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Transcript of Chapter 142 MÉNIÈRE'S DISEASE AND OTHER PERIPHERAL VESTIBULAR DISORDERS Department Of...
Chapter 142
MÉNIÈRE'S DISEASE AND OTHER PERIPHERAL VESTIBULAR
DISORDERS Department Of Oto-rhino-Laryngology Of
Isfahan Medical Science
peripheral vestibular system :
(1) sensory receptor structures (2) vestibular portion of the eighth cranial nerve
horizontal head turning (angular acceleration) :activity horizontal semicircular canals.
front to back by the superior and side to side by the posterior semicircular canals.
If the loss is simultaneous and symmetric: no significant vertigo, worsened balance,
especially in the dark+ oscillopsia: inefficient vestibulo-ocular and vestibulospinal system.
CLINICAL RELEVANCE :
Vertigo is the illusion of motion
History (1)duration(2) frequency(3) the effect of head
movements (4) specific position that induced (5) associated aural symptoms
(6) concomitant ear disease (e.g., otorrhea, prior surgery, trauma).
Vertigo lasting seconds: BPPV
Vertigo lasting minutes to hours : a. Idiopathic endolymphatic hydrops (Ménière'
disease) b. Secondary endolymphatic hydrops: 1.Oticsyphilis 2.Delayed endolymphatic hydrops 3.Cogan's disease 4.Recurrent vestibulopathy
Vertigo lasting days: vestibular neuronitis
Vertigo of variable duration: a. Inner ear fistula b. Inner ear trauma c. Familial vestibulopathy d. Superior semicircular canal dehiscence syndrome
5.Bilateral vestibular deficit
VERTIGO LASTING MINUTES TO HOURS
Ménière's Disease:
F=M or slightly toward women (1.3:1) 10% of visits to the Dizziness The peak incidence is in 40- 60y Familial occurrence :10%- 20% AD- (HLA) B8/DR3 and Cw7
Ménière's disease characterized by : 1) recurring attacks of vertigo 2) sensorineural hearing loss 3) tinnitus 4) fluctuating fullness pathologic basis distortion of the membranous labyrinth
(hallmark endolymphatic hydrops)
Hydrops: 1) labyrinthitis 2) otitis media 3) HT 4)mumps
5)meningitis.
Endolymphatic hydrops :most inferior (cochlea and saccule )
1)Reissner's membrane distension, 2) contact the wall of cochlear duct, 3)obliterating the scala tympani, 4)Saccular expansion can distort the utricle and semicircular contact the undersurface of the stapes footplate :vertigo by pressure of footplate, also called Hennebert's sign
Imaging studies(CT): 1) hypoplasia endolymphatic sac and duct 2) decreased visualization of the vestibular
aqueduct 3) reduction in periaqueductal pneumatization 4) reduced retrolabyrinthine bone
(MRI) : 1) smaller and shorter endolymph drainage
systems 2) Enhancement of the endolymphatic sac
electron microscopic : shortening of stereocilia
significant to the pathophysiology of Ménière‘:
Ruptures in the membranous labyrinth
leakage of K (eighth cranial nerve,hair cell ) depolarize ,decrease auditory and vestibular neural HL , acute vestibular paralysis in Ménière's attack.
Healing of the membranes with termination of attack and improvement in vestibular and auditory function.
Etiology of Ménière's Disease:
hearing loss, tinnitus, vertigo= Ménière's syndrome. If the cause is unknown=Ménière's disease. If a disease is known= secondary endolymphatic
hydrops Numerous mechanisms of the disease: 1)Obstruction of the endolymphatic duct 2) viral (CMV,HSV1) 3) immunologically 4) ischemia 5) failure of the endolymphatic absorption system
Autoimmune: 1)macrophages and lymphocytes as well as
plasma cells ,IgG, IgA in perisaccular tissues 2)IgG and complement in endolymphatic
fluids. 3)type III immune complex-mediated vascular
injury. 4) Antibodies against elements inner ear in the
serum. 5)type II collagen immunoreactivity
(otosclerosis-like). 6)Antibodies against a bovine 68-kilodalton
(HSP70) 50% bilateral Ménière's disease.
