Evaluation of vestibular functions in children with vertigo attacks

Aim: To examine vestibular system functions in children with episodic vertigo attacks. Methods: Thirty four children (20 males) aged 4–18 years with paroxysmal dizziness and/or vertigo attacks were evaluated. A medical history for vestibular symptoms and migraine was taken. Vestibular and auditory functions were assessed. Results: Chronic headache attacks consistent with migraine were reported in 12 children and motion sickness was reported in 30. Family history in first degree relatives was positive for migraine in 29 children and for episodic vertigo in 22. Electronystagmography and videonystagmography showed two types of nystagmus: spontaneous vestibular nystagmus (41%) and benign paroxysmal positional nystagmus (BPPN) (59%). The first type of nystagmus was assessed as a sign of vestibulopathy and the patients with BPPN were diagnosed as having benign paroxysmal positional vertigo (BPPV). Audiometric examination in four cases revealed bilateral sensory neural hearing loss in low frequencies. Pure tone averages in 30 cases were within normal ranges; however low frequencies in 28 of them were approximately 10 dB lower than high frequencies. Unilateral caloric responses diminished in eight children. Conclusions: Peripheral vestibular problems in childhood present in a wide spectrum, which varies from a short episode of dizziness to a typical vestibular attack with fluctuating sensory neural hearing loss or episodes of BPPV. A considerable number of these vestibular problems might be related to the migraine syndrome.     

Evaluation of vestibular functions in children with vertigo attacks.

AIM: To examine vestibular system functions in children with episodic vertigo attacks. METHODS: Thirty four children (20 males) aged 4-18 years with paroxysmal dizziness and/or vertigo attacks were evaluated. A medical history for vestibular symptoms and migraine was taken. Vestibular and auditory functions were assessed. RESULTS: Chronic headache attacks consistent with migraine were reported in 12 children and motion sickness was reported in 30. Family history in first degree relatives was positive for migraine in 29 children and for episodic vertigo in 22. Electronystagmography and videonystagmography showed two types of nystagmus: spontaneous vestibular nystagmus (41%) and benign paroxysmal positional nystagmus (BPPN) (59%). The first type of nystagmus was assessed as a sign of vestibulopathy and the patients with BPPN were diagnosed as having benign paroxysmal positional vertigo (BPPV). Audiometric examination in four cases revealed bilateral sensory neural hearing loss in low frequencies. Pure tone averages in 30 cases were within normal ranges; however low frequencies in 28 of them were approximately 10 dB lower than high frequencies. Unilateral caloric responses diminished in eight children. CONCLUSIONS: Peripheral vestibular problems in childhood present in a wide spectrum, which varies from a short episode of dizziness to a typical vestibular attack with fluctuating sensory neural hearing loss or episodes of BPPV. A considerable number of these vestibular problems might be related to the migraine syndrome.     

Infantile and acquired nystagmus in childhood

Nystagmus is an involuntary, periodic eye movement caused by a slow drift of fixation which is followed by a fast refixation saccade (jerk nystagmus) or a slow movement back to fixation (pendular nystagmus). In childhood most cases are benign forms of nystagmus: idiopathic infantile, ocular or latent nystagmus. They arise at the age of 3 months, without oscillopsia and show the absence of the physiologic opto-kinetic nystagmus. A full ophthalmologic evaluation is all that is needed in most cases: albinism, macular or optic nerve hypoplasia and congenital retinal dystrophies are the most common forms of ocular nystagmus. Idiopathic infantile nystagmus can be hereditary, the most common and best analyzed form being a mutation of the FRMD7 gene on chromosome Xq26.2. The mutation shows a mild genotype–phenotype correlation. In all female carriers the opto-kinetic nystagmus is absent and half had mild nystagmus. Latent nystagmus is part of the infantile esotropia syndrome and shows the unique feature of change of direction when the fixing eye changes: it is always beating to the side of the fixing eye. There is no cure for infantile nystagmus but therapeutic options include magnifying visual aids or eye muscle surgery at the age of 6–8 y in patients with head turn.Less than 20% of childhood nystagmus are acquired and need further neurological and imaging work-up. Alarming signs and symptoms are: onset after the age of 4 months, oscillopsia, dissociated (asymmetric) nystagmus, preserved opto-kinetic nystagmus, afferent pupillary defect, papilloedema and neurological symptoms like vertigo and nausea. The most common cause is due to pathology of the anterior optic pathway (e.g. optic nerve gliomas). It shows the same clinical feature of dissociated nystagmus as spasmus nutans but has a higher frequency as in INO. Other forms of acquired nystagmus are due to brainstem, cerebellar or metabolic diseases.     

