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ent.meniere-disease.core.v1

Ménière's disease (recurrent audiovestibular hydrops)

general_internal_medicineacutesubacutechronicadultgeriatricacuteoutpatientinpatient

NEUROTOLOGY-framed engine — two inseparable jobs: positively diagnose definite-vs-probable Ménière against the Bárány/AAO-HNS 2015/2020 criteria anchored on the AUDIOGRAM (the objective pivot), run the stepped management ladder (lifestyle/low-salt+diuretic/betahistine → intratympanic corticosteroid → ablative intratympanic gentamicin → endolymphatic-sac surgery/vestibular-neurectomy/labyrinthectomy) with the Tumarkin drop-attack safety issue and vestibular rehab; AND distinguish vestibular migraine (commonest mimic, often comorbid), BPPV, vestibular neuritis, posterior-circulation TIA/stroke, SSNHL, and autoimmune inner-ear disease. This engine is the RECIPROCAL routing target referenced by ent.bppv.core.v1, ent.vestibular-neuritis.core.v1, and ent.sudden-sensorineural-hearing-loss.core.v1 (all already wire to ent.meniere-disease.core.v1). Definitive stroke/TIA, BPPV repositioning, vestibular neuritis, vestibular migraine, SSNHL, and the geriatric falls programme are recognised then routed OUT by engine_id (neuro.posterior-circulation-stroke.core.v1, ent.bppv.core.v1, ent.vestibular-neuritis.core.v1, neuro.vestibular-migraine.core.v1, ent.sudden-sensorineural-hearing-loss.core.v1, geriatrics.falls.core.v1) — not re-authored here. RxCUI status (RxNav-verified live 2026-05-17, ingredient TTY confirmed): prochlorperazine 8704, dimenhydrinate 3444, meclizine 6676, ondansetron 26225, diazepam 3322 are the strictly-short-course ACUTE-ATTACK symptomatic axis (AAO-HNS CPG 2020 — suppressants are attack-only, NOT maintenance). Maintenance diuretics: hydrochlorothiazide 5487, triamterene 10763, acetazolamide 167 (all verified live). Betahistine has a stable RxNorm INGREDIENT CUI (1511) but NO US NDCs (confirmed live via RxNav /ndcs — not US-FDA marketed); per the omit-rather-than-fabricate rule the rxcui is INTENTIONALLY OMITTED and betahistine is encoded as a region-dependent non_pharm ladder entry, with the BEMED-negative caveat explicit. Intratympanic dexamethasone, intratympanic gentamicin, endolymphatic-sac surgery, vestibular neurectomy, labyrinthectomy, and vestibular rehabilitation are PROCEDURES (non_pharm) — the definitive escalation steps are procedures, not dispensed drugs; rxcui correctly omitted for all. Bayesian linkage (pre-test Ménière-vs-VM-vs-BPPV-vs-neuritis-vs-TIA prior by attack DURATION + auditory features + spontaneity/triggers + vascular risk; the documented fluctuating low/mid-frequency SNHL on audiogram as the dominant conditionally-independent objective anchor; LRs for the diagnostic-tetrad components; conditional dependence — auditory-symptom LR is conditioned on audiometric documentation, not history alone; decision thresholds for ABLATIVE therapy as an explicit hearing-trade-off; bidirectional cross-engine routing edges by engine_id with ent.bppv.core.v1, ent.vestibular-neuritis.core.v1, ent.sudden-sensorineural-hearing-loss.core.v1, neuro.vestibular-migraine.core.v1, neuro.posterior-circulation-stroke.core.v1) is