{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T19:11:10Z","timestamp":1792782670049,"version":"4.5.0"},"reference-count":125,"publisher":"Springer Science and Business Media LLC","issue":"11","license":[{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"University of Zurich","id":[{"id":"https:\/\/ror.org\/02crff812","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2026,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Vertigo, dizziness, and imbalance are among the most frequent presenting symptoms in primary care and in the emergency department (ED). The clinical diagnosis is based on a structured patient history and bedside examination of the vestibular system and the ocular motor system, looking especially for central ocular motor signs. Recent guidelines, such as the GRACE\u22123 guideline on acute vertigo and dizziness, the increasing availability of bedside diagnostic algorithms, and the use of quantitative vestibular and ocular motor testing in both the ED-setting and in the outpatient center have facilitated and standardized the approach to the dizzy patient. Here we review recent developments in neuro-otology and neuro-ophthalmology, covering the period after the latest neuro-otology update was published in this journal in 2023. Both new-onset acute, episodic and chronic vestibular conditions are discussed separately, highlighting advancements in disease characterization, diagnostic criteria, and treatment strategies. While major progress has been made in terms of the pathophysiology, etiology, genetics, and diagnostic criteria of peripheral, central, and functional vestibular diseases as well as ocular motor disorders, this knowledge has to be transferred into clinical practice. Systematic teaching of doctors to increase knowledge and to improve skills in applying the different tests in clinical practice on a high level are identified challenges. In terms of treatment, there is still a very great need for state-of-the art clinical trials applying approved therapies.<\/jats:p>","DOI":"10.1007\/s00415-026-14153-w","type":"journal-article","created":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T14:30:40Z","timestamp":1791729040000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An update on neuro-otology and neuro-ophthalmology \u2013 focusing on new developments in the last three years"],"prefix":"10.1007","volume":"273","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6984-6958","authenticated-orcid":false,"given":"Alexander A.","family":"Tarnutzer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael L.","family":"Strupp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louisa","family":"Lehner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,11]]},"reference":[{"issue":"5","key":"14153_CR1","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1111\/acem.14728","volume":"30","author":"JA Edlow","year":"2023","unstructured":"Edlow JA, Carpenter C, Akhter M, Khoujah D, Marcolini E, Meurer WJ, Morrill D, Naples JG, Ohle R, Omron R, Sharif S, Siket M, Upadhye S, LOJ ES, SundbergTartt EKS, Vanni, Newman-TokerBellolio DEF (2023) Guidelines for reasonable and appropriate care in the emergency department 3 (GRACE-3): Acute dizziness and vertigo in the emergency department. Acad Emerg Med 30(5):442\u2013486","journal-title":"Acad Emerg Med"},{"key":"14153_CR2","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1007\/s00106-025-01599-z","volume":"73","author":"AA Tarnutzer","year":"2025","unstructured":"Tarnutzer AA, Kerkeni H, Diener S, Kalla R, Candreia C, Piantanida R, Maire R, Welge-Lussen A, Budweg J, Zwergal A, Dlugaiczyk J (2025) Diagnosis and treatment of vertigo and dizziness: interdisciplinary guidance paper for clinical practice. HNO 73:357\u2013369","journal-title":"HNO"},{"key":"14153_CR3","doi-asserted-by":"publisher","first-page":"6170","DOI":"10.1007\/s00415-023-11922-9","volume":"270","author":"GM Halmagyi","year":"2023","unstructured":"Halmagyi GM, Akdal G, Welgampola MS, Wang C (2023) Neurological update: neuro-otology 2023. J Neurol 270:6170\u20136192","journal-title":"J Neurol"},{"key":"14153_CR4","unstructured":"ICD-11 (Mortality and Morbidity Statistics). In:"},{"key":"14153_CR5","doi-asserted-by":"publisher","DOI":"10.1111\/ene.70651","volume":"33","author":"K Schmidt","year":"2026","unstructured":"Schmidt K, Zwergal A, Kaski D, Tarnutzer AA (2026) The epidemiology and clinical presentation of the acute imbalance syndrome (AIS)-a systematic review and meta-analysis. Eur J Neurol 33:e70651","journal-title":"Eur J Neurol"},{"issue":"3","key":"14153_CR6","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1007\/s00415-026-13720-5","volume":"273","author":"FA Coban","year":"2026","unstructured":"Coban FA, Tarnutzer AA (2026) The epidemiology and clinical presentation of acute vestibular syndromes: a systematic review of the literature. J Neurol 273(3):173","journal-title":"J Neurol"},{"issue":"9","key":"14153_CR7","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000218624","volume":"107","author":"K M\u00f6hwald","year":"2026","unstructured":"M\u00f6hwald K, Hadzhikolev H, Eberle L, Jaufenthaler P, Strupp M, Jahn K, Conrad J, Zwergal A (2026) Acute imbalance syndrome (AIS) versus acute vestibular syndrome (AVS): clinical and topographical differentiation. Neurology 107(9):e218624","journal-title":"Neurology"},{"key":"14153_CR8","doi-asserted-by":"publisher","first-page":"389","DOI":"10.3233\/VES-220201","volume":"32","author":"M Strupp","year":"2022","unstructured":"Strupp M, Bisdorff A, Furman J, Hornibrook J, Jahn K, Maire R, Newman-Toker D, Magnusson M (2022) Acute unilateral vestibulopathy\/vestibular neuritis: diagnostic criteria. J Vestib Res 32:389\u2013406","journal-title":"J Vestib Res"},{"key":"14153_CR9","doi-asserted-by":"publisher","first-page":"343","DOI":"10.1016\/j.anl.2023.09.009","volume":"51","author":"S Iwasaki","year":"2024","unstructured":"Iwasaki S, Kawahara T, Miyashita T, Shindo S, Tsubota M, Inoue A, Sunami K, Shojaku H, Committee for Epidemiology of the Japan Society for Equilibrium R (2024) Estimated incidence and characteristics of vestibular neuritis in Japan: a nationwide survey. Auris Nasus Larynx 51:343\u2013346","journal-title":"Auris Nasus Larynx"},{"key":"14153_CR10","doi-asserted-by":"publisher","first-page":"507","DOI":"10.1111\/coa.14284","volume":"50","author":"C Fujimoto","year":"2025","unstructured":"Fujimoto C, Sugasawa K, Ichijo K, Oka M, Kamogashira T, Kinoshita M, Kawahara T, Yamasoba T (2025) Vestibular function in patients with vestibular neuritis experiencing prodromal dizziness. Clin Otolaryngol 50:507\u2013513","journal-title":"Clin Otolaryngol"},{"key":"14153_CR11","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1177\/09574271241300326","volume":"35","author":"X Liu","year":"2025","unstructured":"Liu X, Xu X (2025) Reconsideration of acute unilateral vestibulopathy\/vestibular neuritis: a prospective cohort study with function test-based classification. J Vestib Res 35:73\u201381","journal-title":"J Vestib Res"},{"key":"14153_CR12","doi-asserted-by":"publisher","first-page":"2030","DOI":"10.1002\/acn3.52123","volume":"11","author":"KT Kim","year":"2024","unstructured":"Kim KT, Park S, Lee SU, Park E, Kim B, Kim BJ, Kim JS (2024) Four-hour-delayed 3D-FLAIR MRIs in patients with acute unilateral peripheral vestibulopathy. Ann Clin Transl Neurol 11:2030\u20132039","journal-title":"Ann Clin Transl Neurol"},{"key":"14153_CR13","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2024.1339039","volume":"15","author":"N