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The Tullio phenomenon: a neurologically neglected presentation

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Abstract

The Tullio phenomenon refers to sound-induced disequilibrium or oscillopsia. Patients with this condition frequently present to neurologists, many of whom are unfamiliar with the condition and its diagnostic criteria. Indeed, due to the unusual nature of the symptoms patients are often misdiagnosed as having psychiatric disturbances. Tullio patients describe disequilibrium, auditory and visual symptoms, which are recurrent, brief, and often triggered by loud noises or middle ear pressure changes, e.g. the Valsalva manoeuvre. Many cases are associated with superior semicircular canal dehiscence (SCCD). Early work suggested that the presence of sound-induced torsional eye movements and visual field tilts were consequent upon a utricular-mediated ocular tilt reaction. However, more recent evidence from imaging and oculographic research, as well as data from our patient series indicates that these ocular abnormalities are usually the result of superior semicircular canal stimulation. The clinical history and a focussed examination are often sufficient to make the diagnosis, which can be confirmed with high resolution CT imaging of the temporal bones. In some patients, surgical occlusion or resurfacing of the affected canal can ameliorate symptoms and signs. The aim of this paper is two-fold: Firstly, to review the clinical features of the Tullio phenomenon, and secondly, to highlight our own observations in three cases with a new clinical syndrome consisting of Tullio’s phenomenon with bilateral vestibular failure, a pure horizontal nystagmus in response to sound, and no evidence of canal dehiscence.

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References

  1. Tullio P (1929) Das Ohr und die Entstehung der Sprache und Schrift. Urban and Schwarzenberg, Berlin

    Google Scholar 

  2. van Eunen AJH, Huizing HC, Huizinga E (1943) Die Tulliosche Reaktion in Zusammenhang mit der Funktion des Mittelohres. Acta Otolaryngol 31(3–4):265–339

    Article  Google Scholar 

  3. Benjamins CE (1938) Les reactions acoustiques de Tullio chez l’homme. Acta Otolaryngol (Stockh) 26:213–216

    Article  Google Scholar 

  4. Cawthorne T (1948) Surgical treatment of Ménière’s disease. Br Med J 2(4585):914–915

    Article  Google Scholar 

  5. Dickson EDD, Chadwick DL (1951) Observations on disturbances of equilibrium and other symptoms induced by Jet-engine noise. J Laryngol Otol 65:154–165

    PubMed  CAS  Google Scholar 

  6. Brandt T, Dieterich M, Fries W (1988) Otolithic Tullio phenomenon typically presents as paroxysmal ocular tilt reaction. Adv Otorhinolaryngol 42:153–156

    PubMed  CAS  Google Scholar 

  7. Minor LB, Solomon D, Zinreich JS, Zee DS (1998) Sound and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal. Arch Otolaryngol Head Neck Surg 124:249–258

    PubMed  CAS  Google Scholar 

  8. Aw ST, Todd MJ, Aw GE, Magnussen JS, Curthoys IS, Halmagyi GM (2006) Click-evoked vestibulo-ocular reflex: stimulus-response properties in superior canal dehiscence. Neurology 66(7):1079–1087

    Article  PubMed  CAS  Google Scholar 

  9. Minor LB (2005) Clinical manifestations of superior semicircular canal dehiscence. Laryngoscope 115(10):1717–1727

    Article  PubMed  Google Scholar 

  10. Yuen HW, Eikelboom RH, Atlas MD (2009) Auditory manifestations of superior semicircular canal dehiscence. Otol Neurotol 30(3):280–285

    Article  PubMed  Google Scholar 

  11. Albuquerque W, Bronstein AM (2004) “Doctor, I can hear my eyes”: report of two cases with different mechanisms. J Neurol Neurosurg Psychiatry 75(9):1363–1364

    Article  PubMed  CAS  Google Scholar 

  12. Hamilton WF, Woodbury RA, Harper HT (1936) Physiologic relationships between intrathoracic, intraspinal and arterial pressures. JAMA 107:853–856

    Article  Google Scholar 

  13. Tilikete C, Krolak-Salmon P, Truy E, Vighetto A (2004) Pulse-synchronous eye oscillations revealing bone superior canal dehiscence. Ann Neurol 56:556–560

    Article  PubMed  Google Scholar 

  14. Halamgyi GM, Curthoys IS, Colebatch JG, Aw ST (2005) Vestibular responses to sound. Ann N Y Acad Sci 1039:54–67

    Article  Google Scholar 

  15. Rosengren SM, Welgampola MS, Halmagyi GM, Colebatch JG. Vestibular evoked myogenic potentials: past, present and future. Clin Neurophysiol (submitted for publication)