Asymmetric horizontal tropias, DVD, and manifest latent nystagmus: an explanation of dissociated horizontal deviation

Four patients with dissociated horizontal deviation (DHD) are described and an explanation of their horizontal comitant deviations is offered. The patients had asymmetric comitant horizontal deviations dependent on the fixing eye. All of them had unequal visual acuity (VA) and asymmetric manifest latent nystagmus (MLN) documented with eye movement recordings. The subjects appear to use convergence to reduce their nystagmus when viewing with the eye having more severe nystagmus and hence poorer vision. When patients viewed with the eye having less nystagmus, little or no convergence was exhibited. We suggest that DHD may be the manifestation of an asymmetric nystagmus blockage syndrome (NBS). The dampening effect of convergence on nystagmus has been well documented in the past and seems to apply here. The patients demonstrate that a true, but asymmetric NBS, can be found with MLN.     

Acquired monocular nystagmus in monozygous twins

Twins girls presented with monocular nystagmus, head-nodding, and tilting in early infancy. This disorder resolved without therapy or residual defect. This case represents the first documented report of spasmus nutans presenting with only monocular nystagmus in twins. The etiology of spasmus nutans and monocular nystagmus is discussed in light of recent clinical and experimental observations.     

Transitory postural and positional nystagmus in children and its diagnostic significance

Among 587 children at an age from 5 to 14 years, examined in the university clinic of O.R.L. Essen in a 10 years period because of dizziness, 201 times the subjective sensations could be objectified by a vestibular nystagmus. After elimination of all cases with a pathologic ear state 39 times a posture or positioning nystagmus was recorded. The examination was regularly effected by a systematic search for spontaneous and provoked nystagmus by Frenzel's luminous glasses and was completed by an experimental excitability test of the labyrinth according to Hallpike. The nystagmus findings are divided form analytically in transitory directional appointed, irregular and regular direction changing posture resp. positioning nystagmus with and without latency or contrary course. An etio-pathogenetic explanation is given.     

Nutritional nystagmus in infants

Two infants presented with acquired infantile nystagmus. On examination, they manifested peculiar facies, growth and developmental delay, failure to thrive, and a Wernicke's type of nystagmus. Suspicion of a B vitamin deficiency resulted in correct diagnosis of dietary deficiency and/or enzymatic defect. In both instances, dietary treatment with B vitamins resulted in resolution of the nystagmus and improved growth and development of both individuals. Physicians, especially pediatricians and ophthalmologists, should be alert to the possibility of a Wernicke's type nystagmus in such infants, since the diagnosis may be critical to accurate diagnosis and treatment.     