Jaganathan","year":"2024","unstructured":"Jaganathan N, Mohamed MH, Md Pauzi AL, Mahayidin H, Hanapai AF, Wan Sulaiman WA, Basri H, Inche Mat L (2024) Video head impulse test in stroke: a review of published studies. Front Neurol 15:1339039","journal-title":"Front Neurol"},{"key":"14153_CR14","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1177\/09574271241307649","volume":"35","author":"J Sjogren","year":"2025","unstructured":"Sjogren J, Fransson PA, Magnusson M, Karlberg M, Tjernstrom F (2025) Acute unilateral vestibulopathy and corticosteroid treatment - a randomized placebo-controlled double-blind trial. J Vestib Res 35:91\u2013101","journal-title":"J Vestib Res"},{"key":"14153_CR15","doi-asserted-by":"publisher","first-page":"354","DOI":"10.1056\/NEJMoa033280","volume":"351","author":"M Strupp","year":"2004","unstructured":"Strupp M, Zingler VC, Arbusow V, Niklas D, Maag KP, Dieterich M, Bense S, Theil D, Jahn K, Brandt T (2004) Methylprednisolone, valacyclovir, or the combination for vestibular neuritis. N Engl J Med 351:354\u2013361","journal-title":"N Engl J Med"},{"key":"14153_CR16","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1278307","volume":"14","author":"J Chen","year":"2023","unstructured":"Chen J, Liu Z, Xie Y, Jin S (2023) Effects of vestibular rehabilitation training combined with anti-vertigo drugs on vertigo and balance function in patients with vestibular neuronitis: a systematic review and meta-analysis. Front Neurol 14:1278307","journal-title":"Front Neurol"},{"key":"14153_CR17","doi-asserted-by":"publisher","first-page":"1396891","DOI":"10.3389\/fneur.2024.1396891","volume":"15","author":"HE Agger-Nielsen","year":"2024","unstructured":"Agger-Nielsen HE, Grondberg TS, Berg-Beckhoff G, Ovesen T (2024) Early vestibular rehabilitation training of peripheral acute vestibular syndrome-a systematic review and meta-analysis. Front Neurol 15:1396891","journal-title":"Front Neurol"},{"key":"14153_CR18","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1097\/NPT.0000000000000382","volume":"46","author":"CD Hall","year":"2022","unstructured":"Hall CD, Herdman SJ, Whitney SL, Anson ER, Carender WJ, Hoppes CW, Cass SP, Christy JB, Cohen HS, Fife TD, Furman JM, Shepard NT, Clendaniel RA, Dishman JD, Goebel JA, Meldrum D, Ryan C, Wallace RL, Woodward NJ (2022) 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 46:118\u2013177","journal-title":"J Neurol Phys Ther"},{"key":"14153_CR19","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2025.1687181","volume":"16","author":"HJ Lee","year":"2025","unstructured":"Lee HJ, Lee DH, Seo JH, Son EJ, Jeon EJ (2025) Effectiveness of three vestibular rehabilitation exercises for treating acute unilateral peripheral vestibular dysfunction: a multicenter randomized study. Front Neurol 16:1687181","journal-title":"Front Neurol"},{"key":"14153_CR20","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1175481","volume":"14","author":"M Antons","year":"2023","unstructured":"Antons M, Lindner M, Eilles E, Gunther L, Delker A, Branner C, Kramer A, Beck R, Oos R, Wuehr M, Ziegler S, Strupp M, Zwergal A (2023) Dose- and application route-dependent effects of betahistine on behavioral recovery and neuroplasticity after acute unilateral labyrinthectomy in rats. Front Neurol 14:1175481","journal-title":"Front Neurol"},{"key":"14153_CR21","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1208902","volume":"14","author":"M Wuthrich","year":"2023","unstructured":"Wuthrich M, Wang Z, Martinez CM, Carmona S, Mantokoudis G, Tarnutzer AA (2023) Systematic review and meta-analysis of the diagnostic accuracy of spontaneous nystagmus patterns in acute vestibular syndrome. Front Neurol 14:1208902","journal-title":"Front Neurol"},{"key":"14153_CR22","doi-asserted-by":"publisher","first-page":"986","DOI":"10.1111\/acem.12223","volume":"20","author":"DE Newman-Toker","year":"2013","unstructured":"Newman-Toker DE, Kerber KA, Hsieh YH, Pula JH, Omron R, Saber Tehrani AS, Mantokoudis G, Hanley DF, Zee DS, Kattah JC (2013) HINTS outperforms ABCD2 to screen for stroke in acute continuous vertigo and dizziness. Acad Emerg Med 20:986\u2013996","journal-title":"Acad Emerg Med"},{"key":"14153_CR23","doi-asserted-by":"publisher","DOI":"10.3390\/brainsci15070725","author":"AA Tarnutzer","year":"2025","unstructured":"Tarnutzer AA, Lee SU, Kim JS, Kaski D (2025) The challenge of diagnosing labyrinthine stroke-a critical review. Brain Sci. https:\/\/doi.org\/10.3390\/brainsci15070725","journal-title":"Brain Sci"},{"key":"14153_CR24","doi-asserted-by":"publisher","first-page":"1781","DOI":"10.1007\/s00405-023-08296-z","volume":"281","author":"M von Werdt","year":"2024","unstructured":"von Werdt M, Korda A, Zamaro E, Wagner F, Kompis M, Caversaccio MD, Mantokoudis G (2024) The acute vestibular syndrome: prevalence of new hearing loss and its diagnostic value. Eur Arch Otorhinolaryngol 281:1781\u20131787","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR25","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2024.1448989","volume":"15","author":"SH Kim","year":"2024","unstructured":"Kim SH, Kim H, Lee SU, Park E, Cho BH, Cho KH, Kim GJ, Yu S, Kim JS (2024) Bilaterally positive head-impulse tests can differentiate AICA infarction from labyrinthitis. Front Neurol 15:1448989","journal-title":"Front Neurol"},{"key":"14153_CR26","doi-asserted-by":"publisher","first-page":"3504","DOI":"10.1161\/STROKEAHA.109.551234","volume":"40","author":"JC Kattah","year":"2009","unstructured":"Kattah JC, Talkad AV, Wang DZ, Hsieh YH, Newman-Toker DE (2009) HINTS to diagnose stroke in the acute vestibular syndrome: three-step bedside oculomotor examination more sensitive than early MRI diffusion-weighted imaging. Stroke 40:3504\u20133510","journal-title":"Stroke"},{"key":"14153_CR27","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2025.1690103","volume":"16","author":"S Pan","year":"2025","unstructured":"Pan S, Zhang Y, Ding X, Pan X, Chen X, Lv X, You H, Zhu J (2025) Acute vestibular syndrome induced by supratentorial stroke: clinical features and network compensation mechanisms. Front Neurol 16:1690103","journal-title":"Front Neurol"},{"key":"14153_CR28","doi-asserted-by":"publisher","first-page":"2174","DOI":"10.1007\/s00415-023-11566-9","volume":"270","author":"JY Park","year":"2023","unstructured":"Park JY, Choi JH, Kwon JH, Weon YC, Lee SM, Kim HJ, Choi SY, Oh EH, Kim HA, Lee H, Kim JS, Choi KD (2023) Incidence, characteristics, and neuroanatomical substrates of vestibular symptoms in supratentorial stroke. J Neurol 270:2174\u20132183","journal-title":"J Neurol"},{"key":"14153_CR29","doi-asserted-by":"publisher","first-page":"1785","DOI":"10.1111\/ene.15729","volume":"30","author":"S Carmona","year":"2023","unstructured":"Carmona S, Martinez C, Zalazar G, Koohi N, Kaski D (2023) Acute truncal ataxia without nystagmus in patients with acute vertigo. Eur J Neurol 30:1785\u20131790","journal-title":"Eur J Neurol"},{"key":"14153_CR30","doi-asserted-by":"publisher","DOI":"10.1111\/ene.16066","volume":"31","author":"F Nikles","year":"2024","unstructured":"Nikles F, Kerkeni H, Zamaro E, Korda A, Wagner F, Sauter TC, Kalla R, Morrison M, Mantokoudis G (2024) Do monosymptomatic stroke patients with dizziness present a vestibular syndrome without nystagmus? An underestimated entity. Eur J Neurol 31:e16066","journal-title":"Eur J Neurol"},{"key":"14153_CR31","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-025-13163-4","volume":"272","author":"G