Light Cupula: To Be Or Not to Be?

Summary

Benign paroxysmal positional vertigo (BPPV) represents the most common form of positional vertigo. It is caused by dislodged otoconia that freely float in the semicircular canals (canalolithiasis) or attach to the cupula (cupulolithiasis). A cupulolithiasis-type (or a heavy cupula-type) of BPPV implicating the lateral semicircular canal (LSCC) exhibits persistent ageotropic direction-changing positional nystagmus (DCPN) in a head-roll test. However, in some cases, unlike any type of BPPV, persistent geotropic DCPN cannot be explained by any mechanisms of BPPV, and don’t fit the current classifications. Recently, the notion of light cupula has been introduced to refer to the persistent geotropic DCPN. In this study, we looked at the clinical features of light cuplula and discussed the possible mechanisms and therapeutic strategies of the condition. The notion of light cupula is a helpful addition to the theory of peripheral positional vertigo and nystagmus.

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References

  1. 1

    Parnes LS, Agrawal SK, Atlas J. Diagnosis and management of benign paroxysmal positional vertigo (BPPV). Can Med Assoc J, 2003, 169(7):681–693

    Google Scholar 

  2. 2

    Hall SF, Ruby RR, McClure JA. The mechanics of benign paroxysmal vertigo. J Otolaryngol, 1979, 8(2):151–158

    CAS  PubMed  Google Scholar 

  3. 3

    Schuknecht HF. Positional vertigo: clinical and experimental observations. Trans Am Acad Ophthalmol Otolaryngol, 1962, 66:319–332

    CAS  PubMed  Google Scholar 

  4. 4

    Seo T, Shiraishi K, Kobayashi T, et al. Clinical course of persistent geotropic direction-changing positional nystagmus with neutral position-Light cupula. Acta Oto-laryngol, 2016, 136(1):34–37

    Google Scholar 

  5. 5

    Shigeno K, Oku R, Takahashi H, et al. Static direction-changing horizontal positional nystagmus of peripheral origin. J Vest Res, 2001–2002,11:243–244

    Google Scholar 

  6. 6

    Nuti D, Masini M, Mandala M. Benign paroxysmal positional vertigo and its variants. Handb Clin Neurol, 2016, 137:241–256

    CAS  PubMed  Google Scholar 

  7. 7

    Kim CH, Hong SM. Is the modified cupulolith repositioning maneuver effective for treatment of persistent geotropic direction-changing positional nystagmus? Eur Arch Otorhinolaryngol, 2018,275: 7

    Google Scholar 

  8. 8

    Shim DB, Ko KM, Lee JH, et al. Natural history of horizontal canal benign paroxysmal positional vertigo is truly short. J Neurol, 2015, 262(1):74–80

    PubMed  Google Scholar 

  9. 9

    Ichijo H. Recurrence in patients with benign paroxysmal positional vertigo of the lateral semicircular canal. Auris Nasus Larynx, 2019, 19:30362–30371

    Google Scholar 

  10. 10

    Aschan G, Bergstedt M, Goldberg L, et al. Positional nystagmus in man during and after alcohol intoxication. Q J Stud Alcohol, 1956, 17(3):381–405

    CAS  PubMed  Google Scholar 

  11. 11

    Tomanovic T, Bergenius J. Can the nystagmus pattern in patients with a’ light cupula’ be reproduced in hemilabyrinthectomized subjects during positional alcohol nystagmus 1?. Acta Oto-laryngol, 2011,131(9):929–936

    Google Scholar 

  12. 12

    Hiruma K, Numata T. Positional nystagmus showing neutral points. ORL J Otorhinolaryngol Relat Spec, 2004, 66(1):46–50

    PubMed  Google Scholar 

  13. 13

    Kim CH, Shin JE, Kim YW. A new method for evaluating lateral semicircular canal cupulopathy. Laryngoscope, 2015, 125(8):1921–1925