THE RELATIONSHIP OF VESIBULAR RESPONSES AND DEVELOPMENTAL FUNCTIONS IN HIGH RISK INFANTS

The purposes of this study were to investigate the relationship of vestibular, developmental, and neurological functions in a sample of high risk infants, to determine the usefulness of the postrotary nystagmus test in a developmental evaluation clinic, and to test the hypothesis that high risk infants demonstrating suspect or abnormal neurological findings in conjunction with motor delays will exhibit nystagmus of less or greater duration than that seen in a sample of normal full-term infants on a postrotary nystagmus test. Vestibular responses, neurological functions, and motor and cognitive skills were evaluated in a sample of 55 high risk infants ranging in age from 3 to 24 months. The primary neonatal problems of the infants in the sample were low birth weight, prematurity, and respiratory distress syndrome. The mental and motor Bayley Scales of Infant Development, a neurological assessment, and the postrotary nystagmus test were administered to a sample of 20 normal full-term infants ranging in age from 3 to 17 months fro the purpose of comparing the duration of nystagmus with that found in the sample of high risk infants. Data analyses revealed that a significant proportion of high risk infants with abnormal or suspect neurological functions and with delayed motor skills exhibited nystagmus of lesser or greater duration than that seen in the normal infants. These findings strongly suggest the importance of assessment of vestibular responses using the postrotary nystagmus test in the developmental assessment of the high risk infant.     

Near-evoked nystagmus in spasmus nutans

Near-evoked nystagmus was evident in two children with spasmus nutans by clinical observation and electro-oculographic recording. In one child the nystagmus appeared to be evoked by fusional convergence and in the other by convergence-accommodation. These cases represent an atypical form of spasmus nutans in which the nystagmus is modulated by centers controlling visuomotor changes with near viewing.     

Acquired esotropia as initial manifestation of Arnold-Chiari malformation

A 13-year-old patient originally presented with a divergence palsy and gaze-evoked nystagmus. Over a short period of time, the esotropia became increasingly comitant and was successfully treated with strabismus surgery. Three years later, she developed downbeat nystagmus. An Arnold-Chiari Malformation could only be demonstrated using nuclear magnetic resonance imaging (MRI). Subsequent neurosurgical decompression resulted in resolution of the downbeat nystagmus with maintenance of single binocularity.     

Nystagmus im Kindesalter

The aim of this article is to familiarize the reader with the different types of infantile nystagmus. Common forms of early-onset and acquired nystagmus are described in detail. The main focus lies on the differential diagnostic aspects which will help to make the important differentiation between early-onset and acquired nystagmus. We depict the diagnostic steps of the different types and address the options for therapeutic measures.     

VISUAL MOTOR DIFFICULTIES IN EMOTIONALLY DISTURBED CHILDREN WITH HYPORESPONSIVE NYSTAGMUS

The investigators explored the relationship between hyporesponsive postrotary nystagmus and subnormal visual motor control skill in emotionally disturbed children. Twenty-three emotionally disturbed children were administered an array of psychological and neuropsychological examinations, including the Bender Visual Motor Gestalt Test, and Beery Visual Motor Integration Test, and the Southern California Postrotary Nystagmus Test. Children with short nystagmus durations appeared to exhibit problems in visual motor control. Complexities involving a number of extraneous variables necessitate caution in interpreting these results, but the data provide evidence of a relationship between vestibular function as measured by postrotary nystagmus duration and oculomotor control in children with emotional disorders.     

Habitual head turning in spasmus nutans: an oculographic study

Head turning in patients with spasmus nutans seems to transiently dampen the nystagmus. This was evident in two children by clinical observation and electrooculographic recording. The suppression of the nystagmus may have been mediated by the vestibular system.     

Developmental Status of Children Exhibiting Postrotatory Systagmus Durations of Zero Seconds

This research. Using a sample of 4 ½ year-old children identified during the first weeks of life as high risk, had two purposes. The first was to describe the developmental status of those children having Southern California Postrotary Nystagmus Test scores of zero seconds. The second was to compare the visual motor, gross motor, and cognitive development of the children with postrotatory nystagmus scores of zero seconds with children having nystagmus durations within a normal range. On all seven measures used in this research, the zero nystagmus group performed worse than the normal nystagmus group. On two measures, the Peabody Development Gross Motor Scale and scissor cutting errors, results were significant at p<.007. For three other measures, WPPSIVerbal, standing balance. And the Frostig Eye-Motor Coordination Subtest, results were significant at p<.05. Possible functional implication for children with zero nystagmus were discussed.     