Akdal","year":"2025","unstructured":"Akdal G, Nham B, Kwok B, Ozcelik P, Bradshaw A, Wang C, Halmagyi GM, Welgampola MS (2025) Vestibular event monitoring in acute posterior circulation stroke: from emergency room to stroke unit and beyond. J Neurol 272:510","journal-title":"J Neurol"},{"key":"14153_CR32","first-page":"419","volume":"34","author":"S Vanni","year":"2014","unstructured":"Vanni S, Pecci R, Casati C, Moroni F, Risso M, Ottaviani M, Nazerian P, Grifoni S, Vannucchi P (2014) STANDING, a four-step bedside algorithm for differential diagnosis of acute vertigo in the Emergency Department. Acta Otorhinolaryngol Ital 34:419\u2013426","journal-title":"Acta Otorhinolaryngol Ital"},{"key":"14153_CR33","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1016\/j.annemergmed.2024.06.003","volume":"85","author":"R Ohle","year":"2025","unstructured":"Ohle R, Savage DW, Roy D, McIsaac S, Singh R, Lelli D, Tse D, Johns P, Yadav K, Perry JJ (2025) Development of a clinical risk score to risk stratify for a serious cause of vertigo in patients presenting to the Emergency Department. Ann Emerg Med 85:122\u2013131","journal-title":"Ann Emerg Med"},{"key":"14153_CR34","doi-asserted-by":"publisher","first-page":"2244","DOI":"10.1007\/s12311-024-01718-6","volume":"23","author":"C Martinez","year":"2024","unstructured":"Martinez C, Wang Z, Zalazar G, Carmona S, Kattah J, Tarnutzer AA (2024) Systematic review and meta-analysis of the diagnostic accuracy of a graded gait and truncal instability rating in acutely dizzy and ataxic patients. Cerebellum 23:2244\u20132256","journal-title":"Cerebellum"},{"key":"14153_CR35","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-025-11007-9","volume":"15","author":"E Cortese","year":"2025","unstructured":"Cortese E, Rochelle P, Patel F, Koohi N, Kaski D (2025) Integrated diagnostic algorithm for acute vertigo combining TiTrATE, STANDING, and HINTS: a validation study in the emergency department. Sci Rep 15:25403","journal-title":"Sci Rep"},{"key":"14153_CR36","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1002\/ana.26661","volume":"94","author":"AA Tarnutzer","year":"2023","unstructured":"Tarnutzer AA, Gold D, Wang Z, Robinson KA, Kattah JC, Mantokoudis G, Saber Tehrani AS, Zee DS, Edlow JA, Newman-Toker DE (2023) Impact of clinician training background and stroke location on bedside diagnostic test accuracy in the acute vestibular syndrome - a meta-analysis. Ann Neurol 94:295\u2013308","journal-title":"Ann Neurol"},{"key":"14153_CR37","doi-asserted-by":"publisher","first-page":"487","DOI":"10.1111\/acem.14659","volume":"30","author":"C Gerlier","year":"2023","unstructured":"Gerlier C, Fels A, Vitaux H, Mousset C, Perugini A, Chatellier G, Ganansia O (2023) Effectiveness and reliability of the four-step STANDING algorithm performed by interns and senior emergency physicians for predicting central causes of vertigo. Acad Emerg Med 30:487\u2013500","journal-title":"Acad Emerg Med"},{"key":"14153_CR38","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1136\/emermed-2025-214902","volume":"42","author":"M Ronchetti","year":"2025","unstructured":"Ronchetti M, Bartalucci P, Pepe G, Canaroli G, Magazzini S, De Curtis E, Di Sacco F, Bartolucci M, Pecci R, Casula C, Pelagatti L, Fabiani G, Pavellini A, Caviglioli C, Nazerian P, Vannucchi P, Vanni S, National S, Research Center of Simeu SIdMdEU (2025) Diagnostic accuracy of the STANDING algorithm in patients with isolated vertigo: a multicentre prospective study (STANDING-M). Emerg Med J 42:791\u2013797","journal-title":"Emerg Med J"},{"key":"14153_CR39","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1007\/s00415-026-13641-3","volume":"273","author":"G Anburajan","year":"2026","unstructured":"Anburajan G, Kattah J, Wang Z, Mantokoudis G, Newman-Toker DE, Tarnutzer AA (2026) HINTS+ to diagnose AICA stroke: systematic review of the diagnostic impact of acute unilateral hearing loss in the acute vestibular syndrome. J Neurol 273:131","journal-title":"J Neurol"},{"key":"14153_CR40","doi-asserted-by":"publisher","first-page":"767","DOI":"10.3390\/audiolres13050068","volume":"13","author":"JC Kattah","year":"2023","unstructured":"Kattah JC (2023) Concordant GRADE-3 truncal ataxia and ocular laterodeviation in acute medullary stroke. Audiol Res 13:767\u2013778","journal-title":"Audiol Res"},{"key":"14153_CR41","doi-asserted-by":"publisher","first-page":"248","DOI":"10.1007\/s00415-025-12918-3","volume":"272","author":"C Wang","year":"2025","unstructured":"Wang C, Sreerama J, Nham B, Reid N, Ozalp N, Thomas JO, Cappelen-Smith C, Calic Z, Bradshaw AP, Rosengren SM, Akdal G, Halmagyi GM, Black DA, Burke D, Prasad M, Bharathy GK, Welgampola MS (2025) Separation of stroke from vestibular neuritis using the video head impulse test: machine learning models versus expert clinicians. J Neurol 272:248","journal-title":"J Neurol"},{"key":"14153_CR42","doi-asserted-by":"publisher","first-page":"436","DOI":"10.1007\/s00415-025-13174-1","volume":"272","author":"N Ferri","year":"2025","unstructured":"Ferri N, Bracci A, Metta Franceschelli G, Gnoli G, Turolla A, Pillastrini P, Manzari L, Tramontano M (2025) Accuracy of video head impulse test in stroke diagnosis during acute vestibular syndrome: a systematic review with meta-analysis. J Neurol 272:436","journal-title":"J Neurol"},{"key":"14153_CR43","doi-asserted-by":"publisher","first-page":"774","DOI":"10.1186\/s40001-025-03046-9","volume":"30","author":"MM Jin","year":"2025","unstructured":"Jin MM, Mao XL, Song YL, Zhou YY, Zhang DL, Zhuang QQ, Huang XD, Zhu SL, He SS, Chen W, Zhang X, Zhu JH (2025) Eye movement features for assisting differentiation between posterior circulation infarction and vestibular neuritis in patients of acute vestibular syndrome. Eur J Med Res 30:774","journal-title":"Eur J Med Res"},{"key":"14153_CR44","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000200375","volume":"15","author":"S Badihian","year":"2025","unstructured":"Badihian S, Samaha E, Newman-Toker DE, Zee DS, Kattah JC (2025) Radiographic horizontal conjugate gaze deviation: clinical correlates. Neurol Clin Pract 15:e200375","journal-title":"Neurol Clin Pract"},{"key":"14153_CR45","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000214861","volume":"106","author":"M Strupp","year":"2026","unstructured":"Strupp M, Bhandari A, Brandt T, Halmagyi MG, Kim JS, Kingma H, von Brevern M, Whitney SL, Zhang B, Zuma EMF, Zee DS, Schubert MC (2026) Benign Paroxysmal Positional Vertigo: The Case for Renaming It Peripheral Paroxysmal Positional Vertigo. Neurology 106:e214861","journal-title":"Neurology"},{"key":"14153_CR46","first-page":"6611253","volume":"2025","author":"RG Harrell","year":"2025","unstructured":"Harrell RG, Whitney SL (2025) Improvement in Functional Outcomes in Patients with Benign Paroxysmal Positional Vertigo. Case Rep Neurol Med 2025:6611253","journal-title":"Case Rep Neurol Med"},{"key":"14153_CR47","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2025.1575816","volume":"16","author":"H Li","year":"2025","unstructured":"Li H, Tong Y, Wang Y, Chen M, Cui Y, Wu X, Ju Y (2025) Atypical symptoms and delayed diagnosis are more common in elderly patients with benign paroxysmal positional vertigo: a single-center study. Front Neurol 16:1575816","journal-title":"Front Neurol"},{"key":"14153_CR48","doi-asserted-by":"publisher","DOI":"10.1111\/hex.70551","volume":"29","author":"SJ Lee","year":"2026","unstructured":"Lee SJ, Hur Y (2026) Living with the fear of recurrence in benign paroxysmal