    PubMed  Google Scholar 

  14. 14

    Ichijo H. Neutral position of persistent direction-changing positional nystagmus. Eur Arch Otorhino-laryngol, 2016, 273(2):311–316

    Google Scholar 

  15. 15

    Kim YW, Shin JE, Lee YS, et al. Persistent Positional Vertigo in a Patient with Sudden Sensorineural Hearing Loss: A Case Report. J Audiol Otol, 2015, 19(2):104–107

    PubMed  PubMed Central  Google Scholar 

  16. 16

    Ichijo H. Caloric testing in patients with heavy or light cupula of the lateral semicircular canal. Laryngoscope Investig Otolaryngol, 2016, 1(6):163–168

    PubMed  PubMed Central  Google Scholar 

  17. 17

    Seo T, Saito K, Doi K. Intractable persistent direction-changing geotropic nystagmus improved by lateral semicircular canal plugging. Case Rep Otolaryngol, 2015, 2015:192764

    PubMed  PubMed Central  Google Scholar 

  18. 18

    Imai T, Matsuda K, Takeda N, et al. Light cupula: the pathophysiological basis of persistent geotropic positional nystagmus. BMJ Open, 2015, 5(1):e006607

    PubMed  PubMed Central  Google Scholar 

  19. 19

    Kim MB, Hong SM, Choi H, et al. The Light Cupula: An Emerging New Concept for Positional Vertigo. J Audiol Otol, 2018, 22(1):1–5

    Google Scholar 

  20. 20

    Choi JY, Lee ES, Kim HJ, et al. Persistent geotropic positional nystagmus after meningitis: Evidence for light cupula. J Neurol Sci, 2017, 379:279–280

    Google Scholar 

  21. 21

    Kim CH, Kim MB, Ban JH. Persistent geotropic direction-changing positional nystagmus with a null plane: the light cupula. Laryngoscope, 2014, 124(1):E15–E19

    Google Scholar 

  22. 22

    Hiruma K, Numata T, Mitsuhashi T, et al. Two types of direction-changing positional nystagmus with neutral points. Auris Nasus Larynx, 2011, 38(1):46–51

    PubMed  Google Scholar 

  23. 23

    Park JS, Kim SY, Kim MB. Effect of intratympanic steroid injection in light cupula. Acta Oto-laryngol, 2018, 138(9):769–774

    CAS  Google Scholar 

  24. 24

    Ban JH, Kim MB, Hong SM. Immediate and ShortTerm Therapeutic Results Between Direction-Changing Positional Nystagmus with Short- and Long-Duration Groups. Ear Hear, 2016, 37(2):243–246

    PubMed  Google Scholar 

  25. 25

    Kim CH, Shin JE, Shin DH, et al. “Light cupula” involving all three semicircular canals: A frequently misdiagnosed disorder. Med Hypotheses, 2014, 83(5):541–544

    PubMed  Google Scholar 

  26. 26

    Argaet EC, Bradshaw AP, Welgampola MS. Benign positional vertigo, its diagnosis, treatment and mimics. Clin Neurophysiol Pract, 2019, 4:97–111

    CAS  PubMed  PubMed Central  Google Scholar 

  27. 27

    Spoendlin H. [Structural properties of the vestibular receptors]. Schweiz Arch Neurol Neurochir Psychiatr, 1965, 96(2):219–230

    CAS  PubMed  Google Scholar 

  28. 28

    Tang X, Huang Q, Chen L, et al. Clinical Findings in Patients With Persistent Positional Nystagmus: The Designation of “Heavy and Light Cupula”. Front Neurol, 2019, 10:326

    PubMed  PubMed Central  Google Scholar 

  29. 29

    Money KE, Johnson WH, Corlett BM. Role of semicircular canals in positional alcohol nystagmus. Am J Physiol, 1965, 208:1065–1070

