Vestibular Hypersensitivity to Clicks Is Characteristic of the Tullio Phenomenon
Total Page:16
File Type:pdf, Size:1020Kb
670 J Neurol Neurosurg Psychiatry 1998;65:670–678 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.5.670 on 1 November 1998. Downloaded from Vestibular hypersensitivity to clicks is characteristic of the Tullio phenomenon J G Colebatch, B L Day, A M Bronstein, R A Davies, M A Gresty, L M Luxon, J C Rothwell Abstract sound and it is only when extremely loud Objectives—The frequency of pathologi- sounds are encountered, such as are achieved cally reduced click thresholds for vestibu- near jet engines or explosions, that there is evi- lar activation was explored in patients dence of vestibular activation.1–3 Various dis- with the Tullio phenomenon (sound in- ease states have been reported to underlie the duced vestibular activation). pathological sound sensitivity that occurs in the Methods—Seven patients (eight aVected Tullio phenomenon, including dislocation of ears) with symptoms of oscillopsia and the stapes footplate, labyrinthine fistulas, en- unsteadiness in response to loud external dolymphatic hydrops, and dehiscence of the sounds or to the patient’s own voice were superior semicircular canal.4–7 examined. In all but one patient, vestibu- We have recently reported a patient with the lar hypersensitivity to sound was con- Tullio phenomenon in whom there was a firmed by the fact that eye movements pathological increase in her sensitivity to a new 8 could be produced by pure tones of 110 dB test of vestibular function using clicks. This intensity or less. Conventional diagnostic finding is of particular importance for various imaging was normal in all cases and three reasons, not least being that conventional tests of the patients had normal middle ears at of vestibular function in these patients show no surgical exploration. Thresholds for click consistent abnormality. Sound sensitivity does evoked vestibulocollic reflexes were com- not necessarily imply abnormal sensitivity to pared with those of a group of normal clicks: if the abnormal vestibular activation subjects. Galvanic stimulation was used as were highly frequency selective, dependent on a complementary method of examining temporal summation at one or more synapses copyright. the excitability of vestibular reflexes. or an indirect consequence of reflexes evoked by the sound,6 then no abnormality would be Results—All the patients showed a re- duced threshold for click activation of ves- expected in response to the very short acoustic tibulocollic reflexes arising from the stimulation caused by clicks. Conversely, ab- aVected ear. Short latency EMG responses normal vestibular sensitivity to clicks would to clicks were also present in posterior strongly suggest an intrinsic abnormality of neck and leg muscles, suggesting that vestibular receptors, most probably the sac- these muscles receive vestibular projec- cule, the vestibular receptor with the greatest Department of sensitivity to this form of stimulus.9–12 Neurology, Prince of tions. Galvanic stimulation produced a normal pattern of body sway in four of the In view of the potential importance of our Wales Hospital, Sydney initial finding we have investigated a series of 2031, Australia five patients tested. http://jnnp.bmj.com/ patients with Tullio phenomenon using clicks as J G Colebatch —A pathologically reduced Conclusions well as galvanic vestibular stimulation, the threshold to click activation (<70 dB NHL MRC Human second to assess the excitability of vestibular (average normal hearing level)) seems to Movement and pathways proximal to the receptors. We have in be a consistent feature of the Tullio phe- Balance Unit addition used the opportunity presented by nomenon and a useful diagnostic crite- BLDay these patients’ low thresholds to click activation A M Bronstein rion. This in turn is most likely to be due to demonstrate the pattern of short latency ves- M A Gresty to an increased eVectiveness of the trans- J C Rothwell tibular reflex projections to other neck and limb mission of sound energy to saccular re- on September 29, 2021 by guest. Protected muscles. Department of ceptors. Activation of these receptors Neuro-Otology, probably contributed to the vestibular National Hospital, symptoms experienced by the patients. Methods Queen Square, (J Neurol Neurosurg Psychiatry 1998;65:670–678) Click evoked vestibulocollic reflexes were re- London, UK R A Davies Keywords: Tullio phenomenon; vestibular function corded from seven patients who had presented L M Luxon tests; vestibular nerve; acoustic stimulation; vestibuloco- with symptoms suggestive of the Tullio phenom- llic reflex enon to the National Hospital for Nervous Dis- Correspondence to: eases, Queen Square and from 25 normal Dr JG Colebatch, Department of Neurology, volunteers. One of the patients has been 813 Prince of Wales Hospital, The Tullio phenomenon is a rare but remark- reported