  16. Robinson K, Rudge P (1977) Abnormalities of the auditory evoked potentials in multiple sclerosis. Brain 100:19–40

    Article  PubMed  Google Scholar 

  17. Bickford RG, Jacobson JL, Cody DT (1964) Nature of average evoked potentials to sound and other stimuli in man. Ann N Y Acad Sci 112:204–223

    Article  PubMed  CAS  Google Scholar 

  18. Colebatch JG, Rothwell JC, Bronstein A, Ludman H (1994) Click-evoked vestibular activation in the Tullio phenomenon. J Neurol Neurosurg Psychiatry 57(12):1538–1540

    Article  PubMed  CAS  Google Scholar 

  19. Bronstein AM, Faldon M, Rothwell J, Gresty MA, Colebatch J, Ludman H (1995) Clinical and electrophysiological findings in the Tullio phenomenon. Acta Otolaryngol Suppl 520(Pt 1):209–211

    Article  PubMed  Google Scholar 

  20. Brantberg K, Granath K, Schart N (2007) Age-related changes in vestibular evoked myogenic potentials. Audiol Neurootol 12(4):247–253

    Article  PubMed  Google Scholar 

  21. Colebatch JG, Day BL, Bronstein AM, Davies RA, Gresty MA, Luxon LM (1998) Vestibular hypersensitivity to clicks is characteristic of the Tullio phenomenon. J Neurol Neurosurg Psychiatry 65:670–678

    Article  PubMed  CAS  Google Scholar 

  22. Merchant SN, Nakajima HH, Halpin C, Nadol JB Jr, Lee DJ, Innis WP et al (2007) Clinical investigation and mechanism of air-bone gaps in large vestibular aqueduct syndrome. Ann Otol Rhinol Laryngol 116(7):532–541

    PubMed  Google Scholar 

  23. Townsend GL, Cody DT (1971) The averaged inion response evoked by acoustic stimulation: its relation to the saccule. Ann Otol Rhinol Laryngol 80(1):121–131

    PubMed  CAS  Google Scholar 

  24. Young ED, Fernández C, Goldberg JM (1977) Responses of squirrel monkey vestibular neurons to audiofrequency sound and head vibration. Acta Otolaryngol 84(5–6):352–360

    Article  PubMed  CAS  Google Scholar 

  25. Didier A, Cazals Y (1989) Acoustic responses recorded from the saccular bundle on the eighth nerve of the guinea pig. Hear Res 37:123–128

    Article  PubMed  CAS  Google Scholar 

  26. Murofushi T, Curthoys IS (1997) Physiological and anatomical study of click-sensitive primary vestibular afferents in the guinea pig. Acta Otolaryngol 117(1):66–72

    Article  PubMed  CAS  Google Scholar 

  27. Curthoys IS (2010) A critical review of the neurophysiological evidence underlying clinical vestibular testing using sound, vibration and galvanic stimuli. Clin Neurophysiol 122(2):132–144

    Google Scholar 

  28. Murofushi T, Curthoys IS, Topple AN, Colebatch JG, Halmagyi GM (1995) Responses of guinea pig primary afferent vestibular neurons to clicks. Exp Brain Res 103:174–178

    Article  PubMed  CAS  Google Scholar 

  29. Curthoys IS (2008) The scientific basis of new clinical tests of otolith function. In: XXV Barany Society Meeting, Kyoto

  30. McCue MP, Guinan JJ Jr (1995) Spontaneous activity and frequency selectivity of acoustically responsive vestibular afferents in the cat. J Neurophysiol 74(4):1563–1572

    PubMed  CAS  Google Scholar 

  31. Jauregui-Renaud K, Faldon M, Clarke A, Bronstein AM, Gresty MA (1996) Skew deviation of the eyes in normal human subjects induced by semicircular canal stimulation. Neurosci Lett 205(2):135–137

    Article  PubMed  CAS  Google Scholar 

  32. Jauregui-Renaud K, Faldon ME, Gresty MA, Bronstein AM (2001) Horizontal ocular vergence and the threedimensional response to whole-body roll motion. Exp Brain Res 136(1):79–92

    Article  PubMed  CAS  Google Scholar 

  33. Puwanarajah P, Pretorius P, Bottrill I (2008) Superior semicircular canal dehiscence syndrome: a new aetiology. J Laryngol Otol 122(7):741–744

    Article  PubMed  CAS  Google Scholar 

  34. Cremer PD, Minor LB, Carey MD, Della Santina CC (2000) Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal. Neurology 55:1833–1841