Diagnosis : no test that makes the diagnosis of Ménière's
disease.
Clinical presentation:
vertigo (96.2%) tinnitus (91.1%) ipsilateral HL(87.7%) most commonly 2 to 3 hours. cochlear Ménière's disease (80%),vestibular
Ménière's disease10% _20%develop classic Ménière's disease .
PTA loss of 50 dB, SDS:53%, caloric response reduction :50%.
History and Physical Findings: Vertigo: with any head movement.
N&V,diarrhea,sweating.
Sudden unexplained falls without loss of consciousness..1)otriculosacul change 2) inappropriate postural adjustment the vestibulospinal pathway . ((Tumarkin))
Lermoyez attacks: spell with increased tinnitus and hearing loss, dramatically resolve with onset of vertigo.
Early nystagmus towards affected ear (irritative) Later nystagmus towards healthy ear (paralytic) attack subsides: nystagm towards affected ear (recovery)
HL and tinnitus: SNHL fluctuating and progressive ,low-frequency. peaked" or "tent-like" audiogram. (profoundly deaf 1% to 2%)
Diplacusis (43.6%) recruitment (56%) Nonpulsatile tinitus.
EOG recording of eye movements after caloric. Dehydrating agents: (standard glycerol test 1.5 g/kg) followed by serial PTA over 3 hours.
positive test: 16% improvement SDS or PTA 25 dB at three consecutive frequencies.
Electrocochleography: SP/AP ratio increases.
Evoked and spontaneous otoacoustic emissions abnormal in some Ménière's disease
)
CAP:activity auditory nerve ,sp:sensory haire cell(IHC)
Treatment : no proven cure for Ménière's disease. Current therapy is aimed reduction of symptoms. both medical and surgical: improvement 60%-80%. Medical Therapy:
1) dietary modification 2) diuretics 3)Vasodilators:adenosine,triphosphate,isosorbide,hist
amine,betahistin 4) reduction in immune reactivity : steroids,
immunoglobulin, allergy therapy
Surgical Treatment :
failed medical management classified : procedures spare hearing and
those that do not. vestibular neurectomy intratympanic gentamicin ,streptomycin cryosurgery,ultrasound cochlear dialysis.
Cochlear implantation effective in bilateral severe-to-profound hearing loss.
Hearing-conservative nonvestibular ablative surgery Endolymphatic sac decompression : Complete resolution of vertigo 50%-75%
best results : surgery early disease when symptoms were milder,fluctuating.Improvement of hearing 55%.
Hearing-conservative vestibular ablative surgery Vestibular neurectomy: 1)middle fossa approach : complete resolution (vertigo
95%).(hearing 51%-83%)
2)suboccipital approach: Complete remission vertigo 82%.
3) Both the retrosigmoid and suboccipital approaches: 93% complete response
Intratympanic injection of aminoglycoside : Gentamicin most commonly used.
goal of the procedure: ablate vestibular function in treated ear.
Non-Hearing-Conservative Vestibular Ablative Therapy:
Labyrinthectomy: when serviceable hearing is absent (pure tone average >60 dB, SDS<50%):
ablation with hypertonic saline,as well as transcanal, transmastoid, translabyrinthine cochleovestibular neurectomy.
Bilateral Ménière's Disease: Parenteral Streptomycin until the ice water response was absent
symptoms recur, retreatment with a lesser dose.
Thanks for atteton
Thanks for atteton
Chapter 142
MÉNIÈRE'S DISEASE AND OTHER PERIPHERAL VESTIBULAR DISORDERS
Otologic Syphilis: 6.5% of unexplained SNHL,7% of patients have
Ménière's disease, 30%congenital syphilis and 80% of symptomatic neurosyphilis patients experience hearing loss as a major symptom.
early syphilis: within 2 years of exposure, Vestibular symptoms
are less frequent. meningoneurolabyrinthitis
late otologic syphilis: when symptom after 2 y.
otic capsule involvement:ossicular involvment (CHL), Obliterative endarteritis and mononuclear infiltration,periostitis (endosteal inflammation especially in the semicircular canals)gumm-fibrose of labranrin membran-anal stenosis-hydrops
Interstitial keratitis : 90% late otologic syphilis
Hennebert's sign (nystagmus and vertigo by pressure change on the ear) by gumma on the deformation of labyrantin membran
Tullio phenomenon (nystagmus and vertigo caused by loud noise)
RPR non-treponemal tests most used screening tests for syphilis.