positional vertigo. Health Expect 29:e70551","journal-title":"Health Expect"},{"key":"14153_CR49","doi-asserted-by":"publisher","first-page":"326","DOI":"10.21053\/ceo.2025-00030","volume":"18","author":"M Karabulut","year":"2025","unstructured":"Karabulut M, Kutlu S, Viechtbauer W, Melliti A, Meco C, Mohamad A, Ozgirgin ON, Bhandari A, Kingma H, van de Berg R (2025) Co-occurrence of otologic disorders and benign paroxysmal positional vertigo: a systematic review and meta-analysis. Clin Exp Otorhinolaryngol 18:326\u2013338","journal-title":"Clin Exp Otorhinolaryngol"},{"key":"14153_CR50","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1177\/09574271251347261","volume":"36","author":"MK Kim","year":"2026","unstructured":"Kim MK, Kim HJ, Choi JY, Kim JS (2026) Clinical features and outcome predictors in benign paroxysmal positional vertigo and its variant: perspective in a primary care neurology clinic. J Vestib Res 36:57\u201366","journal-title":"J Vestib Res"},{"key":"14153_CR51","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2025.1654404","volume":"16","author":"M Hentze","year":"2025","unstructured":"Hentze M, Hougaard DD, Kingma H (2025) Impact of head orientation and head movement in traditional manual diagnostics of benign paroxysmal positional vertigo: a randomized controlled crossover study. Front Neurol 16:1654404","journal-title":"Front Neurol"},{"key":"14153_CR52","doi-asserted-by":"publisher","first-page":"624","DOI":"10.1097\/MAO.0000000000004823","volume":"47","author":"T Magos","year":"2026","unstructured":"Magos T, McLeod R, Nia FH, Rutka JA (2026) Traumatic BPPV-a large series analysis of 4839 patients, including comprehensive vestibular testing. Otol Neurotol 47:624\u2013629","journal-title":"Otol Neurotol"},{"key":"14153_CR53","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-030-78260-3","volume-title":"Vertigo and dizziness - common complaints","author":"M Strupp","year":"2023","unstructured":"Strupp M, Brandt T, Dieterich M (2023) Vertigo and dizziness - common complaints. SpringerNature, London"},{"key":"14153_CR54","doi-asserted-by":"publisher","first-page":"1865","DOI":"10.1007\/s00405-024-09094-x","volume":"282","author":"L Acle-Cervera","year":"2025","unstructured":"Acle-Cervera L, Carballo-Lahoz L, Esteban-Sanchez J, Alvarez-Morujo-de-Sande MG, Montilla-Ibanez MA, Becares-Martinez C, Gonzalez-Aguado R, Rodriguez-Montesdeoca I, Manrique-Huarte R, Domenech-Vadillo E, Guerra-Jimenez G, Dominguez-Duran E (2025) Life after benign paroxysmal positional vertigo: one-year analysis of recurrence, headaches, neck pain, falls, and functional vestibular symptoms. Eur Arch Otorhinolaryngol 282:1865\u20131877","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR55","doi-asserted-by":"publisher","DOI":"10.1177\/01455613241304913","author":"S Yan","year":"2025","unstructured":"Yan S, Li Z, Chen P, Wu W (2025) Influencing factors for residual symptoms following canalith repositioning maneuver in patients with benign paroxysmal positional vertigo. Ear Nose Throat J. https:\/\/doi.org\/10.1177\/01455613241304913","journal-title":"Ear Nose Throat J"},{"key":"14153_CR56","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.652573","volume":"12","author":"M Strupp","year":"2021","unstructured":"Strupp M, Goldschagg N, Vinck AS, Bayer O, Vandenbroeck S, Salerni L, Hennig A, Obrist D, Mandala M (2021) BPPV: comparison of the SemontPLUS with the Semont maneuver: a prospective randomized trial. Front Neurol 12:652573","journal-title":"Front Neurol"},{"key":"14153_CR57","doi-asserted-by":"publisher","first-page":"798","DOI":"10.1001\/jamaneurol.2023.1408","volume":"80","author":"M Strupp","year":"2023","unstructured":"Strupp M, Mandala M, Vinck AS, Van Breda L, Salerni L, Gerb J, Bayer O, Mavrodiev V, Goldschagg N (2023) The Semont-Plus Maneuver or the Epley Maneuver in Posterior Canal Benign Paroxysmal Positional Vertigo: A Randomized Clinical Study. JAMA Neurol 80:798\u2013804","journal-title":"JAMA Neurol"},{"key":"14153_CR58","doi-asserted-by":"publisher","first-page":"3883","DOI":"10.1007\/s00405-025-09304-0","volume":"282","author":"AR Alashram","year":"2025","unstructured":"Alashram AR (2025) Barbecue roll maneuver for horizontal canal benign paroxysmal positional vertigo: a systematic review of randomized controlled trials. Eur Arch Otorhinolaryngol 282:3883\u20133896","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR59","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3233\/VES-150549","volume":"25","author":"JA Lopez-Escamez","year":"2015","unstructured":"Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandal\u00c3 M, Newman-Toker DE, Strupp M, Suzuki M, Trabalzini F, Bisdorff A (2015) Diagnostic criteria for Meniere\u2019s disease. J Vestib Res 25:1\u20137","journal-title":"J Vestib Res"},{"key":"14153_CR60","doi-asserted-by":"publisher","first-page":"1191","DOI":"10.1097\/MAO.0000000000004644","volume":"46","author":"EGM Vosbeek","year":"2025","unstructured":"Vosbeek EGM, Seelen M, Schermer TR, Terwindt GM, Bruintjes TD (2025) The differences in caloric test and vHIT results between Meniere\u2019s disease and vestibular migraine: a systematic review and meta-analysis. Otol Neurotol 46:1191\u20131201","journal-title":"Otol Neurotol"},{"key":"14153_CR61","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-025-13291-x","volume":"272","author":"D Huppert","year":"2025","unstructured":"Huppert D, Grill E, Becker-Bense S, Zwergal A, Strobl R (2025) Diagnostic challenges in vestibular migraine-clinical differentiation from Meniere\u2019s disease and discrepancies with current classification criteria. J Neurol 272:558","journal-title":"J Neurol"},{"key":"14153_CR62","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1080\/00016489.2025.2450222","volume":"145","author":"P Guo","year":"2025","unstructured":"Guo P, Qin F, Ye L, Li H, Li W (2025) The role of audiological features and horizontal semicircular canal function at various frequencies in vestibular migraine and Meniere\u2019s disease. Acta Otolaryngol 145:115\u2013122","journal-title":"Acta Otolaryngol"},{"key":"14153_CR63","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1639916","volume":"16","author":"VG Yuan","year":"2025","unstructured":"Yuan VG, Xia A, Santa Maria PL (2025) Immunological mechanisms in Meniere\u2019s disease. Front Immunol 16:1639916","journal-title":"Front Immunol"},{"key":"14153_CR64","doi-asserted-by":"publisher","first-page":"485","DOI":"10.1097\/MAO.0000000000004491","volume":"46","author":"TJ Hudson","year":"2025","unstructured":"Hudson TJ, Tan LSH, Phillips V, Muzaffar J, Bance M (2025) Long-term risk of progression from unilateral to bilateral Meniere\u2019s disease: a systematic review and meta-analysis. Otol Neurotol 46:485\u2013493","journal-title":"Otol Neurotol"},{"key":"14153_CR65","doi-asserted-by":"publisher","first-page":"3897","DOI":"10.1007\/s00405-025-09329-5","volume":"282","author":"H Osce","year":"2025","unstructured":"Osce H, Loos E, Huygen A, Desloovere C (2025) Treatment of Meniere\u2019s disease: a scoping review of the current evidence. Eur Arch Otorhinolaryngol 282:3897\u20133923","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR66","doi-asserted-by":"publisher","first-page":"1507","DOI":"10.1007\/s00405-025-09764-4","volume":"283","author":"HG Martinez","year":"2026","unstructured":"Martinez HG, Larrosa F, Sandoval M, Remacha J, Bernal-Sprekelsen M (2026) Intratympanic corticosteroid injection for Meniere\u2019s disease: systematic review