    PubMed  CAS  Google Scholar 

  35. Suzuki JI, Tokumasu K, Goto K (1969) Eye movements from single utricular nerve stimulation in the cat. Acta Otol Laryngol 68:350–362

    Article  CAS  Google Scholar 

  36. Songer JE, Rosowski JJ (2006) The effect of superior-canal opening on middle-ear input admittance and air-conducted stapes velocity in chinchilla. J Acoust Soc Am 120(1):258–269

    Article  PubMed  Google Scholar 

  37. Carey JP, Hirvonen TP, Hullar TE, Minor LB (2004) Acoustic responses of vestibular afferents in a model of superior canal dehiscence. Otol Neurotol 25(3):345–352

    Article  PubMed  Google Scholar 

  38. Rosowski JJ, Songer JE, Nakajima HH, Brinsko KM, Merchant SN (2004) Clinical, experimental, and theoretical investigations of the effect of superior semicircular canal dehiscence on hearing mechanisms. Otol Neurotol 25(3):323–332

    Article  PubMed  Google Scholar 

  39. Rosengren SM, Aw ST, Halmagyi GM, Todd NP, Colebatch JG (2008) Ocular vestibular evoked myogenic potentials in superior canal dehiscence. J Neurol Neurosurg Psychiatry 79(5):559–568

    Article  PubMed  CAS  Google Scholar 

  40. Curthoys IS, Iwasaki S, Chihara Y, Ushio M, McGarvie LA, Burgess AM (2011) The ocular vestibular-evoked myogenic potential to air conducted sound; probable superior vestibular nerve origin. Clin Neurophysiol 122(3):611–616

    Article  PubMed  Google Scholar 

  41. Halmagyi GM, Curthoys IS (1988) A clinical sign of canal paresis. Arch Neurol 45(7):737–739

    Article  PubMed  CAS  Google Scholar 

  42. Carey JP, Migliaccio AA, Minor LB (2007) Semicircular canal function before and after surgery for superior canal dehiscence. Otol Neurotol 28(3):356–364

    Article  PubMed  Google Scholar 

  43. Crane BT, Minor LB, Carey JP (2008) Three dimensional computed tomography of superior canal dehiscence syndrome. Otol Neurotol 29(5):699–705

    Article  PubMed  Google Scholar 

  44. Belden CJ, Weg N, Minor LB, Zinreich SJ (2003) CT evaluation of bone dehiscence of the superior semicircular canal as a cause of sound- and/or pressure-induced vertigo. Radiol 226(2):337–343

    Article  Google Scholar 

  45. Williamson RA, Vrabec JT, Coker NJ, Sandlin M (2003) Coronal computed tomography prevalence of superior semicircular canal dehiscence. Otolaryngol Head Neck Surg 129(5):481–489

    Article  PubMed  Google Scholar 

  46. Carey JP, Minor LB, Nager GT (2000) Dehiscence or thinning of bone overlying the superior semicircular canal in a temporal bone survey. Archives of Otolaryngology and Head and Neck Surgery 126:137–147

    CAS  Google Scholar 

  47. Kwee HL (1976) The occurrence of the Tullio phenomenon in congenitally deaf children. J Laryngol Otol 84:155–166

    Google Scholar 

  48. Mayer O, Fraser JS (1936) Pathological changes in the ear in late congenital syphilis. J Laryngol Otol 52:683–714

    Google Scholar 

  49. Nields JA, Kveton JF (1991) Tullio phenomenon and sero-negative lyme borreliosis. Lancet 338:128–129

    Article  PubMed  CAS  Google Scholar 

  50. Pyykko I, Ishizaki H, Aalot H, Starck J (1992) Relevance of the Tullio phenomenon in assessing perilymphatic leak in vertiginous patients. Am J Otol 13(4):339–342

    PubMed  CAS  Google Scholar 

  51. Sheykholeslami K, Schmerber S, Kermany MH, Kaga K (2004) Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct. Hearing Res 190:161–168

    Article  Google Scholar 

  52. Gopen Q, Zhou G, Poe D, Kenna M, Jones D (2010) Posterior semicircular canal dehiscence: first reported case series. Otol Neurotol 31:339–344

    Article  PubMed  Google Scholar 

  53. Kuhn JJ, Clenney T (2010) The association between semicircular canal dehiscence and Chiari type I malformation. Arch Otolaryngol Head Neck Surg 136(10):1009–1014