The FTA or MHATP choice for the detection of otologic syphilis, 95% or more late disease.
treatment of otologic syphilis: in the early stages :penicillin 2.4 million u IM+10-day
steroids in late-stage disease: 1.8 million u procaine penicillin
IM daily, Antibiotic(e.g,3 weeks-1 year) + 500 mg probenicid PO qid for 17 to 21 days.
Ceftriaxone: neurosyphilis in HIV-infected patients.
Delayed Endolymphatic Hydrops: vertigo, identical Ménière's disease, in a patient who has
had a previous profound loss of hearing.
cause :unknown structures necessary for endolymph homeostasis!
Diagnosis: pattern of presentation
Treatment: Labyrinthectomy. .in the only hearing ear, conservative, medical management.
Cogan's Syndrome : Ménière's-like hearing loss, vestibular symptoms,
interstitial keratitis as nonreactive tests for syphilis.
typical form: interstitial keratitis+ heart and lungs involvement
atypical form:(10%) : scleritis,episcleritis, papilledema, retinal detachment. Multisystem involvement.
infiltration of the cornea with lymphocytes and plasma cells corneal neovascularization
In cogan disease: HL is bilateral+ progressive without spontaneous improvement, profound.
Cogan's syndrome : autoimmune basis!! IgG ,IgM titer to Chlamydia sp in active Cogan's.
Treatment: Corticosteroids, cyclophosphamide, methotrexate
Recurrent Vestibulopathy : mean age:37y-M=F Vertigo (lasting minutes to hours)
Focal neurologic features are absent.
without audiologic symptoms
Cause : unknown . A viral etiology! The prognosis: recovery is good unilateral reduction in caloric responses in 22% treatment : Symptomatic and supportive
VERTIGO LASTING SECONDS Benign Paroxysmal Positional Vertigo : 20%-40 peripheral vestibular disease. F>M- 40-50y
most common peripheral vestibular disorder. vertical and torsional nystagmus
utricular destruction, damage structures supplied by anterior vestibular artery
posterior canal crista (source dysfunction).
loose otoconia from utricle displaced cupula of posterior canal (cupulolithiasis)
motion of debris within PC (fatigability nystagmus). fixed deposits on cupula (non fatigable).
theory of posterior canal BPPV:
(1) canalithiasis (latency of nystagmus) (2) nystagmus duration (3) vertical (upbeating) and torsional (4) reversal of nystagmus (5) fatigability nystagmus
most common known cause :
1) closed HT 2) 15% vestibular neuronitis 3) infections 4) surgery (stapedectomy) 5) prolonged bed rest 7) Ménière's disease 8) recurrent vestibulopathy
Diagnosis; History:
1) head position 2) suddenly and last seconds 3) lightheadedness
Findings on examination: Dix-Hallpike maneuver
pattern of response :
(1) nystagmus vertical upbeating - rotary (torsional) (2) latency (seconds) (3) Duration (<1 minute) (4) associated symptoms (5) fatigable (6) Symptoms recur
posterior SCC : 1) vertical upbeating - rotary (torsional) nystagmus 2) Canalithiasis
lateral SCC 17% of cases: 1) horizental nystagmus 2) shorter latency 3) less susceptible to fatigue 4) Cupulolithiasis- canalithiasis
superior SCC : 1) low 2) downbeat + torsional nystagmus
Treatment :
1)Early therapy
Left posterior canal
Semont maneuver
Mastoid vibration avoided; 1) retinal detachment 2) high myopia
Treatment maneuver lateral canal BPPV: If geotropic nystagmus: affected ear up for 12 hours
treatment maneuver superior canal BPPV:
Posterior can be converted to lateral canal BPPV during Epley : resolves in several days.
superior and posterior canals: sustained pure torsional nystagmus
Surgical Treatment: singular neurectomy PSCC occlusion
VERTIGO LASTING DAYS TO WEEKS: Vestibular Neuronitis :
second most common vestibular vertigo periphery.
dizziness lasts days
gradual improvemen.
