEEM TO AFFECT THE HAIR CELLS OR SPIRAL GANGLION IN ANY WAY
OTOSCLEROTIC INNER EAR DISEASE (OIED) (3%)—INNER EAR CHL—McCABE
NL SDS WITH EPISODIC VERTIGO AND CHL
Rx:
OBSERVATION AND AMPLIFICATION
STAPEDECTOMY—-STAPEDOTOMY
FOR UNILAT Dz TRY TO MATCH THE OTHER EAR
PTS MUST HAVE B COCHLEAR RESERVE TO EVEN CONSIDER BREACHING THE INNER EAR
B Dz—THE HAPPIEST PTS WHEN YOU DO THE FIRST EAR
IF B WAIT MIN 1 YEAR BETWEEN SIDES
DO NOT OPERATE ON 2ND EAR IF PT HAS VESTIBULAR Sx
MAY HELP IF YOU CORRECT UP TO THE “SPEECH BANNANA”
STAPES MOBILIZATION (ROSEN)
CRUROTOMY
HORIZONTAL SCC FENESTRATION(LEMPERT)
SODIUM FLORIDE—20-120MG P.O. Q D—-PRIMARY S.A. = GASTRIC DISTRESS—75%
3% ARTHRALGIAS/ FLOUROSIS—BRITTLE BONE SYNDROME—RARE RENAL PROBLEMS(UROLITHIASIS)
CALCIUM CARBONATE 10 MG P.O. Q D OR (FLORICAL)
VIT D 200 MCG P.O. Q D
MONOCAL–BETTER TOLERATED—TID FOR UP TO 2 YEARS
ETIDRONATE(A BIPHOSPHONATE USED—-USED MORE FOR PAGETS)
BIOFLAVINOIDS
LOOK FOR DISAPPEARANCE OF SCHWARTZE’S SIGN, STABILITY(50%)OR IMPROVED(30%) AUDIO (20% STILL WORSEN)
IMPROVED CT APPEARANCE OF OTIC CAPSULE (CHANGE OF OTOSPONGIOTIC LESIONS TO OTOSCLEROTIC LESIONS)
SEE EAR SURGERY FILE FOR STEPS