Tullio Phenomenon


Article Author:
Benjamin Lehmkuhl


Article Editor:
Claudio Andaloro


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Phillip Hynes
Kavin Sugumar


Updated:
5/13/2019 1:22:10 PM

Introduction

The Tullio phenomenon is a symptom or physical exam finding whereas nystagmus or vertigo are induced in response to sound. This was described in animals nearly 90 years ago by professor Pietro Tullio in Bologna, Italy. In humans, the phenomenon was first observed in patients suffering from advanced syphilis, and since that time, numerous reports and studies have shown that the Tullio phenomenon can be seen in various pathologies, including patient’s with no pathology at all [1] Today, however, the Tullio phenomenon is most commonly associated with superior semicircular canal dehiscence  [2].

Function

Professor Tullio was the director of the Laboratory of Experimental Physiology in Bologna during the early twentieth century. He was inspired by the work of Frenchman Jean Flourens who had discovered that pigeons will make sudden and impetuous movements in the same plane as an injured semicircular canal [1]. Tullio performed a majority of his experiments on live pigeons. Pigeons were well known in testing labyrinthine physiology because of their favorable anatomy, as well as prominent head nystagmus allowing for easy observations of responses[3].

Tullio’s first experiments were made observing the movements of water on tuning forks, followed by particle currents in vibrating water. He then began introducing particles into the endolymph and perilymph of pigeons and observed the current movement in response to sounds. These currents were seen to wave rhythmically against the ampulla, generating a movement in the head of the pigeon in the plane of the canal. As Tullio increased the intensity of the sound, the current would begin to oscillate and the head of the animal would nystagmus at a frequency corresponding to the oscillation of the current. Sounds stimulating the ear affected all three canals at the same time; however, the difference in the intensity of the currents distributed through these canals generated head movement. When opening a semicircular canal, its current will dominate the other two canals, leading to observable movement of the open canal.

Tullio later analyzed the head movements made by pigeons after opening each canal by attaching a lever to the beak to create a graphic of the reflexes made in response the sound stimuli. He found that the pigeons would move their heads in the same plane as the opened canal. Tullio would then apply cocaine to the osseous openings which would paralyze the nerve endings, and the pigeon would not move to further stimulation. After opening all three canals, the heads would remain motionless from the fenestrations neutralizing each other. Tullio was able to produce head rotation around an anterior-posterior axis with the head turning toward to unaffected ear. This is due to utricle excitation and persisted after the ampullae of all three canals had been paralyzed. In later experiments, Tullio recreated the lesions induced by Flourens and concluded that irritative stretching of ampullae nerves causes labyrinth ataxia. This is now known as Flourens syndrome [1]

After 20 years of research, Tullio presented his findings in Some Experiments and Considerations on Experimental Otology and Phonetics in 1929, and his work was nominated for the Nobel Prize in Physiology or Medicine in 1932 [1].

After Tullio’s work and its implications with labyrinthine fistula, a number of investigators have reported the phenomenon occurring in other conditions. Initially, the presence of the Tullio phenomenon in humans was considered to be an indication of congenital syphilis where patients developed osteomyelitis of the labyrinth, with miliary gumma in the endosteum capable of creating labyrinthine fistulas, and was considered to be an indication of congenital syphilis [1]. The presence of this fistula was thought to cause the Tullio phenomenon. Cawthorne suggested that the Tullio phenomenon only occurred in humans when more than one mobile window opened into the internal ear on the vestibular side of the vestibular membrane [4].  However, a number of studies over the past 60 years have indicated the presence of the Tullio phenomenon in a wide range of situations.

