Clinical and electrophysiological findings in the Tullio phenomenon.

A 55 year old female with idiopathic Tullio phenomenon is presented. Binocular, scleral search eye coil recordings demonstrated a predominantly torsional left-beating and vertical down-beating nystagmus in response to sound intensities over 100 dB HL to the left ear, increasing in amplitude and slow phase velocity with sound intensity and removal of visual fixation. The vertical ocular movement was conjugate, i.e. without skew deviation. Neuro-imaging, all other neuro-otological features, including ipsilateral-contralateral stapedius muscle reflexes, and surgical exploration of the middle ear, were normal. Click-evoked vestibulo-collic potentials were normal from the right ear but showed low threshold (70 dB) and increased amplitude from the left. There was no evidence that the Tullio phenomenon in this patient arises from stapes footplate hypermobility. The findings suggest that some cases of the Tullio phenomenon may be due to a hyperexcitability of the normal vestibular response to sound.

Click-evoked vestibular activation in the Tullio phenomenon.

Click-evoked vestibulocollic reflexes were studied in a patient with a unilateral Tullio phenomenon (sound induced vestibular symptoms) and the findings were compared with those of a group of normal subjects. Compared with normal subjects, the reflexes elicited from her symptomatic side were large and had an abnormally low threshold, but retained a normal waveform. The click-evoked responses in this patient show changes consistent with her symptomatology and are indicative of a pathological increase in the normal vestibular sensitivity to sound.

An unusual cause of benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo (BPPV) is a self-limiting condition characterized by vertigo and nystagmus induced by certain head positions. The most common causes of BPPV are post-traumatic following head injury and post-viral labyrinthitis. We present an interesting case of BPPV following an otherwise uneventful neurosurgical removal of a parietal osteoma using hammer and chisel. Caution should be exercised during such procedures as disabling vertigo can result for a considerable period of time.

Clinical and vestibulometric status following labyrinthectomy or vestibular nerve section for Menière's disease.

A clinical and vestibulometric comparison of 10 patients following labyrinthectomy, 5 following vestibular nerve section, and 10 control subjects was undertaken in order to evaluate the long-term effectiveness of these procedures. Testing was performed a mean of 8.5 years after surgery. Vestibulometry assessed time constants, gain, and phase advance of the vestibulo-ocular reflex (VOR) in both horizontal and vertical planes of head movement. Loss of the anticipated increase in phase advance in the vertical VOR of the labyrinthectomy group, significantly different from the vestibular nerve section group (P less than 0.05 and P less than 0.01 for each direction of rotation), suggests that posterior and superior semicircular canal function remains intact following per-meatal labyrinthectomy. Although stabilometry suggested that balance in the labyrinthectomy group was poorer than in the two other groups (P less than 0.025 without optic fixation), clinical assessment demonstrated excellent functional results. Per-meatal labyrinthectomy remains a safe and effective procedure for the relief of vestibular symptoms, although vestibular nerve section may provide more complete removal of contributory end-organ function.

Persisting nystagmus following vestibular nerve section for Menière's disease.

Unilateral vestibular nerve section (VNS) creates a state of acute dysequilibrium which resolves by a process of central compensation. This disturbance resolves quickly and central compensation is complete generally within a month with resolution of symptoms and signs. The course of central compensation following VNS will be similar to that seen after labyrinthectomy because the detachment of hemi-labyrinthine input that is achieved by both will be identical. Six patients are presented who have undergone VNS at least 2.7 years ago (Average 3.5 years); all of them have persisting spontaneous peripheral type horizontal jerk nystagmus, present with optic fixation in five. This is obvious clinically and was confirmed in each case by agreement of three independent observers and has been recorded by electronystagmography (ENG). They are free from marked vestibular symptoms. The explanations of mechanisms involved in central compensation are discussed with respect to this previously unrecorded clinical observation.

Thrombotic thrombocytopenic purpura: report of a case with a possible response to high-dose intravenous gamma globulin.

The case history of a 71-year-old woman with three episodes of a microangiopathic hemolytic anemia over a 22-year span is detailed. During the last episode a possible response of her thrombotic thrombocytopenic purpura (TTP)-like syndrome to the administration of intravenous immunoglobulin is documented. In retrospect it became apparent that she only improved in her prior episodes after receiving plasma-containing blood products. Prior case reports of TTP responses to intravenous immunoglobulin are reviewed with specific attention to the dosage used. Since a prospectively randomized series is unlikely to be reported, investigators should be encouraged to report their experience with intravenously administered gamma globulin.

Temporal bone venous anomaly of surgical significance.

Variability in the size of the dural sinuses and jugular bulb is not uncommon and usually manifests as a high jugular bulb encroaching upon the floor of the middle ear. A rarer entity is the superior and medial extension of the jugular bulb into the bone of the posterior wall of the internal auditory meatus. We report a case where this anomaly was encountered during acoustic neuroma surgery making exposure of the fundus of the internal auditory meatus technically impossible. The possibility of a communication with the superior petrosal sinus is discussed.

Transtympanic surgery for hemifacial spasm--an electrophysiological study.

Producing deliberate damage to the facial nerve in the middle ear is an established method of treating idiopathic hemifacial spasm. However, no previous electrophysiological studies have investigated the effect of this operation on facial nerve function. Eleven patients undergoing transtympanic facial nerve needling were investigated with electromyography. Following operation reduction in the abnormal discharges and synkinesis typical of hemifacial spasm was seen in the majority of patients. The success of this type of operation, although temporary in some patients, together with other neurophysiological studies conflicts with the hypothesis that this disorder is due to ephaptic transmission caused by vascular compression of the facial nerve in the cerebello-pontine angle.

Hemifacial spasm: operative treatment.

This paper describes experience with a transtympanic facial nerve needling operation in 62 patients with hemifacial spasm, over a 15-year period. The procedure is simple and effective, but unfortunately it is usually marred by recurrence of the spasm. Repeat operations are easy to perform, and usually valuable the first time. Despite recurrence, most patients are finally less troubled than before operation. A small number have proved very resistant to treatment, and the aetiological implications of this are presented. Other operative options are also discussed, together with our experience in a small number treated by facial nerve needling at other sites.

Damage to the middle ear and the inner ear in underwater divers.

Postmortem human tissue from recently deceased divers was processed histologically to assess any inner and middle ear damage that could have resulted from the effects of pressure during diving. The following new findings are particularly noteworthy. In one diver, ascent while breath holding resulted in the rupture of the ear drum and blood in the middle ear, in addition to pulmonary barotrauma. In a second diver, following inner ear decompression sickness, new bone growth, similar to that described earlier in experimental studies with the squirrel monkey, was observed in the arms of one of the semicircular canals. These observations are further confirmation that otologic disorders can be a serious threat to divers.