of randomized controlled trials. Eur Arch Otorhinolaryngol 283:1507\u20131515","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR67","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1097\/MAO.0000000000004732","volume":"47","author":"E Lindell","year":"2026","unstructured":"Lindell E, Tomanovic T, Dahlin Redfors Y, Engmer Berglin C, Karlberg BM (2026) Latanoprost for treatment of Meniere disease: a randomized, double-blind, placebo-controlled study to determine the efficacy of intratympanic management for symptom control. Otol Neurotol 47:328\u2013335","journal-title":"Otol Neurotol"},{"key":"14153_CR68","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.h6816","volume":"352","author":"C Adrion","year":"2016","unstructured":"Adrion C, Fischer CS, Wagner J, Gurkov R, Mansmann U, Strupp M, Group BS (2016) Efficacy and safety of betahistine treatment in patients with Meniere\u2019s disease: primary results of a long term, multicentre, double blind, randomised, placebo controlled, dose defining trial (BEMED trial). BMJ 352:h6816","journal-title":"BMJ"},{"key":"14153_CR69","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1271640","volume":"14","author":"M Strupp","year":"2023","unstructured":"Strupp M, Churchill GC, Naumann I, Mansmann U, Al Tawil A, Golentsova A, Goldschagg N (2023) Examination of betahistine bioavailability in combination with the monoamine oxidase B inhibitor, selegiline, in humans-a non-randomized, single-sequence, two-period titration, open label single-center phase 1 study (PK-BeST). Front Neurol 14:1271640","journal-title":"Front Neurol"},{"key":"14153_CR70","doi-asserted-by":"publisher","first-page":"306","DOI":"10.1097\/MOO.0000000000001002","volume":"32","author":"JA Lopez-Escamez","year":"2024","unstructured":"Lopez-Escamez JA, Perez-Carpena P (2024) Update on the pathophysiology, diagnosis and management of Meniere\u2019s disease. Curr Opin Otolaryngol Head Neck Surg 32:306\u2013312","journal-title":"Curr Opin Otolaryngol Head Neck Surg"},{"key":"14153_CR71","doi-asserted-by":"publisher","DOI":"10.1002\/lio2.70032","volume":"9","author":"I Yellin","year":"2024","unstructured":"Yellin I, Pathak S, Vambutas A (2024) Effect of betahistine on pro-inflammatory cytokine expression in autoimmune inner ear disease and Meniere\u2019s disease patients. Laryngoscope Investig Otolaryngol 9:e70032","journal-title":"Laryngoscope Investig Otolaryngol"},{"key":"14153_CR72","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3233\/VES-201644","volume":"32","author":"T Lempert","year":"2022","unstructured":"Lempert T, Olesen J, Furman J, Waterston J, Seemungal B, Carey J, Bisdorff A, Versino M, Evers S, Kheradmand A, Newman-Toker D (2022) Vestibular migraine: Diagnostic criteria1. J Vestib Res 32:1\u20136","journal-title":"J Vestib Res"},{"key":"14153_CR73","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000214248","volume":"105","author":"YH Tu","year":"2025","unstructured":"Tu YH, Huang TC, Tzeng YS, Chen SP, Wang SJ, Wang YF (2025) Prevalence, clinical correlates, and functional implications of vestibular symptoms in patients with migraine. Neurology 105:e214248","journal-title":"Neurology"},{"key":"14153_CR74","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1097\/WCO.0000000000001447","volume":"39","author":"MD Villar-Martinez","year":"2026","unstructured":"Villar-Martinez MD, Abdalla A, Goadsby PJ (2026) Vestibular migraine. Clinical and diagnostic challenges, and emerging therapeutic approaches. Curr Opin Neurol 39:42\u201347","journal-title":"Curr Opin Neurol"},{"key":"14153_CR75","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1111\/head.14924","volume":"65","author":"PD Suarez-Barcena","year":"2025","unstructured":"Suarez-Barcena PD, Parra-Perez AM, Martin-Lagos J, Gallego-Martinez A, Lopez-Escamez JA, Perez-Carpena P (2025) Machine learning models and classification algorithms in the diagnosis of vestibular migraine: a systematic review and meta-analysis. Headache 65:695\u2013708","journal-title":"Headache"},{"key":"14153_CR76","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1136\/jnnp-2024-334419","volume":"96","author":"V Kirsch","year":"2025","unstructured":"Kirsch V, Boegle R, Gerb J, Kierig E, Ertl-Wagner BB, Becker-Bense S, Brandt T, Dieterich M (2025) Imaging endolymphatic space of the inner ear in vestibular migraine. J Neurol Neurosurg Psychiatry 96:415\u2013423","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"14153_CR77","doi-asserted-by":"publisher","first-page":"676","DOI":"10.1001\/jamaneurol.2025.1006","volume":"82","author":"JP Staab","year":"2025","unstructured":"Staab JP, Eggers SDZ, Jen JC, LeMahieu AM, Geske JR, Liu H, Hofschulte DR, Gonzalez GR, Neff BA, Shepard NT, McCaslin DL, Baloh RW (2025) Rizatriptan vs placebo for attacks of vestibular migraine: a randomized clinical trial. JAMA Neurol 82:676\u2013686","journal-title":"JAMA Neurol"},{"key":"14153_CR78","doi-asserted-by":"publisher","first-page":"1264","DOI":"10.1111\/head.14835","volume":"64","author":"JD Sharon","year":"2024","unstructured":"Sharon JD, Krauter R, Chae R, Gardi A, Hum M, Allen I, Levin M (2024) A placebo controlled, randomized clinical trial of galcanezumab for vestibular migraine: the INVESTMENT study. Headache 64:1264\u20131272","journal-title":"Headache"},{"key":"14153_CR79","doi-asserted-by":"publisher","DOI":"10.1186\/s12883-025-04490-0","volume":"25","author":"S Vasireddy","year":"2025","unstructured":"Vasireddy S, Biswas S, Kollu R, Krishnan E, Khan MS, C F, Jayakumar A, Acharya R (2025) Comparative effectiveness and safety of preventive treatments for vestibular migraine: a systematic review and network meta-analysis. BMC Neurol 25:513","journal-title":"BMC Neurol"},{"key":"14153_CR80","doi-asserted-by":"publisher","DOI":"10.3390\/jcm14144828","author":"M Cantillo-Martinez","year":"2025","unstructured":"Cantillo-Martinez M, Lorente-Piera J, Manrique-Huarte R, Sanchez-Del-Rio M, Perez-Fernandez N, Chico-Vila C, Moreno-Ajona D, Irimia P (2025) Insights into vestibular migraine: diagnostic challenges, differential spectrum and therapeutic horizons. J Clin Med. https:\/\/doi.org\/10.3390\/jcm14144828","journal-title":"J Clin Med"},{"key":"14153_CR81","doi-asserted-by":"publisher","first-page":"12","DOI":"10.3390\/audiolres13010002","volume":"13","author":"P Tabet","year":"2023","unstructured":"Tabet P, Elblidi A, Saliba I (2023) Vestibular migraine versus M\u00e9niere\u2019s disease: diagnostic utility of electrocochleography. Audiol Res 13:12\u201322","journal-title":"Audiol Res"},{"key":"14153_CR82","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2024.1449261","volume":"15","author":"V Mavrodiev","year":"2024","unstructured":"Mavrodiev V, Strupp M, Vinck AS, van de Berg R, Lehner L (2024) The dissociation between pathological caloric testing and a normal video head impulse test helps differentiate between Meniere\u2019s disease, vestibular migraine, and other vestibular disorders: a confirmatory study in a large cohort of 2,101 patients. Front Neurol 15:1449261","journal-title":"Front Neurol"},{"key":"14153_CR83","doi-asserted-by":"publisher","first-page":"177","DOI":"10.3233\/VES-170619","volume":"27","author":"M Strupp","year":"2017","unstructured":"Strupp M, Kim JS, Murofushi T, Straumann D, Jen JC, Rosengren SM, Della Santina CC, Kingma H (2017) Bilateral vestibulopathy: diagnostic criteria consensus document of the classification committee of the Barany Society. J Vestib Res 27:177\u2013189","journal-title":"J Vestib