documented in the co-located _design-brief.md + _research-bundle.md; first-class TS LR fields remain schema-blocked (same constraint as ent.bppv.core.v1 / ent.vestibular-neuritis.core.v1 / derm.cellulitis.core.v1). Effect sizes (≥5): BEMED betahistine vs placebo attack-rate ratios 1.036 (95% CI 0.942-1.140) low-dose and 1.012 (0.919-1.114) high-dose, no difference P=0.759, with overall monthly attack rate falling 0.758 across all arms (placebo response) (Adrion/Strupp BMJ 2016, PMID 26797774); IT methylprednisolone vs gentamicin vertigo reduction 90% vs 87%, mean difference -0.9 (95% CI -3.4 to 1.6) — non-inferior without ablation/hearing risk (Patel Lancet 2016, PMID 27865535); IT corticosteroid vs placebo improvement RR 1.00 (95% CI 0.92-1.10) at 6-12 mo with proportion-of-vertigo-days -0.05 (95% CI -0.07 to -0.02), 10 RCTs/952 pts, low certainty + large placebo response (Webster Cochrane 2023, PMID 36847608); IT gentamicin improvement RR up to 33.0 (95% CI 2.15-507) but VERY-LOW certainty with unquantified hearing harm (Webster Cochrane 2023, PMID 36847592); vestibular neurotomy abolished vertigo in 90.5% and Tumarkin drop attacks in 100% at mean 12.4 y with hearing preserved/improved in 84.4% (Véleine J Neurosurg 2022, PMID 34996039); natural history — bilaterality up to ~35% within 10 y and ~47% within 20 y, hearing loss ~50-60 dB and vestibular decrement ~35-50% mainly in the first 5-10 y (Huppert/Strupp/Brandt Acta Otolaryngol 2010, PMID 20001444). Clinical-uncertainty notes: (1) The entire MD pharmacologic ladder rests on LOW / VERY-LOW certainty evidence (Cochrane 2023 series — Webster CD015171/CD015245/CD015246) with a consistently LARGE PLACEBO RESPONSE; betahistine is BEMED-negative; diuretics/dietary salt restriction are guideline-endorsed but low-quality — encoded as low-harm first tier with explicit expectation-setting, NOT as proven disease modification. (2) The ablative IT-gentamicin step is a genuine HEARING-vs-VERTIGO trade-off — Patel Lancet 2016 shows IT steroid is non-inferior without ablation, so gentamicin is gated behind a hearing guardrail and not first-line escalation. (3) Bárány definite-MD REQUIRES audiometric documentation of low/mid SNHL — probable MD (no documented SNHL) is a distinct, lower-certainty category and overlaps heavily with vestibular migraine, the commonest mimic and frequent comorbidity. (4) ICD-10 H81.01-09 (Ménière, with laterality), H81.39x (other peripheral vertigo), H83.2X9 (labyrinthine dysfunction), H93.13 (tinnitus), R42 (dizziness), H81.4 (central vertigo), G45.0 (vertebrobasilar TIA), H91.20 (SSNHL) are standard ICD-10-CM; per memory the terminology pipeline is ⚠ partial — codes are canonical but flagged for scripts/terminology/ revalidation. (5) Special populations encoded: bilateral MD (hearing-preservation, avoid bilateral ablation), elderly/fall-risk (Tumarkin → falls programme), pregnancy (avoid routine diuretic/acetazolamide; low-salt + non-pharm ± IT steroid), delayed endolymphatic hydrops post-SSNHL (managed on this ladder), autoimmune inner-ear overlap (steroid-responsive, separate immunologic work-up), and driving/occupational safety counselling (fitness-to-drive reporting per jurisdiction).

