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Periodic alternating nystagmus associated with Arnold-Chiari malformation.

The Arnold-Chiari malformation type I is a rare congenital condition characterized by herniation of the cerebellar tonsils into the upper cervical spinal tract. Various otoneurological manifestations occur in patients with the disease, which has usually been associated with a downbeat type of nystagmus. In this paper a case with this condition is reported presenting with periodic alternating nystagmus (PAN), which is a rare type of nystagmus observed in diseases of the central nervous system of different pathologies. The patient had a complete otoneurological examination and vestibular function testing. Diagnosis was established by using magnetic resonance imaging (MRI).

Adult↗

Middle fossa vestibular nerve section in the management of Ménière's disease.

The triad of fluctuating hearing loss, tinnitus, and episodic attacks of vertigo known as Ménière's disease can be a frustrating therapeutic problem. While most patients respond to a medical regime consisting of a low salt diet, diuretics, diazepam, and propantheline bromide, there is a significant group who remain refractory to conservative management. These individuals either continue to experience their distressing symptoms or undergo some type of surgical procedure in an attempt to seek relief. There are a multitude of such techniques available to the otologic surgeon and he must make the decision as to which procedure is best for any given individual. The authors' employ three surgical approaches for the management of Ménire's disease. These are the translabyrinthine labyrinthectomy, the endolymphatic subarachnoid shunt, and the middle fossa vestibular nerve section. In this paper the indications for surgery, technique, results and complications are covered in detail. Thirty-one patients are reviewed who have been followed from one to four years postoperatively. Seventeen had total and 14 had superior vestibular nerve sections. The results were much better in the total group, as 94 percent had complete relief of vertigo and 76 percent were able to maintain their preoperative hearing level. The authors feel the middle fossa vestibular nerve section is a valuable addition to the otologist's armamentarium in the treatment of the patient who is refractory to medical management.

Adult↗

Vestibular dysfunction associated with benign paroxysmal vertigo.

Benign paroxysmal vertigo (BPV) is a clinical syndrome of vestibular origin although generally no evidence of vestibular dysfunction can be demonstrated with conventional tests. In a review of 1350 consecutive dizzy patients, there were 125 with BPV and of these, 33 underwent a quantitative rotational test of vestibular function. The rotational results showed reduces vestibular system gain for these BPV patients. In addition, they could be subdivided on the basis of a normal or shorter cupular time constant (Tc). Separation of patients into diagnostic categories revealed that those categorized as cupulolithiasis and viral labyrinthitis had a normal Tc range and those categorized as trauma and idiopathic had a short Tc. The reduced gain and short Tc in the latter group suggest hair cell and/or nerve damage since these same changes occur in patients with destructive peripheral vestibular disease.

Adult↗

Labyrinthine trauma during ear surgery.

Surgical trauma inflicted upon the membranous labyrinth is an uncommon, but often catastrophic, complication of chronic ear surgery. Areas most vulnerable to injury are the oval window and the lateral semicircular canal. Methods of injury include subluxation of the footplate, fracture of the lateral canal by drill or chisel, and accidental opening of a fistula. Three cases of labyrinthotomy of the lateral canal during ear surgery are presented. Two occurred while drilling, and the other was associated with a dehiscence of the bony and membranous labyrinth from a cholesteatoma. The cases are documented by pre and postoperative audiograms, vestibular function tests, and photographs. Of interest is the fact that in none of these cases was cochlear function compromised. In one case the hearing actually improved. The dictum that an accidentally opened labyrinth will automatically result in a dead ear is shown to be not necessarily true. Possible explanations for this phenomenon are discussed.

Adult↗

Eye tracking abnormalities in patients with cerebrovascular disease.

Ten patients with disequilibrium, ataxia, and cerebrovascular abnormalities were studied using computer aided eye tracking and vestibular function tests. The patients had severe abnormalities in smooth pursuit (decreased gain) and saccade tests (increased delay, decreased accuracy). Optokinetic responses were less affected. The gain of the vestibulo-oculomotor reflex was sometimes affected. Patients with eye tracking abnormalities frequently had symptoms of difficulty reading and watching television. When the lesion was asymmetrical, abnormal eye tracking tests usually pointed to the side of the lesion. Computer aided eye tracking tests are a useful adjunct to the evaluation of the patient with suspected vascular disease. They help to locate areas of central nervous system dysfunction, and produce an objective measurement of the severity of impairment. Conversely, when impairment of the smooth pursuit or saccade system is detected in patients being evaluated for disequilibrium, an assessment of the patients' cerebrovascular system should be considered.

Adult↗

The diagnostic value of cerebellar-vestibular tests in detecting learning disabilities, dyslexia, and attention deficit disorder.

Neurological and optokinetic measures of cerebellar-vestibular (CV) dysfunctioning were shown to be of significant diagnostic value in differentiating between learning disabled subjects and controls matched for chronological age, sex, handedness, IQ, and background (ns = 35). Although traditionally used electronystagmographic positional and caloric parameters were not similarly discriminating, quantitative measures of vertical nystagmus in various eyes-closed positions appeared to have diagnostic potential and were related significantly to such CV-determined neurological signs as positive monopedal Romberg. As a substantial majority of learning disabled (82.9%) evidenced ADD-like symptoms and since learning disabled subsamples with and without Attention Deficit Disorder (ADD) shared similar coexisting symptoms and CV signs, it appeared probable that learning disabilities and ADD were reflections of the same underlying CV determinants.

Adolescent↗