Botulinum treatment of infantile esotropia with abduction nystagmus

PURPOSE: To examine the effect of botulinum toxin type A (BTA) on the final correction of esotropia, A and V patterns, overaction of the oblique muscles, and dissociated vertical deviation (DVD) in infantile esotropia with nystagmus in abduction. METHODS: This retrospective study examined 54 patients treated with simultaneous bilateral medial injection of BTA. Subjects were divided into two groups: group 1 (first injection <18 months of age) and group 2 (first injection >18 months of age). RESULTS: Pre-BTA, group 1 patients had an angle of esotropia noticeably higher and fewer A patterns than group 2 patients. Post-BTA, group 2 received significantly fewer injections of BTA than group 1. The magnitude of the A patterns improved. Prior frequency of DVD increased significantly in group 1 (100%): 79% of DVD was decompensated compared with 47% in group 2. Overall success was obtained in 14% and 58% of groups 1 and 2, respectively. CONCLUSION: We do not support BTA treatment in infantile esotropia with nystagmus in abduction prior to age 18 months. After 18 months, the horizontal results are excellent and neither the incidence nor the degree of previous DVD are decompensated, resulting in high success rates for overall deviation and improving anisotropy in A.     

Visual pathway abnormalities in albinism and infantile nystagmus: VECPs and stereoacuity measurements

The visual pathway of albinos is characterized by abnormal optic nerve fiber decussation with increased contralateral projection. This results in hemispheric asymmetry of monocular visually evoked cortical potentials (VECPs) and may be related to lack of stereopsis. Flash VECP, Random Dot E (RDE), and Titmus stereotest data were collected from 40 nystagmus patients, 19 albino and 21 with normal pigmentation. Data were compared with results obtained from 19 race- and age-matched normal subjects. All albino patients demonstrated hemispheric VECP asymmetries exceeding +/- 2.0 standard deviations from the normal mean, showing either delayed ipsilateral P latency (88%), reduced ipsilateral P amplitude (59%), or both (47%). None of the normally pigmented infantile nystagmus patients exceeded this criteria on either VECP measure. While only one albinotic patient showed positive RDE and stereofly results, 86% of normally pigmented nystagmus patients passed the stereofly test, 60% demonstrated 400 sec (Titmus) stereopsis, and 66% showed positive RDE results. These findings reveal that normally pigmented infantile nystagmus patients have variable degrees of stereopsis and no excessive abnormalities of optic nerve fiber decussation as evidenced by flash VECP. Additionally, this study verifies visual pathway abnormalities and the lack of stereopsis in albino nystagmus patients.     

Cone-rod dystrophy and acquired dissociated vertical nystagmus

Although usually a manifestation of vestibular or neurological disease, acquired nystagmus occasionally can be seen in ocular diseases such as ocular albinism and cone dystrophy. The present case of cone-rod dystrophy presenting with acquired dissociated vertical nystagmus probably demonstrates another unusual manifestation of cone-rod dystrophy.     

Congenital seesaw nystagmus

A 14-year-old boy with congenital seesaw and horizontal pendular nystagmus associated with decreased visual acuity, high myopia, esotropia, and normal peripheral visual fields is reported. Neurologic and computed tomographic evaluation, as well as auditory evoked potentials, were normal. The findings in, and recommended evaluation of, seesaw nystagmus in young patients are reviewed.     

Acute onset of dizziness caused by a cavernous malformation lateral to the fourth ventricle: a case report

Dizziness, diplopia, and nystagmus may be nonspecific symptoms and in part attributed to central causes. We report a case with brain stem lesion and discuss these nonspecific symptoms. A 13-year-old boy presented to our emergency department with a lasting dizziness for 1 week and diplopia for 5 days. New onset horizontal nystagmus was also noted during the physical examination. A cavernous malformation in the right middle cerebral peduncle lateral to the fourth ventricle was suspected by brain computed tomography and confirmed by magnetic resonance imaging. Without progression of the lesion, conservative treatment and regular follow-up were performed. This case report demonstrates the importance of primary physicians paying attention to children with dizziness, diplopia, and nystagmus for considering possible central nervous problems.