Kacker and Hinchcliffe reported a positive Tullio sign in two patients with Meniere syndrome with negative fistula tests, and one patient with a history of trauma where the stapes was found to have subluxed into the vestibule. In all three cases, serologic testing for syphilis was negative [5]. Kwee investigated the incidence of the Tullio phenomenon in a group of 150 deaf children and found that 50 demonstrated a positive Tullio phenomenon in one or both ears, representing 76 of the 300 ears. He reported that, in all cases, the direction of the nystagmus was toward the stimulated ear, and in some cases, there were accompanying side effects such as deviation of the head away from the source of the tone and subjective sensations of dizziness. It was also noted, as would be expected, that there was no incidence of a Tullio phenomenon in the absence of a calorically excitable labyrinth [6]. Nadol found in a patient with bilateral deafness after an upper respiratory infection in childhood and positive Hennebert sign, fibrous adhesions to parts of the membranous labyrinth which was named vestibulofibrosis, and suggested a pathological basis for a positive fistula sign with intact tympanic membrane [7]. Later studies also found this histologic finding in several post-inflammatory or progressive inner ear disorders including, congenital syphilis, viral labrynthitis, and Meniere disease. These findings are of note because it may be the pathophysiology behind sound-induced vestibular symptoms in patients with endolymphatic hydrops, where the membranous labyrinth becomes distended enough to contact the medial aspect of the stapes footplate. All patient’s with Meniere disease had a positive Tullio in the Erlich study [8]

The high incidence of Tullio phenomenon in healthy subjects and patients with various cochleovestibular disorders suggest that, when properly monitored for, sound-induced vestibular stimulation can be observed. Parker examined the physiologic response in the vestibular system to acoustic stimulation and found that either pressure changes or high-intensity sound caused measurable displacement of the stapes paralleling changes in the perilymph pressure, as well as measurable changes in the rates vestibular neuron firings. It demonstrated that in otologically normal patients exhibited nystagmus and other vestibular symptoms in response to intense pressure or audio frequency stimulation. Treatment of the Tullio phenomenon is unique to each particular cause [9].

Issues of Concern

While the Tullio phenomenon cannot be considered a diagnostic tool for a particular disease, which Tullio had hoped for, it can point towards a cochleovestibular or other otologic pathology that warrants further investigation. A detailed history and thorough physical exam can narrow the differential, but additional testing may be required including audiogram, tympanogram, stapedial reflexes, fistula test, videonystagmography, electrocochleography, and CT imaging.

Clinical Significance

Currently, the Tullio phenomenon is most commonly associated with superior semicircular canal dehiscence (SSCD). Table 1 lists several conditions linked with the phenomenon. Superior semicircular canal dehiscence was first described in 1998 and is a disease characterized by sound-induced vertigo and eye movements, chronic dysequilibrium, conductive hearing loss (CHL), and decreased hearing thresholds for bone conducted sounds. Conductive hyperacusis may lead to autophony (hearing one’s own voice), pulsatile tinnitus, or hearing one’s eye movements [10]. The disorder is caused by absent bone over the superior semicircular canal. When patients suffering from SSCD exhibit the Tullio phenomenon, they experience a mixed vertical-torsional nystagmus in which the slow phase of nystagmus rotates up and away from the affected ear. This type of nystagmus aligns in the same plane as the superior semicircular canal that is affected and is pathognomonic for SSCD[2] [11]. Depending on the type of stimulus, either excitation or inhibition of the superior canal may occur. Excitation is caused by ampullofugal displacement of the cupola through positive external auditory canal (EAC) pressure in the forms of tragal compression, sound or nasal Valsalva.  Inhibition is caused by ampullopetal deflection of the cupola through negative EAC pressure via jugulovenous compression or glottis Valsalva. Patient’s may also experience the Hennebert sign, eye movement induced by pressure in the external auditory canal [12]. Diagnosis of SSCD is made with CT imaging which shows thin or dehiscence bone over the superior semicircular canal. Treatment is surgical where the superior canal is plugged with bone chip and fascia and covered with bone cement via a middle fossa approach. The transmastoid, endoscopic and transcanal or endaural approaches have also been used as of recent [13].

Table 1: Conditions linked to the Tullio phenomenon [14][15][8][16][6][17][18][6]

  • Superior semicircular canal dehiscence
  • Otosclerosis
  • Congenital syphilis
  • Ménière’s disease
  • Perilymph fistula
  • Cholesteatoma with semicircular canal erosion & fenestration
  • Head trauma
  • Normal individuals
  • Post stapedectomy
  • Post tympanomastoidectomy
  • Collapsed canal syndrome
  • Congenital deafness
  • Seronegative Lyme Borreliosis
  • Middle ear osteoma

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Tullio Phenomenon - Questions

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Which of the following describes the Tullio phenomenon?



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What is the Tullio phenomenon?



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A patient presents to the office with a new complaint of room-spinning sensations and rapid eye movements while attending a concert last night. Which of the following is patient describing?



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Which of the following statements about the Tullio phenomenon is true?