    CAS  PubMed  Google Scholar 

  30. 30

    Gernandt B. The effect of the centrifugal force upon the nerve discharge from the horizontal canal. Acta Physiol Scand, 1950, 21(1):61–72

    CAS  PubMed  Google Scholar 

  31. 31

    Sugiura M, Naganawa S, Teranishi M, et al. Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings in patients with sudden sensorineural hearing loss. Laryngoscope, 2006, 116(8):1451–1454

    PubMed  Google Scholar 

  32. 32

    Naganawa S, Ishihara S, Iwano S, et al. Detection of presumed hemorrhage in the ampullar endolymph of the semicircular canal: a case report. Magn Reson Med Sci, 2009, 8(4):187–191

    PubMed  Google Scholar 

  33. 33

    Ichijo H. Persistent direction-changing geotropic positional nystagmus. Eur Arch Otorhinolaryngol, 2012, 269(3):747–751

    PubMed  Google Scholar 

  34. 34

    Kim CH, Pham NC. Density difference between perilymph and endolymph: A new hypothesis for light cupula phenomenon. Med Hypotheses, 2019, 123:55–59

    PubMed  Google Scholar 

  35. 35

    Iversen MM, Rabbitt RD. Wave Mechanics of the Vestibular Semicircular Canals. Biophys J, 2017,113(5): 1133–1149

    CAS  PubMed  PubMed Central  Google Scholar 

  36. 36

    Shigeno K, Egami T, Sasano T. Experimental study of nystagmus induced by injecting various solutions into the middle ear cavity. Acta Oto-laryngol, 1989, 108(1–2):31–37

    CAS  Google Scholar 

  37. 37

    Radtke A, von Brevern M, Neuhauser H, et al. Vestibular migraine: long-term follow-up of clinical symptoms and vestibulo-cochlear findings. Neurology, 2012, 79(15):1607–1614

    PubMed  Google Scholar 

  38. 38

    Polensek SH, Tusa RJ. Nystagmus during attacks of vestibular migraine: an aid in diagnosis. Audiol Neurootol, 2010, 15(4):241–246

    PubMed  Google Scholar 

  39. 39

    Tomanovic T, Bergenius J. Vestibular findings in patients with persistent geotropic positional nystagmus: the “light cupula” phenomenon. Acta Oto-laryngoL 2014,134(9):904–914

    Google Scholar 

  40. 40

    Espinosa-Sanchez JM, Lopez-Escamez JA. New insights into pathophysiology of vestibular migraine. Front Neurol, 2015, 6:12

    PubMed  PubMed Central  Google Scholar 

  41. 41

    Shin JH, Kim YK, Kim HJ, et al. Altered brain metabolism in vestibular migraine: comparison of interictal and ictal findings. Cephalalgia, 2014, 34(1):58–67

    PubMed  Google Scholar 

  42. 42

    Vass Z, Dai CF, Steyger PS, et al. Co-localization of the vanilloid capsaicin receptor and substance P in sensory nerve fibers innervating cochlear and vertebro-basilar arteries. Neuroscience, 2004, 124(4):919–927

    CAS  PubMed  PubMed Central  Google Scholar 

  43. 43

    Lee HJ, Kim ES, Kim M, et al. Isolated horizontal positional nystagmus from a posterior fossa lesion. Ann Neurol, 2014, 76(6):905–910

    PubMed  Google Scholar 

  44. 44

    Yang TH, Oh SY. Geotropic central paroxysmal positional nystagmus in a patient with human immunodeficiency virus encephalopathy. J Neuroophthalmol, 2014, 34(2):159–161

    PubMed  Google Scholar 

  45. 45

    Choi SY, Jang JY, Oh EH, et al. Persistent geotropic positional nystagmus in unilateral cerebellar lesions. Neurology, 2018,91(11): e1053–e1057

