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.

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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

Don't makean effortbecausethe best things happen,when youleast expect them