Res"},{"key":"14153_CR84","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.71025","volume":"15","author":"V Oertle","year":"2025","unstructured":"Oertle V, Becker-Bense S, Brandt T, Dieterich M, Gerb J (2025) Spatial Orientation Impairment in Patients With Bilateral Vestibulopathy and Persistent Postural-Perceptual Dizziness. Brain Behav 15:e71025","journal-title":"Brain Behav"},{"key":"14153_CR85","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1007\/s00405-025-09692-3","volume":"283","author":"FMP Lucieer","year":"2026","unstructured":"Lucieer FMP, Paredis S, Perez-Fornos A, Guinand N, Van Rompaey V, Kingma H, Joore M, van de Berg R (2026) Bilateral vestibulopathy - insight in impact on quality of life and economic burden. Eur Arch Otorhinolaryngol 283:711\u2013718","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"14153_CR86","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1007\/s12311-025-01825-y","volume":"24","author":"M Szymanska Heydel","year":"2025","unstructured":"Szymanska Heydel M, Heindl F, Hartmann A, Borsche M, Traschutz A, Straumann D, Strupp M, Tarnutzer AA (2025) The spectrum of peripheral-vestibular deficits and their change over time in CANVAS\/RFC1-related ataxia systematic review and meta-analysis of quantitative head-impulse testing. Cerebellum 24:67","journal-title":"Cerebellum"},{"key":"14153_CR87","doi-asserted-by":"publisher","first-page":"1887","DOI":"10.1093\/brain\/awad436","volume":"147","author":"R Curro","year":"2024","unstructured":"Curro R, Dominik N, Facchini S, Vegezzi E, Sullivan R, Galassi Deforie V, Fernandez-Eulate G, Traschutz A, Rossi S, Garibaldi M, Kwarciany M, Taroni F, Brusco A, Good JM, Cavalcanti F, Hammans S, Ravenscroft G, Roxburgh RH, Parolin Schnekenberg R, Rugginini B, Abati E, Manini A, Quartesan I, Ghia A, Lopez de Munain A, Manganelli F, Kennerson M, Santorelli FM, Infante J, Marques W, Jokela M, Murphy SM, Mandich P, Fabrizi GM, Briani C, Gosal D, Pareyson D, Ferrari A, Prados F, Yousry T, Khurana V, Kuo SH, Miller J, Troakes C, Jaunmuktane Z, Giunti P, Hartmann A, Basak N, Synofzik M, Stojkovic T, Hadjivassiliou M, Reilly MM, Houlden H, Cortese A (2024) Role of the repeat expansion size in predicting age of onset and severity in RFC1 disease. Brain 147:1887\u20131898","journal-title":"Brain"},{"key":"14153_CR88","first-page":"167","volume":"122","author":"H Erdmann","year":"2025","unstructured":"Erdmann H, Abicht A, Strupp M (2025) A 50-year cough: cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS). Dtsch Arztebl Int 122:167","journal-title":"Dtsch Arztebl Int"},{"key":"14153_CR89","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1136\/jnnp-2023-331490","volume":"95","author":"D Pellerin","year":"2024","unstructured":"Pellerin D, Wilke C, Traschutz A, Nagy S, Curro R, Dicaire MJ, Garcia-Moreno H, Anheim M, Wirth T, Faber J, Timmann D, Depienne C, Rujescu D, Gazulla J, Reilly MM, Giunti P, Brais B, Houlden H, Schols L, Strupp M, Cortese A, Synofzik M (2024) Intronic FGF14 GAA repeat expansions are a common cause of ataxia syndromes with neuropathy and bilateral vestibulopathy. J Neurol Neurosurg Psychiatry 95:175\u2013179","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"14153_CR90","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1056\/NEJMoa2207406","volume":"388","author":"D Pellerin","year":"2023","unstructured":"Pellerin D, Danzi MC, Wilke C, Renaud M, Fazal S, Dicaire MJ, Scriba CK, Ashton C, Yanick C, Beijer D, Rebelo A, Rocca C, Jaunmuktane Z, Sonnen JA, Lariviere R, Genis D, Molina Porcel L, Choquet K, Sakalla R, Provost S, Robertson R, Allard-Chamard X, Tetreault M, Reiling SJ, Nagy S, Nishadham V, Purushottam M, Vengalil S, Bardhan M, Nalini A, Chen Z, Mathieu J, Massie R, Chalk CH, Lafontaine AL, Evoy F, Rioux MF, Ragoussis J, Boycott KM, Dube MP, Duquette A, Houlden H, Ravenscroft G, Laing NG, Lamont PJ, Saporta MA, Schule R, Schols L, La Piana R, Synofzik M, Zuchner S, Brais B (2023) Deep intronic FGF14 GAA repeat expansion in late-onset cerebellar ataxia. N Engl J Med 388:128\u2013141","journal-title":"N Engl J Med"},{"key":"14153_CR91","doi-asserted-by":"publisher","first-page":"e1001","DOI":"10.1212\/WNL.0000000000207553","volume":"101","author":"A Traschutz","year":"2023","unstructured":"Traschutz A, Heindl F, Bilal M, Hartmann AM, Dufke C, Riess O, Zwergal A, Rujescu D, Haack T, Synofzik M, Strupp M (2023) Frequency and phenotype of RFC1 repeat expansions in bilateral vestibulopathy. Neurology 101:e1001\u2013e1013","journal-title":"Neurology"},{"issue":"6","key":"14153_CR92","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1007\/s00415-026-13867-1","volume":"273","author":"D Pellerin","year":"2026","unstructured":"Pellerin D, Heindl F, Traschutz A, Iruzubieta P, Dicaire MJ, Zuchner S, Hartmann AM, Rujescu D, Houlden H, Brais B, Strupp M, Synofzik M (2026) Frequency and phenotype of GAA-FGF14 disease in bilateral vestibulopathy syndromes: insights from repeat expansion carriers, including a case of co-occurrence with RFC1-related CANVAS. J Neurol 273(6):339","journal-title":"J Neurol"},{"issue":"5","key":"14153_CR93","doi-asserted-by":"publisher","first-page":"e200435","DOI":"10.1212\/NXG.0000000000200435","volume":"12","author":"FH Heindl","year":"2026","unstructured":"Heindl FH, Strupp M, Hartmann AM, Zwergal A, Konte B, Rujescu D, Giegling I (2026) Bilateral Vestibulopathy: Strong Association with the ZNF91\/LINC01224 Locus - A Genome-Wide Association Study. Neurology Genetics 12(5):e200435","journal-title":"Neurology Genetics"},{"key":"14153_CR94","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1007\/s00415-025-13150-9","volume":"272","author":"M Dupre","year":"2025","unstructured":"Dupre M, Hermann R, Vidoni L, Quadrio I, Latour P, Subtil F, Froment Tilikete C (2025) Impact of the intronic RFC1 expansion size in CANVAS phenotype: an oculomotor study. J Neurol 272:442","journal-title":"J Neurol"},{"key":"14153_CR95","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2024.105076","volume":"102","author":"D Pellerin","year":"2024","unstructured":"Pellerin D, Heindl F, Wilke C, Danzi MC, Traschutz A, Ashton C, Dicaire MJ, Cuillerier A, Del Gobbo G, Boycott KM, Claassen J, Rujescu D, Hartmann AM, Zuchner S, Brais B, Strupp M, Synofzik M (2024) GAA-FGF14 disease: defining its frequency, molecular basis, and 4-aminopyridine response in a large downbeat nystagmus cohort. EBioMedicine 102:105076","journal-title":"EBioMedicine"},{"key":"14153_CR96","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1007\/s12311-025-01906-y","volume":"24","author":"LE Ariello","year":"2025","unstructured":"Ariello LE, Gold DR, Mu W, Schubert MC, Allen C, Paul A, Rastall DPW (2025) Ocular motor and vestibular profile in spinocerebellar ataxia type 27B: toward a practical bedside diagnostic framework. Cerebellum 24:149","journal-title":"Cerebellum"},{"key":"14153_CR97","doi-asserted-by":"publisher","first-page":"1023","DOI":"10.1007\/s00415-023-12050-0","volume":"271","author":"M Borsche","year":"2024","unstructured":"Borsche M, Thomsen M, Szmulewicz DJ, Lubbers B, Hinrichs F, Lockhart PJ, Lohmann K, Helmchen C, Bruggemann N (2024) Bilateral vestibulopathy in RFC1-positive CANVAS is distinctly different compared to FGF14-linked spinocerebellar ataxia 27B. J Neurol 271:1023\u20131027","journal-title":"J Neurol"},{"key":"14153_CR98","doi-asserted-by":"publisher","first-page":"679","DOI":"10.1007\/s00415-025-13433-1","volume":"272","author":"LE Ariello","year":"2025","unstructured":"Ariello LE, Gold DR, Millar