Balance-related complaints may be present for months after resolution.
not change hearing_focal neurologic complaints. caloric response reduction
Pathologic temporal bones: vestibular nerve degeneration (more superior) with
sparing of the peripheral receptor structure
Cause : unknown- viral (HSV, HZV, Borrelia)!!! Nystagmus ;direction to involved ear
Treatment: supportive and symptomatic
methylprednisolone, valacyclovir If SSNHL + vestibular neuronitis-like pattern: as
SSNHL treatment, evaluation for retrocochlear lesion
VERTIGO OF VARIABLE DURATION Inner Ear Fistula : 1) after trauma to the inner ear; barotrauma,
penetrating trauma (rupture limiting membranes labyrinth)
2) Surgery (stapedectomy) most common cause
3) HT 4) congenital malformations (Mondini's deformity)
fistula should be R/O in cases of congenital HL auditory and/or vestibular symptoms PTA threshold and SDS : fluctuate ECOG: larger SP
Fraser test: Improvement PTA or SDS after Trendelenburg
position for 30 minutes
most abnormality : unilateral reduced caloric response in affected ear.
Disequilibrium after increases CSF pressure (nose blowing, lifting = Hennebert's phenomenon
Tullio's phenomenon
positive fistula sign : contralateral slow deviation of eyes + 3-4 ipsilaterally directed beats of nystagmus
free amino acid content of fluid and transferrin, from middle ear
Treatment; (1) bed rest (2) head elevation (3) laxatives (4) surgical exploration: (oval and round windows
patched) ; when HL worsens or vestibular symptoms persist
outcome of surgical :variable. Reduction in vestibular complaints 49% -100%.Hearing improved 24%-49%
TRAUM: Nonpenetrating Trauma Labyrinthine Concussion:
Not otic capsule or intralabyrinthine limiting membranes
auditory (HL : simmilar NIHL 4 kHz or profound) - tinnitus and vestibular complaints
Nystagmus: toward side of lesion acutely ,followed
by contralateral nystagmus. falling in direction of slow phase of nystagmus
reduction caloric response, abnormality of VOR
1)intra-labyrinthine hemorrhage -exudation of fluid into the endolymph and perilymph
2) inflammatory reaction
3) fibrosis and ossification destroys membranous labyrinth
4) vestibular nerve damage progressive HL -persistent vestibular symptoms
Blast Trauma :
waves greater than 200 dB
Perforation TM - ossicular disruption-inner ear damage
inner ear damage most severe when conductive mechanism is not damaged
HL : high frequencies -recovers spontaneously immediate, permanent, profound losses may be
occurred. Pathologic findings: destruction cochlea /HC basilar membrane.
Penetrating Trauma: Violation of the Otic Capsule/Temporal Bone :
inner ear or eighth cranial nerve disrupted
Fracture otic capsule, injury 7-8 nerve : more commonly in transverse fractures
inner ear damaged: subluxation stapes into
vestibule (Acute vertigo and SNHL)
Nystagmus direction of healthy ear fall direction of the slow phase of nystagmus.
Barotrauma:
inner ear consequence of atmospheric pressure
flying, diving in deep water (visual and
proprioceptive less effective)
acute vestibular dysfunction symptom
Alternobaric trauma :
26% of divers and 10% -17% of pilots most cases: vertigo, HL, tinnitus 10-15 minutes
Most: while in ascent to surface eustachian tube patency – URI –ME pressure
increased
nystagmus toward the ear with higher middle ear pressure
Pathogenesis: unknown: pressure differences between middle ear and intracranial/ intralabyrinthine space!!