    Article  PubMed  Google Scholar 

  54. Hirvonen TP, Carey JP, Liang CJ, Minor LB (2001) Superior canal dehiscence—mechanisms of pressure sensitivity in a chinchilla model. Arch Otolaryngol Head Neck Surgery 127:1331–1336

    CAS  Google Scholar 

  55. Tsunoda A, Terasaki O (2002) Dehiscence of the bony roof of the superior semicircular canal in the middle cranial fossa. J Laryngol Otol 116(7):514–518

    PubMed  Google Scholar 

  56. Hirvonen TP, Weg N, Zinreich SJ, Minor LB (2003) High-resolution CT findings suggest a developmental abnormality underlying superior canal dehiscence syndrome. Acta Otolaryngol 123:477–481

    Article  PubMed  Google Scholar 

  57. Yuen HW, Boeddinghaus R, Eikelboom RH, Atlas MD (2009) The relationship between the air-bone gap and the size of superior semicircular canal dehiscence. Otolaryngol Head Neck Surg 141(6):689–694

    Article  PubMed  Google Scholar 

  58. Rajan GP, Leaper MR, Goggin L, Atlas MD, Boeddinghaus R, Eikelboom RK (2008) The effects f superior semicircular canal dehiscence on the labyrinth: does size matter. Otol Neurotol 29(7):972–975

    Article  PubMed  Google Scholar 

  59. Lip G, Nichols DM (2009) Measurement of defect angle in superior semicircular canal dehiscence. Clin Radiol 64(12):1210–1213

    Article  PubMed  CAS  Google Scholar 

  60. Friedland DR, Michel MA (2006) Cranial thickness in superior canal dehiscence syndrome: implications for canal resurfacing surgery. Otol Neurotol 27:346–354

    Article  PubMed  Google Scholar 

  61. Minor LB (2000) Superior canal dehiscence syndrome. Am J Otol 21:9–19

    PubMed  CAS  Google Scholar 

  62. Smullen JL, Andrist EC, Gianoli GJ (1999) Superior semicircular canal dehiscence: a new cause of vertigo. J La State Med Soc 151(8):397–400

    PubMed  CAS  Google Scholar 

  63. Kertesz TR, Shelton C, Wiggins R, Salzman KL, Glastonbury CM, Harnsberger R (2001) Superior semi-circular canal dehiscence: resurfacing with calcium phosphate bone cement. Aust J Otolaryngol 4(3):167–173

    Google Scholar 

  64. Silverstein H, Van Ess MJ (2009) Complete round window niche occlusion for superior semicircular canal dehiscence syndrome: a minimally invasive approach. Ear Nose Throat J 88(8):1042–1056

    PubMed  Google Scholar 

  65. Minor LB, Carey JP, Cremer PD, Lustig LR, Streubel SO, Ruckenstein MJ (2003) Dehiscence of bone overlying the superior canal as a cause of apparent conductive hearing loss. Otol Neurotol 24(2):270–278

    Article  PubMed  Google Scholar 

  66. Crane BT, Lin FR, Minor LB, Carey JP (2010) Improvement in autophony symptoms after superior canal dehiscence repair. Otol Neurotol 31(1):140–146

    Article  PubMed  Google Scholar 

  67. Walgampola MS, Myrie OA, Minor LB, Carey JP (2008) Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence. Neurology 70:464–472

    Article  Google Scholar 

  68. Kanayama R, Bronstein AM, Gresty MA, Brookes GB (1995) Vertical and torsional VOR in posterior canal occlusion. Acta Otolaryngol 520(2):362–365

    Article  Google Scholar 

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Acknowledgments

We extend our gratitude to Dr Harold Ludman, Dr Gerald Brooks, and Professor Michael Gresty for providing clinical information on patients. We also thank Dr Tim Beale and Dr John Stevens for reviewing many of the radiological images and Mary Faldon for her assistance with patient testing.

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Authors and Affiliations

  1. Department of Neuro-otology, Imperial College London, Charing Cross Hospital, Fulham Palace Road, London, W6 8RF, UK

    Diego Kaski & A. M. Bronstein

  2. National Hospital for Neurology and Neurosurgery, London, WC1N 3BG, UK

    Diego Kaski, R. Davies, L. Luxon, A. M. Bronstein & P. Rudge

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  1. Diego Kaski
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  2. R. Davies
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  3. L. Luxon
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  4. A. M. Bronstein
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Correspondence to Diego Kaski.

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Kaski, D., Davies, R., Luxon, L. et al. The Tullio phenomenon: a neurologically neglected presentation. J Neurol 259, 4–21 (2012). https://doi.org/10.1007/s00415-011-6130-x

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