Entry points (5)

  • symptom
    Recurrent SPONTANEOUS episodic vertigo, each spell lasting 20 min to 12 h, with nausea/imbalance, recovering between attacks — the defining Ménière vertigo window (Bárány/AAO-HNS criteria, Lopez-Escamez J Vestib Res 2015; AAO-HNS CPG Basura 2020)
    recurrent_spontaneous_vertigo_20min_to_12h
  • symptom
    Fluctuating unilateral hearing loss + tinnitus + aural fullness (especially worse around vertigo spells) — the cochlear arm of the Ménière tetrad (Lopez-Escamez 2015; Basura OHNS 2020)
    fluctuating_unilateral_hearing_tinnitus_fullness
  • imaging
    Audiogram showing low-to-mid-frequency sensorineural hearing loss in one ear (documented before/during/after a vertigo episode) — the OBJECTIVE diagnostic anchor that defines definite MD (Lopez-Escamez J Vestib Res 2015 — audiometric documentation required)
    audiogram_low_mid_frequency_snhl
  • history
    Established / recurrent Ménière on a longitudinal problem list (≥2 prior definitive spells, on stepped therapy, or escalating frequency) — chronic stepped-care + bilaterality-surveillance entry (Huppert Acta Otolaryngol 2010 — bilaterality rises with duration; AAO-HNS CPG 2020)
    recurrent_audiovestibular_episodes_followup
  • symptom
    Sudden unprovoked drop/“pushed to the ground” without LOC (Tumarkin otolithic crisis) in a patient with known/suspected Ménière — high-acuity SAFETY entry (Véleine J Neurosurg 2022; Huppert Acta Otolaryngol 2010; AAO-HNS CPG 2020)
    tumarkin_drop_attack_otolithic_crisis

Required inputs (16)

  • vertigo_spell_duration_20min_to_12hrequired
    symptom • used at ENTRY
    The single most discriminating history item — MD spells are 20 min-12 h spontaneous; BPPV is seconds, vestibular neuritis a single sustained AVS over days, vestibular migraine minutes-days; duration reroutes the entire differential (Lopez-Escamez J Vestib Res 2015 Bárány criteria)
  • spontaneous_vs_positional_triggerrequired
    symptom • used at CONTEXT
    MD vertigo is SPONTANEOUS (not obligately position-triggered); an obligate head-position trigger with <1-min spells points to BPPV — route to ent.bppv.core.v1 (Lopez-Escamez 2015; AAO-HNS BPPV CPG)
  • fluctuating_low_mid_frequency_hearing_lossrequired
    symptom • used at CONTEXT
    Fluctuating LOW/MID-frequency SNHL in the affected ear is the cochlear pivot; purely high-frequency, non-fluctuating, or absent cochlear involvement argues against MD and toward presbycusis / vestibular migraine / neuritis (Lopez-Escamez 2015; Basura OHNS 2020)
  • tinnitus_and_aural_fullness_affected_earrequired
    symptom • used at CONTEXT
    Unilateral tinnitus + aural fullness completing the tetrad; bilateral or absent aural symptoms lowers the MD prior and raises migraine / systemic / autoimmune mimics (Lopez-Escamez 2015)
  • pure_tone_audiometry_documentationrequired
    imaging • used at INITIAL_WORKUP
    Definite MD REQUIRES audiometrically documented low/mid SNHL in the affected ear on at least one occasion — the objective anchor that separates definite from probable MD and from purely-history mimics (Lopez-Escamez J Vestib Res 2015; AAO-HNS CPG Basura 2020)
  • migraine_history_photophobia_aurarequired
    history • used at CONTEXT
    Migraine history / headache / photophobia-phonophobia with episodic vertigo points to vestibular migraine — the commonest MD mimic AND frequently comorbid; alters route and treatment (Lopez-Escamez 2015 — VM is the principal differential; Webster Cochrane 2023 VM prophylaxis)
  • central_neuro_4Ds_gait_during_or_between_spellsrequired
    symptom • used at RED_FLAGS
    The 4 D's (diplopia, dysarthria, dysphagia, dysmetria), severe truncal/gait ataxia, or other focal signs — episodic posterior-circulation TIA / AICA infarct can mimic recurrent audiovestibular spells; route OUT to neuro.posterior-circulation-stroke.core.v1 (AAO-HNS CPG 2020; clinical)
  • vascular_risk_factorsrequired
    history • used at CONTEXT
    Age, HTN, DM, AF, smoking, hyperlipidaemia, prior stroke/TIA raise the pre-test posterior-circulation-TIA prior for recurrent vertigo+hearing spells and lower the imaging/route-out threshold (clinical; ABCD2 context)
  • sudden_severe_unilateral_hearing_loss_componentrequired
