Guideline sources The following summarized guidelines for the evaluation and management of M ni re's disease are prepared by our editorial team based on guidelines from the American Cochlear Implant Alliance (ACI Alliance 2022), the American Physical Therapy Association (APTA 2022), the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2020), the Danish Health Authority (DHA 2018), and the American Academy of Neurology (AAN 2017). 1 2 3 4 5 Calculator ICVD criteria for M ni re's disea https://web.pathway.md/diseases/recsjA1sLlSz8w5Nb 1/5 6/30/23, 1:06 AM M ni re's disease Pathway Guidelines 1. Screening and diagnosis Diagnostic criteria: Diagnose definite or probable M ni re's disease in patients presenting with 2 episodes of vertigo lasting 20 minutes to 12 hours (definite) or up to 24 hours (probable) and fluctuating or non-fluctuating sensorineural hearing loss, tinnitus, or pressure in the affected ear, when these symptoms are not better accounted for by another disorder. B Determine if patients meet diagnostic criteria for vestibular migraine when assessing for M ni re's disease. B 2. Diagnostic investigations Audiometry: obtain audiometry when assessing patients for the diagnosis of M ni re's disease. B Magnetic resonance imaging: consider obtaining MRI of the internal auditory canal and posterior fossa in patients with possible M ni re's disease and audiometrically verified asymmetric sensorineural hearing loss. C Vestibular function testing: As per AAO-HNSF 2020 guidelines, do not obtain routine vestibular function testing to establish the diagnosis of M ni re's disease. D As per AAN 2017 guidelines: Consider obtaining cervical vestibular evoked myogenic potential testing as an ancillary test for vestibular dysfunction in M ni re disease. C Insufficient evidence to recommend cervical or ocular vestibular evoked myogenic potential testing for the diagnosis of M ni re disease. I 3. Diagnostic procedures Electrocochleography: do not obtain routine electrocochleography to establish the diagnosis of M ni re's disease. D 4. Medical management Acute therapy: offer a limited course of vestibular suppressants for the management of acute vertigo attacks in patients with M ni re's disease. B Maintenance therapy: As per AAO-HNSF 2020 guidelines, consider offering diuretics and/or betahistine for maintenance therapy to reduce symptoms or prevent attacks in patients with M ni re's disease. https://web.pathway.md/diseases/recsjA1sLlSz8w5Nb 2/5 6/30/23, 1:06 AM M ni re's disease Pathway C As per DHA 2018 guidelines: Do not use routine betahistine in patients with M ni re's disease, since the beneficial effect is uncertain. D Consider offering routine thiazide diuretics in patients with M ni re's disease. E 5. Nonpharmacologic interventions Dietary and lifestyle changes: advise patients with M ni re's disease on dietary and lifestyle modifications that may reduce or prevent symptoms. B Vestibular rehabilitation: As per APTA 2022 guidelines, offer vestibular physical therapy in patients with acute, subacute, or chronic unilateral vestibular hypofunction. A Show 9 more As per AAO-HNSF 2020 guidelines: Offer vestibular rehabilitation/physical therapy in patients with M ni re's disease and chronic imbalance. B Do not offer vestibular rehabilitation/physical therapy for the management of acute vertigo attacks in patients with M ni re's disease. D As per DHA 2018 guidelines, consider offering vestibular rehabilitation in patients with M ni re's disease. C Psychological support: consider providing psychological support in patients with M ni re's disease. E 6. Therapeutic procedures Positive pressure therapy: As per AAO-HNSF 2020 guidelines, do not perform positive pressure therapy in patients with M ni re's disease. D As per DHA 2018 guidelines, consider performing positive pressure therapy in patients with M ni re's disease. C Intratympanic corticosteroids: As per AAO-HNSF 2020 guidelines, consider performing, or referring to a clinician who can perform, intratympanic corticosteroid injections in patients with active M ni re's disease not responsive to noninvasive treatment. C As per DHA 2018 guidelines, consider performing intratympanic corticosteroid injections in patients with M ni re's disease, if previous treatments have been ineffective. C Intratympanic gentamicin: https://web.pathway.md/diseases/recsjA1sLlSz8w5Nb 3/5 6/30/23, 1:06 AM M ni re's disease Pathway As per AAO-HNSF 2020 guidelines, perform, or refer to a clinician who can perform, intratympanic gentamicin injections in patients with active M ni re's disease not responsive to non-ablative treatment. B As per DHA 2018 guidelines, consider performing intratympanic gentamicin injections in patients with M ni re's disease, if other treatments have been ineffective. C Hearing aids: counsel patients with M ni re's disease and hearing loss on the use of amplification and hearing assistive technology. B Cochlear implants: discuss and offer a trial of a non-surgical hearing technology where possible before undergoing cochlear implantation in patients with single-sided deafness. E Show 10 more 7. Surgical interventions Tympanostomy: consider performing tympanostomy tube placement in patients with M ni re's disease. E Labyrinthectomy: consider performing, or referring to a clinician who can perform, labyrinthectomy in patients with active M ni re's disease failed less definitive therapy and having non-usable hearing. C Endolymphatic sac surgery: perform endolymphatic sac surgery in patients with M ni re's disease only after due consideration. B 8. Patient education Patient education: educate patients with M ni re's disease about the natural history, measures for symptom control, treatment options, and outcomes of this disease. B 9. Follow-up and surveillance Follow-up assessment: document resolution, improvement, or worsening of vertigo, tinnitus, and hearing loss and any change in quality of life in patients with M ni re's disease after treatment. B Clinical findings Symptoms Past medical history Feeling of fullness in the ear Ankylosing spondylitis No prodromal vertigo Hashimoto's disease Tinnitus Migraine Vertigo Rheumatoid arthritis https://web.pathway.md/diseases/recsjA1sLlSz8w5Nb 4/5 6/30/23, 1:06 AM M ni re's disease Pathway SLE Past events Neurological exam Falls Nystagmus Sensorineural hearing loss References 1. Gregory J Basura, Meredith E Adams, Ashkan Monfared et al. Clinical Practice Guideline: M ni re's Disease. Otolaryngol Head Neck Surg. 2020 Apr;162 2_suppl):S1 S55. Open 2. Terry D Fife, James G Colebatch, Kevin A Kerber et al. Practice guideline: Cervical and ocular vestibular evoked myogenic potential testing: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Nov 28;89 22 2288 2296. Open 3. Danish Health Authority. National Clinical Guideline for the Treatment of M ni re's Disease: Quick guide. Sundhedsstyrelsen. 2018 Sep. Open 4. Lisa R Park, Amanda M Griffin, Douglas P Sladen et al. American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single- Sided Deafness. Ear Hear. 2022 Mar/Apr;43 2 255 267. Open 5. Courtney D Hall, Susan J Herdman, Susan L Whitney et al. Vestibular Rehabilitation for Peripheral Vestibular Hypofunction: An Updated Clinical Practice Guideline From the Academy of Neurologic Physical Therapy of the American Physical Therapy Association. J Neurol Phys Ther. 2022 Apr 1;46 2 118 177. Open 6. Jose A Lopez-Escamez, John Carey, Won-Ho Chung et al. Diagnostic criteria for Meni re s disease. J Vestib Res. 2015;25 1 1 7. Open https://web.pathway.md/diseases/recsjA1sLlSz8w5Nb 5/5