    PubMed  PubMed Central  Google Scholar 

  46. 46

    Kim CH, Choi JM, Jung HV, et al. Sudden sensorineural hearing loss with simultaneous positional vertigo showing persistent geotropic direction-changing positional nystagmus. Otol Neurotol, 2014, 35(9):1626–1632

    PubMed  Google Scholar 

  47. 47

    Kim CH, Yang YS, Im D, et al. Nystagmus in patients with unilateral acute otitis media complicated by serous labyrinthitis. Acta Oto-laryngol, 2016, 136(6):559–563

    Google Scholar 

  48. 48

    Kim CH, Choi JW, Han KJ, et al. Direction-fixed and Direction-changing Positional Nystagmus in Ramsay Hunt Syndrome. Otol Neurotol, 2018,39(3): e209–e213

    PubMed  Google Scholar 

  49. 49

    Aschan G, Stahle J. Nystagmus in Meniere’s disease during attacks; a nystagmographical study. Acta Oto-laryngol, 1957, 47(3):189–201

    CAS  Google Scholar 

  50. 50

    Shin JE, Jeong KH, Ahn SH, et al. Conversion between geotropic and apogeotropic persistent direction-changing positional nystagmus. Acta Oto-laryngol, 2015, 135(12):1238–1244

    Google Scholar 

  51. 51

    Neugebauer H, Adrion C, Glaser M, et al. Long-term changes of central ocular motor signs in patients with vestibular migraine. Eur Neurol, 2013, 69(2):102–107

    PubMed  Google Scholar 

  52. 52

    El-Badry MM, Samy H, Kabel AM, et al. Clinical criteria of positional vertical nystagmus in vestibular migraine. Acta Oto-laryngol, 2017, 137(7):720–722

    CAS  Google Scholar 

  53. 53

    Lavigne P, Lavigne F, Saliba I. Intratympanic corticosteroids injections: a systematic review of literature. Eur Arch Otorhinolaryngol, 2016, 273(9):2271–2278

    PubMed  Google Scholar 

  54. 54

    Fukushima M, Kitahara T, Fuse Y, et al. Changes in aquaporin expression in the inner ear of the rat after i.p. injection of steroids. Acta Otolaryngol Suppl, 2004, (553):13–18

  55. 55

    Fukushima M, Kitahara T, Uno Y, et al. Effects of intratympanic injection of steroids on changes in rat inner ear aquaporin expression. Acta Oto-laryngol, 2002, 122(6):600–606

    CAS  Google Scholar 

  56. 56

    Parnes LS, McClure JA. Posterior semicircular canal occlusion for intractable benign paroxysmal positional vertigo. Ann Otol Rhinol Laryngol, 1990,99(5 Pt 1):330–334

    CAS  PubMed  Google Scholar 

  57. 57

    Zhang D, Lv Y, Han Y, et al. Long-term outcomes of triple semicircular canal plugging for the treatment of intractable Meniere’s disease: A single center experience of 361 cases. J Vestib Res, 2019, 29(6):315–322

    PubMed  Google Scholar 

  58. 58

    Cha WW, Song K, Lee HY. Persistent Geotropic Direction-Changing Positional Nystagmus Treated With Transcutaneous Vagus Nerve Stimulation. Brain Stimul, 2016, 9(3):469–470

    PubMed  Google Scholar 

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Correspondence to Su-lin Zhang or Wei-jia Kong.

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The authors declare that they have no conflict of interests.

Additional information

The study was supported by the National Twelfth-Five Year Research Program of China (No. 2012BAI12B02) and the National Natural Science Foundation of China (No. 81873701).

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Zhang, Sl., Tian, E., Xu, Wc. et al. Light Cupula: To Be Or Not to Be?. CURR MED SCI 40, 455–462 (2020). https://doi.org/10.1007/s11596-020-2199-8

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Key words

  • positional vertigo
  • direction-changing positional nystagmus
  • light cupula
  • head-roll test
  • null plane
  • lateral semicircular canal
  • ampulla