    symptom • used at RED_FLAGS
    A SUDDEN (≤72 h) severe SNHL component — especially a first event — is SSNHL until proven otherwise (time-critical, steroid-window) → route to ent.sudden-sensorineural-hearing-loss.core.v1; can also be delayed endolymphatic hydrops post-SSNHL (Basura OHNS 2020; clinical)
  • tumarkin_drop_attacksrequired
    symptom • used at RISK_STRATIFICATION
    Sudden unprovoked falls without LOC (otolithic crisis) carry a high injury/fracture risk and mandate accelerated ablative/surgical escalation + a falls-safety pathway (Véleine J Neurosurg 2022; Huppert Acta Otolaryngol 2010; AAO-HNS CPG 2020)
  • bilateral_or_contralateral_progressionrequired
    history • used at TREATMENT
    Bilateral involvement (rises to ~35% at 10 y / ~47% at 20 y) mandates a HEARING-PRESERVATION strategy and contraindicates bilateral ablation — a pivotal management modifier (Huppert/Strupp/Brandt Acta Otolaryngol 2010; AAO-HNS CPG 2020)
  • autoimmune_features_bilateral_rapidly_progressive
    history • used at DIFFERENTIAL
    Bilateral, rapidly progressive, steroid-responsive audiovestibular loss ± systemic autoimmune disease suggests autoimmune inner-ear disease (AIED) rather than idiopathic MD — different work-up/therapy (Basura OHNS 2020 — exclude alternative causes)
  • prior_sudden_snhl_event
    history • used at CONTEXT
    A prior treated SSNHL episode followed later by recurrent vertigo defines DELAYED endolymphatic hydrops — a recognised MD-spectrum variant managed on the same ladder (Basura OHNS 2020; clinical)
  • pregnancy
    history • used at TREATMENT
    Pregnancy gates the regimen — avoid routine diuretics/acetazolamide (volume/electrolyte, teratogenicity concerns) and limit antiemetic/vestibular-suppressant choice; favour low-salt + non-pharm + IT steroid if needed (AAO-HNS CPG 2020 safety; clinical)
  • hearing_status_affected_and_better_earrequired
    history • used at TREATMENT
    Serviceable hearing in the affected ear (and the only-hearing-ear question) sets the ABLATION GUARDRAIL — IT gentamicin / labyrinthectomy are hearing-destructive and are contraindicated where hearing must be preserved (Patel Lancet 2016; AAO-HNS CPG 2020)
  • driving_occupational_safety_exposure
    symptom • used at FOLLOWUP
    Unpredictable vertigo / drop attacks create driving and occupational (heights, machinery) hazard — mandatory safety counselling and, in many jurisdictions, fitness-to-drive reporting (AAO-HNS CPG 2020 — patient education/counselling)

12-phase flow (12)

  1. 1FRAME
    Frame the encounter as a TWO-CHANNEL problem: (A) positively diagnose definite-vs-probable Ménière against the Bárány/AAO-HNS criteria, anchored on the AUDIOGRAM, and run the stepped management ladder incl. the Tumarkin drop-attack safety issue; (B) robustly distinguish vestibular migraine (commonest mimic, often comorbid), BPPV, vestibular neuritis, posterior-circulation TIA/stroke, SSNHL, and autoimmune inner-ear disease. Definitive stroke/BPPV/neuritis/migraine/SSNHL/falls management is routed OUT by engine_id, not re-authored.
    advance: two-channel scope confirmed; out-of-scope definitive management flagged for engine_id routing
  2. 2ENTRY
    Apply the duration pivot at the door: spontaneous episodic vertigo 20 min-12 h with fluctuating unilateral cochlear symptoms = the Ménière channel; seconds-positional → BPPV channel; single sustained AVS over days → vestibular-neuritis channel; minutes-days + migraine features → vestibular-migraine channel. Capture spell duration first because it reroutes the entire work-up (Lopez-Escamez J Vestib Res 2015 Bárány criteria).
    inputs: vertigo_spell_duration_20min_to_12h
    actions: workup.vertigo
    advance: spell-duration / spontaneity category assigned and the Ménière-vs-mimic channel selected
  3. 3CONTEXT
    Build the pre-test differential: spontaneous (not obligately positional) trigger; fluctuating LOW/MID-frequency SNHL; unilateral tinnitus + aural fullness; migraine history (vestibular migraine — commonest mimic, often comorbid); vascular risk factors (posterior-circulation TIA prior for recurrent spells); prior SSNHL (delayed endolymphatic hydrops). This phase sets the Bayesian prior before audiometry.