on previously (patient 7 ). Six of the High Street, Randwick, able clinical entity in which everyday loud seven patients had been shown to develop eye Sydney, NSW 2031, sounds will activate the vestibular apparatus. Australia. Fax 0061 2 9382 movements in response to loud tones generated 2428. AVected patients develop oscillopsia and un- either during audiometric testing (often noted steadiness in response to, for example, shout- while testing for the stapedial reflex), or more Received 27 February 1998 ing, traYc noise or their own voices. Normally, formally using scleral search coils or video- and in revised form 7 May 1998 despite its proximity to the cochlea, the oculography (VOG: SensoMotoric Instruments, Accepted 15 May 1998. vestibular apparatus is relatively insensitive to Germany). One patient (No 5) complained of a Click EMG responses in Tullio 671 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.5.670 on 1 November 1998. Downloaded from Summary of clinical features and click responses for patients with the Tullio phenomenon (Nos 2 and 6, bilaterally). Conventional cranial imaging with CT was normal in all patients, as Symptomatic Duration Sound threshold p13/n23 p13/n23 Patient Sex, age ear (y) frequency (dB (kHz)) @75dB threshold (dB) was MRI in the four patients so examined. Three patients had their middle ears explored 1 M, 44 L 8 110 (0.5 ) 1.17 65 (by Mr H. Ludman and Mr G Brookes) but no 2 F, 28 L 11 105 (1, 2) 0.86 <70 3 F, 22 R* 5 100 (1) 1.04 <60 abnormality was found in any case. Sound- 4 M, 48 R 8 105 (0.5 ) 1.12 <60 induced eye movements were shown to be 5 M, 63 L 12 † 0.97 <75‡ 6 M, 57 R 18 110§ >1.13¶ <60 present in six patients and characterised in five. L* 18 110§ 0.27¶ <80 In these five patients the lowest threshold for 7 F, 56 L* 10 110 (0.5, 1) ** 77 inducing eye movements varied between 100 *Surgical exploration of the middle ear, which in all three cases was normal. and 110 dB, and the most eVective frequency †patient developed oscillopsia with 90 dB stimuli. was either 500 Hz, 1 kHz, or 2 kHz (table). The ‡Not tested with clicks of <75 dB intensity. induced eye movements, measured during §Frequency not recorded. ¶Tested at 70 dB NHL, following attempted stapediopexy on the left. stimulation of five ears, consisted of torsional **Responded in one of two runs to clicks of 73 dB intensity. slow phases with movement of the upper pole Sound threshold=intensities and frequencies required to produce eye movements; p13-n23 @ 75 directed away from the stimulated ear associated dB=the peak to peak amplitude of the p13-n23 potential expressed as a ratio of the mean back- with, in two patients, upward deviation. Nystag- ground rectified EMG level; p13-n23 threshold=threshold for evoking these potentials in the ipsi- lateral sternocleidomastoid muscle. mus, when present, beat in the opposite direction (see Bronstein et al13 for a detailed sensation of tilting during audiometric assess- desciption of the responses of patient 7). ment. A total of 25 normal volunteers, recruited from laboratory and hospital staV and their rela- CLICK EVOKED VESTIBULAR ACTIVATION tives, and who had no history of either Methods of click evoked vestibular activation neurological or vestibular abnormalities, were were similar to those previously published.814 included to provide reference values. Their ages The patients were tested using a custom ranged from 22 to 65 years, eight were men and designed circuit which delivered 0.1 ms long 17 were women. square wave pulses to calibrated earphones Continuous tone intensities are measured in (TDH 49: Telephonics Corp, New York, USA). dB International Standards Organisation All click intensities are expressed with respect to (ISO) and click intensities in dB NHL (average the typical normal hearing threshold for such normal hearing level; 0 dB=45 dB sound clicks (taken as 45 dB SPL). With this reference, pressure level (SPL)). a 100 dB NHL click corresponds to a pulse with an amplitude of about 9 V delivered to the ear- copyright. CLINICAL FINDINGS phones. For technical reasons, the softest click Several characteristics of the seven patients are that could be given to patient 5 was 75 dB NHL, summarised in the table. One patient was but the other patients were tested down to 60 dB aVected bilaterally (patient 6), giving eight NHL. Data were collected using a 1401plus symptomatic ears (five left, three right). The laboratory interface (Cambridge Electronic De- duration of symptoms varied from 5 to 18 years sign, Cambridge, UK) with commercial soft- (mean 10.3). All had symptoms of vestibular ware (SIGAVG 6.13) which allowed conditional activation by sound either as sound induced averaging. In all click studies, the stimuli were oscillopsia or sound induced imbalance, or both. delivered pseudorandomly either to the right or Two patients (Nos 1 and 2) had noted head jerks left ear at 3/s using a sampling rate of 5 kHz and precipitated by sound.