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A 50-year-old female presents to the office with the complaint of chronic dizziness for the last several months with short bursts of vertigo when exposed to loud noises. Audiogram shows a right-sided conductive hearing loss, greater in the lower frequencies. Stapedial reflexes were normal. Which of the following tests should be ordered next?



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A 39-year-old male presents to the office with the complaint of vertigo and eye movements while he is at work. The patient recently starting working for a construction company and admits to not always wearing hearing protection. He reports no other symptoms. He denies any medical or surgical history. The audiogram revealed normal, symmetric hearing bilaterally. Stapedial reflexes, electrocorticography (ECoG), cervical vestibular myogenic potentials (cVEMPs), and CT scan were normal. Which of the following if the most appropriate next step?



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A 63-year-old female was referring to the office for dizziness and eye movements during a recent flight to Florida. No history of noise exposure, ear surgeries or family history of hearing loss. Audiogram reveals conductive hearing loss on the left. Stapedial reflexes, vestibular evoked myogenic potential (VEMPs) and CT scan, were ordered. Which of the following statements describes the pathology behind the suspected diagnosis?



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Tullio Phenomenon - References

References

The experiments behind the Tullio phenomenon., Addams-Williams J,Wu K,Ray J,, The Journal of laryngology and otology, 2014 Mar     [PubMed]
Tullio phenomenon and postural stability: experimental study in normal subjects and patients with vertigo., Ishizaki H,Pyykkö I,Aalto H,Starck J,, The Annals of otology, rhinology, and laryngology, 1991 Dec     [PubMed]
Tullio phenomenon and seronegative Lyme borreliosis., Nields JA,Kueton JF,, Lancet (London, England), 1991 Jul 13     [PubMed]
The Tullio phenomenon and perilymph fistula., Fox EJ,Balkany TJ,Arenberg IK,, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 1988 Jan     [PubMed]
The Tullio phenomenon, fistula test, and Hennebert's sign: clinical significance., Pillsbury HC 3rd,Postma DS,, Otolaryngologic clinics of North America, 1983 Feb     [PubMed]
The incidence and significance of the Tullio phenomenon in man., Erlich MA,Lawson W,, Otolaryngology and head and neck surgery, 1980 Sep-Oct     [PubMed]
The occurrence of the Tullio phenomenon in congenitally deaf children., Kwee HL,, The Journal of laryngology and otology, 1976 Jun     [PubMed]
Superior canal dehiscence syndrome., Minor LB,, The American journal of otology, 2000 Jan     [PubMed]
Sound- and/or pressure-induced vertigo due to bone dehiscence of the superior semicircular canal., Minor LB,Solomon D,Zinreich JS,Zee DS,, Archives of otolaryngology--head & neck surgery, 1998 Mar     [PubMed]
HUIZINGA E, The physiological and clinical importance of experimental work on the pigeon's labyrinth. The Journal of laryngology and otology. 1955 Apr;     [PubMed]
CAWTHORNE T, The contribution of surgery to the problems of neuro-otology. British medical bulletin. 1956 May;     [PubMed]
Kacker SK,Hinchcliffe R, Unusual Tullio phenomena. The Journal of laryngology and otology. 1970 Feb;     [PubMed]
Nadol JB Jr, Positive     [PubMed]
Golz A,Westerman ST,Westerman LM,Goldenberg D,Netzer A,Wiedmyer T,Fradis M,Joachims HZ, The effects of noise on the vestibular system. American journal of otolaryngology. 2001 May-Jun;     [PubMed]
Ostrowski VB,Byskosh A,Hain TC, Tullio phenomenon with dehiscence of the superior semicircular canal. Otology     [PubMed]
Hornigold R,Pearch BJ,Gleeson MJ, An Osteoma of the Middle Ear Presenting with the Tullio Phenomenon. Skull base : official journal of North American Skull Base Society ... [et al.]. 2003 May;     [PubMed]
Cremer PD,Minor LB,Carey JP,Della Santina CC, Eye movements in patients with superior canal dehiscence syndrome align with the abnormal canal. Neurology. 2000 Dec 26;     [PubMed]
Mau C,Kamal N,Badeti S,Reddy R,Ying YM,Jyung RW,Liu JK, Superior semicircular canal dehiscence: Diagnosis and management. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 2018 Feb;     [PubMed]

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