    inputs: spontaneous_vs_positional_trigger, fluctuating_low_mid_frequency_hearing_loss, tinnitus_and_aural_fullness_affected_ear, migraine_history_photophobia_aura, vascular_risk_factors, prior_sudden_snhl_event
    actions: workup.vertigo
    advance: Ménière-vs-VM-vs-BPPV-vs-neuritis-vs-TIA prior assigned from triggers/duration/auditory/vascular profile
  4. 4RED_FLAGS
    Screen the can't-miss exits: (1) the 4 D's / severe gait-truncal ataxia / focal signs during or between spells, or high vascular risk with recurrent audiovestibular spells → posterior-circulation TIA/AICA-territory stroke → route OUT to neuro.posterior-circulation-stroke.core.v1; (2) a SUDDEN (≤72 h) severe SNHL component (esp. first event) → SSNHL (time-critical steroid window) → route OUT to ent.sudden-sensorineural-hearing-loss.core.v1. These are recognised here and NOT managed here.
    inputs: central_neuro_4Ds_gait_during_or_between_spells, sudden_severe_unilateral_hearing_loss_component
    actions: calc.abcd2, workup.acute_stroke, workup.acute_headache
    advance: central + sudden-SNHL red flags screened and routed OUT by engine_id if positive
  5. 5INITIAL_WORKUP
    Obtain PURE-TONE AUDIOMETRY — definite MD REQUIRES audiometrically documented low-to-mid-frequency SNHL in the affected ear on at least one occasion (the objective anchor). Tympanometry + speech audiometry; document the fluctuation pattern across visits. Routine MRI/IAC imaging is NOT required to diagnose MD but IS indicated to exclude retrocochlear pathology (vestibular schwannoma) when asymmetry/atypia is present (AAO-HNS CPG Basura 2020 — against routine imaging when criteria met, for imaging when atypical).
    inputs: pure_tone_audiometry_documentation
    actions: workup.vertigo
    advance: audiogram obtained + low/mid SNHL documented (definite) OR criteria incomplete (probable) OR retrocochlear imaging triggered
  6. 6BRANCHING_WORKUP
    Classify definite vs probable MD (Bárány/AAO-HNS). If atypical (asymmetric/rapidly progressive, bilateral, systemic features) → MRI IAC to exclude vestibular schwannoma; autoimmune work-up if bilateral rapidly progressive steroid-responsive (AIED); ECochG/vestibular testing optional adjuncts (not required). If migraine features dominate without documented SNHL → reframe toward vestibular migraine and route to neuro.vestibular-migraine.core.v1. If positional component emerges (secondary BPPV can co-exist) → ent.bppv.core.v1 for the canal-positive component.
    actions: workup.vertigo, panel.inflammation
    advance: definite-vs-probable classified; retrocochlear/autoimmune mimic excluded or routed; comorbid VM/BPPV flagged
  7. 7DIFFERENTIAL
    Terminal vestibular differential with named pivots: Ménière (spontaneous 20 min-12 h spells + DOCUMENTED fluctuating low/mid SNHL + tinnitus/fullness — audiogram pivot) vs vestibular migraine (migraine features, often NO documented SNHL, frequently comorbid — migraine-criteria pivot, route to neuro.vestibular-migraine.core.v1) vs BPPV (seconds, obligate positional trigger, no cochlear symptoms — Dix-Hallpike pivot, route to ent.bppv.core.v1) vs vestibular neuritis (single sustained AVS days, no cochlear symptoms — monophasic-course pivot, route to ent.vestibular-neuritis.core.v1) vs posterior-circulation TIA/stroke (vascular risk + central signs — HINTS/4Ds pivot, route to neuro.posterior-circulation-stroke.core.v1) vs SSNHL (sudden severe SNHL ± vertigo — time-course pivot, route to ent.sudden-sensorineural-hearing-loss.core.v1) vs autoimmune inner-ear disease (bilateral rapidly progressive steroid-responsive — bilaterality+steroid-response pivot).
    inputs: autoimmune_features_bilateral_rapidly_progressive
    actions: workup.vertigo
    advance: single best diagnosis (definite/probable MD or a mimic) selected; comorbid VM/BPPV flagged; out-of-scope diagnoses routed by engine_id
  8. 8RISK_STRATIFICATION
    Stratify by attack burden + the Tumarkin drop-attack SAFETY axis + functional/fall-injury risk. Tumarkin otolithic crisis (sudden unprovoked falls, no LOC) is the highest-acuity feature — accelerates the ablative/surgical decision and triggers a falls-safety pathway. Layer hearing status (serviceable vs non-serviceable), bilaterality, frailty, anticoagulation, driving/occupational exposure as management modifiers.