Atmospheric inner ear barotrauma:
abrupt changes in middle ear pressure : 1) damaging middle ear and inner ear structures 2) round window and/or oval window displaced into
the labyrinth - can rupture into the inner ear
depths from 10 -30 feet during descent HL and tinnitus common -vertigo less common
(30%)
HL is typically of high frequency.(4- 8-kHz)
theory mechanisms: 1) explosive : increased intracranial pressure into
inner ear - rupture round and/or oval windows into the middle ear.
2) implosive: increased middle ear pressure, round window and/or oval window displaced into the labyrinth and can rupture into the inner ear
treatment : 1) Bed rest 2) head elevation 3) close monitoring of hearing and balance 4) progressive HL or failure of resolution of
vestibular symptoms in 3 -5 days: Tympanotomy and MEE
Recompression avoided in barotrauma complete recovery: least 3 m If permanent damage : diving should be cautioned
against
Inner ear decompression sickness and isobaric gas counterdiffusion sickness (IEDS-IEEGCS) :
increased use of mixed gas, oxyhelium, for deep water diving( depths greater than 100m)
vestibular and auditory disfunction : often permanent, particularly if treatment is delayed.
vertigo in 50% IEDS bubbles within the intralabyrinthine fluids Pathologic; intralabyrinthine fibrosis and new bone formation like
ischemic injury Treatment Immediate recompression
Familial Vestibulopathy : Rare- AD sudden attacks of vertigo- oscillopsia - chronic
disequilibrium spells triggered by stress Auditory symptoms : absent Bilateral caloric testing and low shortened time
rotary testing
Acetazolamide : spells of vertigo terminated !!
1) migraine headache + vertigo+ hemiplegic migraine (chromosome 19p-dysfunction ion channel)
efficacy of acetazolamide
2) migraine headaches + vertigo+ essential tremor : triggered by stress, exercise, lack of sleep progressive vestibular deficit : not in later disease
Superior Semicircular Canal Dehiscence Syndrome:
vertigo + oscillopsia by loud noises or change middle ear or intracranial pressure
Tullio phenomenon Hennebert's sign third mobile window" into the inner ear eye movements align with affected superior canal
(Ewald's first law)
positive pressure in EAC ,Valsalva: nystagmus slow phase directed upward with
torsional motion of the superior pole of eye away from affected ear
negative pressure in EAC , Valsalva against a closed
glottis, jugular venous compression: oppositely directed eye movements with slow phase
directed downward with torsional motion of the superior pole of the eye toward the affected ear.
length of dehiscence (≥5 mm) : dysfunction canal when rapid head movements in plane of superior canal.
visual fixation can lead to suppression HRCT (1mm) 0.5-mm-collimated helical CT
VEMP responses: short-latency relaxation potentials
Weber test lateralizes to affected ear
VEMP
BC <0 dB (NHL). ABG can exist even when AC thresholds are NL
ABG : 24 ± 7 dB - lower frequencies (250 to 4000 Hz) evoked VOR demonstrated affected ear Postoperatively : 1)vestibular symptoms 2) AC improve 3) intact VEMP
ABG was unchanged !!!
Treatment: 1) mild symptoms not require treatment 2) tympanostomy tube 3) middle cranial fossa approach
Bilateral Vestibular Hypofunction : Bilateral loss of vestibular function + oscillopsia+
disturbances of gait most commonly induced : 1) AG 2) degenerative diseases of the cerebellum 3) meningitis 4) systemic autoimmune diseases 5) trauma 6) bilateral Ménière's disease 7) 25% unkown
ototoxic exacerbate gentamicin : 1) furosemide, cisplatin, ethacrynic acid,
amphotericin B, cyclosporin 2) Impaired renal function 3) hyperthermia 4) mutation
tests of vestibular function: 1) dynamic visual acuity 2) head thrust test
prolonged disability is not uncommon
Treatment:
1) immediate cessation of toxic agent if possible 2) vestibular rehabilitation
Dr karim
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