JL, Schubert MC, Mu W, Rosenthal L, Rastall DPW (2025) Video head impulse test differentiates RFC1-CANVAS, FGF14-SCA27B, and idiopathic late-onset cerebellar ataxias. J Neurol 272:679","journal-title":"J Neurol"},{"key":"14153_CR99","doi-asserted-by":"publisher","first-page":"1408","DOI":"10.1007\/s00415-023-12085-3","volume":"271","author":"M Wuehr","year":"2024","unstructured":"Wuehr M, Eder J, Kellerer S, Amberger T, Jahn K (2024) Mechanisms underlying treatment effects of vestibular noise stimulation on postural instability in patients with bilateral vestibulopathy. J Neurol 271:1408\u20131415","journal-title":"J Neurol"},{"key":"14153_CR100","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0301032","volume":"19","author":"BL Vermorken","year":"2024","unstructured":"Vermorken BL, Volpe B, van Boxel SCJ, Stultiens JJA, van Hoof M, Marcellis R, Loos E, van Soest A, McCrum C, Meijer K, Guinand N, Perez Fornos A, van Rompaey V, Devocht E, van de Berg R (2024) The VertiGO! trial protocol: a prospective, single-center, patient-blinded study to evaluate efficacy and safety of prolonged daily stimulation with a multichannel vestibulocochlear implant prototype in bilateral vestibulopathy patients. PLoS ONE 19:e0301032","journal-title":"PLoS ONE"},{"issue":"2","key":"14153_CR101","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1007\/s12311-026-01982-8","volume":"25","author":"F Heindl","year":"2026","unstructured":"Heindl F, Traschutz A, Synofzik M, Wirth T, Anheim M, Tarnutzer AA, Hartmann AM, Rujescu D, Giegling I, Feil K, Adrion C, Strupp M (2026) RFC1-related disorders: A case series of 4-aminopyridine and acetyl-DL-leucine treatment. Cerebellum 25(2):50","journal-title":"Cerebellum"},{"key":"14153_CR102","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1055\/a-2192-7278","volume":"103","author":"K Feil","year":"2024","unstructured":"Feil K, Rattay TW, Adeyemi AK, Goldschagg N, Strupp ML (2024) What\u2019s behind cerebellar dizziness? - News on diagnosis and therapy. Laryngorhinootologie 103:337\u2013343","journal-title":"Laryngorhinootologie"},{"key":"14153_CR103","doi-asserted-by":"publisher","first-page":"636","DOI":"10.1007\/s00415-025-13387-4","volume":"272","author":"P Iruzubieta","year":"2025","unstructured":"Iruzubieta P, Pellerin D, Ashton C, Villa F, Renaud M, Dicaire MJ, Danzi MC, Aldecoa M, Mathieu J, Massie R, Chalk CH, Lafontaine AL, Evoy F, Rioux MF, Brisson JD, Boycott KM, Houlden H, Synofzik M, La Piana R, Zuchner S, Duquette A, Brais B (2025) Delineating the pathogenic threshold and phenotypic spectrum of SCA27B: findings from a large French-Canadian cohort. J Neurol 272:636","journal-title":"J Neurol"},{"key":"14153_CR104","doi-asserted-by":"publisher","first-page":"7665","DOI":"10.1038\/s41467-024-52148-1","volume":"15","author":"L Mohren","year":"2024","unstructured":"Mohren L, Erdlenbruch F, Leitao E, Kilpert F, Hones GS, Kaya S, Schroder C, Thieme A, Sturm M, Park J, Schluter A, Ruiz M, Morales de la Prida M, Casasnovas C, Becker K, Roggenbuck U, Pechlivanis S, Kaiser FJ, Synofzik M, Wirth T, Anheim M, Haack TB, Lockhart PJ, Jockel KH, Pujol A, Klebe S, Timmann D, Depienne C (2024) Identification and characterisation of pathogenic and non-pathogenic FGF14 repeat expansions. Nat Commun 15:7665","journal-title":"Nat Commun"},{"key":"14153_CR105","doi-asserted-by":"publisher","first-page":"1092","DOI":"10.1002\/ana.27060","volume":"96","author":"W Abou Chaar","year":"2024","unstructured":"Abou Chaar W, Eranki AN, Stevens HA, Watson SL, Wong DY, Avila VS, Delfeld M, Gary AJ, Tawde S, Triebold M, Cherchi M, Xie T, Lockhart PJ, Bahlo M, Pellerin D, Dicaire MJ, Danzi M, Zuchner S, Brais BC, Perlman S, Burmeister M, Paulson H, Srinivasan S, Schut L, Bower M, Bushara K, Liao C, Shakkottai VG, Collins J, Clark HB, Das S, Fogel BL, Gomez CM (2024) Clinical, radiological and pathological features of a large American cohort of spinocerebellar ataxia (SCA27B). Ann Neurol 96:1092\u20131103","journal-title":"Ann Neurol"},{"key":"14153_CR106","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1007\/s12311-025-01882-3","volume":"24","author":"LA Rettenmaier","year":"2025","unstructured":"Rettenmaier LA, Chen JYH, MacMore J, Gupta AS, Lin CC, Stephen CD, Pellerin D, Brais B, Schmahmann JD (2025) Spinocerebellar ataxia type 27B can be suspected based on clinical phenotype: the Massachusetts General Hospital Ataxia Center experience. Cerebellum 24:133","journal-title":"Cerebellum"},{"key":"14153_CR107","doi-asserted-by":"publisher","first-page":"4144","DOI":"10.1093\/brain\/awad157","volume":"146","author":"C Wilke","year":"2023","unstructured":"Wilke C, Pellerin D, Mengel D, Traschutz A, Danzi MC, Dicaire MJ, Neumann M, Lerche H, Bender B, Houlden H, group RFCs, Zuchner S, Schols L, Brais B, Synofzik M (2023) GAA-FGF14 ataxia (SCA27B): phenotypic profile, natural history progression and 4-aminopyridine treatment response. Brain 146:4144\u20134157","journal-title":"Brain"},{"key":"14153_CR108","doi-asserted-by":"publisher","first-page":"2886","DOI":"10.1007\/s00415-024-12229-z","volume":"271","author":"D Pellerin","year":"2024","unstructured":"Pellerin D, Heindl F, Traschutz A, Rujescu D, Hartmann AM, Brais B, Houlden H, Dufke C, Riess O, Haack T, Strupp M, Synofzik M (2024) RFC1 repeat expansions in downbeat nystagmus syndromes: frequency and phenotypic profile. J Neurol 271:2886\u20132892","journal-title":"J Neurol"},{"key":"14153_CR109","doi-asserted-by":"publisher","first-page":"2262","DOI":"10.1002\/mds.30328","volume":"40","author":"E Indelicato","year":"2025","unstructured":"Indelicato E, Fleszar Z, Pellerin D, Nachbauer W, Zuchner S, Traschutz A, Amprosi M, Schols L, Haack TB, Brais B, Boesch S, Synofzik M (2025) GAA-FGF14 expansions and CACNA1A variants: phenotypic overlap and diagnostic implications. Mov Disord 40:2262\u20132268","journal-title":"Mov Disord"},{"key":"14153_CR110","doi-asserted-by":"publisher","first-page":"e438","DOI":"10.1212\/CPJ.0000000000001017","volume":"11","author":"C Muth","year":"2021","unstructured":"Muth C, Teufel J, Schols L, Synofzik M, Franke C, Timmann D, Mansmann U, Strupp M (2021) Fampridine and acetazolamide in EA2 and related familial EA: a prospective randomized placebo-controlled trial. Neurol Clin Pract 11:e438\u2013e446","journal-title":"Neurol Clin Pract"},{"key":"14153_CR111","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1007\/s00415-025-12941-4","volume":"272","author":"L Verriello","year":"2025","unstructured":"Verriello L, Pez S, Pauletto G, Valente M (2025) Efficacy and safety of 4-aminopyridine in episodic ataxia type 2: a case series. J Neurol 272:205","journal-title":"J Neurol"},{"key":"14153_CR112","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1056\/NEJMoa2310151","volume":"390","author":"T Bremova-Ertl","year":"2024","unstructured":"Bremova-Ertl T, Ramaswami U, Brands M, Foltan T, Gautschi M, Gissen P, Gowing F, Hahn A, Jones S, Kay R, Kolnikova M, Arash-Kaps L, Marquardt T, Mengel E, Park JH, Reichmannova S, Schneider SA, Sivananthan S, Walterfang M, Wibawa P, Strupp M, Martakis K (2024) Trial of N-acetyl-L-leucine in Niemann-Pick disease type C. N Engl J Med 390:421\u2013431","journal-title":"N Engl J Med"},{"key":"14153_CR113","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1016\/S1474-4422(26)00158-4","volume":"25","author":"K Martakis","year":"2026","unstructured":"Martakis K, Bremova-Ertl T, Bolton C, Foltan T, Del Mar Garcia Romero M, Gautschi M, Han V, Hahn A, Kolnikova M, Panagioti O, Perlman S, Prasad