    inputs: tumarkin_drop_attacks, vascular_risk_factors
    actions: calc.clinical_frailty_scale, calc.abcd2, calc.news2, workup.falls
    advance: attack burden + Tumarkin/fall-injury risk + hearing/bilaterality modifiers assigned
  9. 9TREATMENT
    STEPPED management ladder (AAO-HNS CPG Basura 2020). Acute attack: short-course antiemetic / vestibular suppressant + reassurance (symptomatic only). Maintenance ladder: (1) lifestyle + low-salt diet ± diuretic ± betahistine (region-dependent; BEMED-negative — set realistic expectations) — low-quality evidence, low-harm first tier; (2) intratympanic CORTICOSTEROID (non-ablative, hearing-sparing — Patel Lancet 2016 non-inferior to gentamicin); (3) intratympanic GENTAMICIN (ABLATIVE — vestibulotoxic, HEARING-RISK guardrail: contraindicated if serviceable hearing must be preserved or only-hearing-ear / bilateral disease); (4) endolymphatic sac surgery / vestibular neurectomy (hearing-preserving) / labyrinthectomy (hearing-destructive) for intractable disease. Vestibular rehabilitation for chronic imbalance/compensation. Bilateral disease → hearing-preservation strategy, AVOID bilateral ablation. Pregnancy → low-salt + non-pharm ± IT steroid, avoid routine diuretic/acetazolamide.
    inputs: bilateral_or_contralateral_progression, hearing_status_affected_and_better_ear, pregnancy
    advance: appropriate ladder step selected with the ablation/hearing guardrail applied; acute-attack symptomatic plan set; bilateral/pregnancy modifiers honoured
  10. 10DISPOSITION
    Most MD is OUTPATIENT (audiology + neurotology longitudinal care). Admit/observe for: intractable vomiting with dehydration, a severe disabling attack precluding safe discharge, recurrent injurious Tumarkin drop attacks, or a red-flag mimic requiring the stroke/SSNHL pathway (route OUT). Intractable disease failing the medical ladder → neurotology referral for IT gentamicin / surgical decision.
    inputs: tumarkin_drop_attacks, central_neuro_4Ds_gait_during_or_between_spells
    actions: workup.falls
    advance: disposition documented; red-flag mimics routed OUT; intractable/Tumarkin cases escalated to neurotology
  11. 11MONITORING
    CHRONIC stepped-care monitoring: SERIAL AUDIOMETRY (track low/mid SNHL fluctuation and stepwise progression — hearing loss accrues mainly in the first 5-10 y, ~50-60 dB; Huppert 2010); vertigo-attack diary (frequency/severity drives ladder step-up/step-down); after IT GENTAMICIN, monitor hearing closely (ablation hearing-loss risk) and balance compensation; after IT steroid, reassess attack control to decide repeat vs escalate; diuretic course → electrolytes/renal monitoring; surveil for CONTRALATERAL progression to bilateral disease (changes the whole hearing-preservation strategy). Counsel that attack frequency tends to burn out over years even though hearing may decline.
    inputs: pure_tone_audiometry_documentation, bilateral_or_contralateral_progression
    actions: workup.vertigo, panel.cmp
    advance: serial audiogram + attack-diary trend reviewed; post-intervention hearing/electrolyte monitoring done; bilaterality surveillance documented
  12. 12FOLLOWUP
    CHRONIC longitudinal arc (the core differentiator): structured neurotology + audiology follow-up with the stepped ladder revisited at each interval; vestibular rehabilitation for residual imbalance; hearing rehabilitation (hearing aid; cochlear implant if profound and the contralateral ear is also affected); psychological support (MD has high anxiety/depression burden); DRIVING and OCCUPATIONAL safety counselling (unpredictable vertigo / Tumarkin — fitness-to-drive reporting per jurisdiction); bilaterality surveillance and hearing-preservation planning; route elderly recurrent drop-attack patients to geriatrics.falls.core.v1 for the multifactorial falls programme.
    inputs: driving_occupational_safety_exposure, bilateral_or_contralateral_progression
    actions: workup.falls
    advance: longitudinal stepped-care + vestibular/hearing rehab + safety-counselling + bilaterality-surveillance plan documented; falls referral made if criteria met