M, Schmahmann J, Skorvanek M, Thakur N, Thiel M, Hoche F (2026) Safety and efficacy of levacetylleucine in ataxia-telangiectasia: a phase 3, randomised, double-blind, placebo-controlled crossover trial. Lancet Neurol 25:633\u2013644","journal-title":"Lancet Neurol"},{"key":"14153_CR114","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1016\/j.ejpn.2024.12.006","volume":"54","author":"K Martakis","year":"2025","unstructured":"Martakis K, Giorgi M, Spanou M, Neubauer BA, Dinopoulos A, Hahn A (2025) N-acetyl-leucine in progressive CACNA1A ataxia: a case series. Eur J Paediatr Neurol 54:64\u201367","journal-title":"Eur J Paediatr Neurol"},{"key":"14153_CR115","doi-asserted-by":"publisher","first-page":"7619","DOI":"10.1038\/s41467-024-51502-7","volume":"15","author":"WH Oertel","year":"2024","unstructured":"Oertel WH, Janzen A, Henrich MT, Geibl FF, Sittig E, Meles SK, Carli G, Leenders K, Booij J, Surmeier DJ, Timmermann L, Strupp M (2024) Acetyl-DL-leucine in two individuals with REM sleep behavior disorder improves symptoms, reverses loss of striatal dopamine-transporter binding and stabilizes pathological metabolic brain pattern-case reports. Nat Commun 15:7619","journal-title":"Nat Commun"},{"key":"14153_CR116","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000213589","volume":"105","author":"MC Patterson","year":"2025","unstructured":"Patterson MC, Ramaswami U, Donald A, Foltan T, Gautschi M, Gissen P, Hahn A, Jones SA, Kay R, Kolnikova M, Park J, Reichmannova S, Walterfang M, Wibawa P, Rohrbach M, Martakis K, Bremova-Ertl T (2025) Disease-modifying, neuroprotective effect of N-acetyl-L-leucine in adult and pediatric patients with Niemann-Pick disease type C. Neurology 105:e213589","journal-title":"Neurology"},{"key":"14153_CR117","volume":"16","author":"J Madrigal","year":"2024","unstructured":"Madrigal J, Herron-Arango AF, Bedoya MJ, Cordero Chen J, Castillo-Bustamante M (2024) Persistent challenges: a comprehensive review of persistent postural-perceptual dizziness, controversies, and clinical complexities. Cureus 16:e60911","journal-title":"Cureus"},{"key":"14153_CR118","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1016\/j.anl.2023.12.008","volume":"51","author":"C Yagi","year":"2024","unstructured":"Yagi C, Kimura A, Horii A (2024) Persistent postural-perceptual dizziness: a functional neuro-otologic disorder. Auris Nasus Larynx 51:588\u2013598","journal-title":"Auris Nasus Larynx"},{"key":"14153_CR119","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1136\/practneurol-2017-001809","volume":"18","author":"S Popkirov","year":"2018","unstructured":"Popkirov S, Staab JP, Stone J (2018) Persistent postural-perceptual dizziness (PPPD): a common, characteristic and treatable cause of chronic dizziness. Pract Neurol 18:5\u201313","journal-title":"Pract Neurol"},{"key":"14153_CR120","doi-asserted-by":"publisher","first-page":"589","DOI":"10.3390\/audiolres12060058","volume":"12","author":"V Gambacorta","year":"2022","unstructured":"Gambacorta V, D\u2019Orazio A, Pugliese V, Di Giovanni A, Ricci G, Faralli M (2022) Persistent Postural Perceptual Dizziness in Episodic Vestibular Disorders. Audiol Res 12:589\u2013595","journal-title":"Audiol Res"},{"key":"14153_CR121","doi-asserted-by":"publisher","DOI":"10.1002\/brb3.70229","volume":"15","author":"C Qin","year":"2025","unstructured":"Qin C, Zhang R, Yan Z (2025) Research progress on the potential pathogenesis of persistent postural-perceptual dizziness. Brain Behav 15:e70229","journal-title":"Brain Behav"},{"key":"14153_CR122","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1007\/s00415-020-10151-8","volume":"267","author":"J Penkava","year":"2020","unstructured":"Penkava J, Bardins S, Brandt T, Wuehr M, Huppert D (2020) Spontaneous visual exploration during locomotion in patients with phobic postural vertigo. J Neurol 267:223\u2013230","journal-title":"J Neurol"},{"key":"14153_CR123","doi-asserted-by":"publisher","DOI":"10.1111\/ene.70339","volume":"32","author":"A Sereda","year":"2025","unstructured":"Sereda A, Lam JC, Hazar AM, Ellmers T, Golding J, Kaski D (2025) Psychological variables mediate symptoms in persistent postural-perceptual dizziness (PPPD): a cross-sectional self-report study. Eur J Neurol 32:e70339","journal-title":"Eur J Neurol"},{"key":"14153_CR124","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1007\/s00415-024-12782-7","volume":"272","author":"JA Edlow","year":"2025","unstructured":"Edlow JA, Tarnutzer AA (2025) Intravenous thrombolysis in patients with acute dizziness or imbalance and suspected ischemic stroke-systematic review. J Neurol 272:91","journal-title":"J Neurol"},{"key":"14153_CR125","doi-asserted-by":"publisher","first-page":"1561202","DOI":"10.3389\/fneur.2025.1561202","volume":"16","author":"SRH De Schryver","year":"2025","unstructured":"De Schryver SRH, de Jonge M, van Luxemburg RP, Geraedts VJ, De Schryver E (2025) Prehospital patients with acute isolated vertigo and imbalance referred for cerebral thrombolysis. Front Neurol 16:1561202","journal-title":"Front Neurol"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-14153-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-026-14153-w","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-026-14153-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T16:01:44Z","timestamp":1791734504000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-026-14153-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,11]]},"references-count":125,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2026,11]]}},"alternative-id":["14153"],"URL":"https:\/\/doi.org\/10.1007\/s00415-026-14153-w","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,11]]},"assertion":[{"value":"23 July 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 September 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 September 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 October 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A. Tarnutzer is in the editorial board of the Journal of Neurological Sciences, Research in Vestibular Science, Brain Sciences, and Clinical and translational neurosciences. He is an associate editor for Frontiers in Neurology. He has received consultant fees from AstraZenea, Medtronic, Mitsubishi Tanabe Pharma, Teva Pharma, Neuraxpharma, Angelini Pharma, and Schwabe Pharma. He has received speaker\u2019s honoraria from Teva Pharma and Schwabe Pharma. He has received travel funds from Balcare. He acts as an unpaid consultant for Vertify. M. Strupp is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology and Section Editor of F1000. He has received speaker\u2019s honoraria from Abbott, Auris Medical, Biogen, Eisai, Gr\u00fcnenthal, GSK, Henning Pharma, Interacoustics, J&J, MSD, NeuroUpdate, Otometrics, Pierre-Fabre, TEVA, UCB, and Viatris. He received support for clinical studies from Decibel, U.S.A., Cure within Reach, U.S.A. and Heel, Germany. He distributes \u201cM-glasses\u201d and \u201cPositional vertigo App\u201d. He acts as a consultant for Abbott, AurisMedical, Bulbitec, Heel, Sensorion, Vifor and Vertify. He is a scientific founder, investor and share-holder of IntraBio L. Lehner has no competing interests to declare.","order":1,"name":"Ethics","label":"Conflicts of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Being a critical review of literature, no ethical approval or consent was necessary.","order":2,"name":"Ethics","label":"Ethical approval, consent","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"657"}}