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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

846 children with cerebral palsy were examined and 642 were selected for a statistical study by correspondence analysis. The aim was to identify without any prior assumptions, the relationships between the aetiological factors and the clinical findings. The study was completed by conventional statistical analysis of 584 of the cases. Small birth weight and a history of abnormal pregnancies was associated with a cerebral palsy affecting both legs, and often with a squint. Resuscitation was associated with athetosis and abnormalities of posture and behaviour. If the resuscitation lasted for more than 15 minutes or there were certain problems during delivery, severe abnormalities of both arms and major speech difficulties were observed. Resuscitation for less than 15 minutes or for an unknown time or intractable vomiting during pregnancy was associated with quadraplegia. Hemiplegia was related to post natal events but the aetiology was not always known. Foeto-maternal incompatibility was associated with athetosis, deafness, severe speech problems and ophthalmoplegias.
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PMID:[Statistical study of the relationships between the etiology and clinical picture of cerebral palsy]. 10 77

The authors present a case of an 11-month-old girl with the medial cleft face syndrome and the following malformations: ocular hyperterolism, primary telecanthus, cleft nose with absent tip, broad nasal root, complete absence of the left upper lid, and abnormal hair-line implantation in the corresponding frontal region, high arched palate, neurosensorial deafness and agenesia of the right kidney. The authors propose that the eyelid is responsible for the area of hair growth suppression around the eyes. This is the first case report of median cleft face syndrome associated with renal agenesia.
J Pediatr Ophthalmol Strabismus
PMID:Median cleft face syndrome or frontonasal dysplasia: a case report with associated kidney malformation. 43 26

Duane's retraction syndrome is a well known congenital musculo-facial anomaly. Various explanations have been given for the aetiology of this syndrome. Inverse Duane's retraction syndrome is a condition with reverse clinical features. Abduction of the affected eye is possible to some extent and is accompanied by retraction of the eyeball, narrowing of the palpebral fissure and pseudoptosis. There may be some restriction of movement on adduction. The primary lesion is suspected to be in the medial rectus muscle. Frequently the muscle is found to be entrapped following trauma to the medial wall of the orbit. A case of bilateral inverse Duane's retraction syndrome and convergent squint along with left-sided perceptive deafness is reported. As is usually the case there was no structural abnormality or entrapment of the muscle from trauma.
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PMID:Bilateral inverse Duane's retraction syndrome--a case report. 181 Aug 84

Endemic cretinism is the most severe manifestation of dietary iodine deficiency. Two forms of the syndrome are traditionally described: neurological and myxoedematous. Although this classification highlights the important neurological sequelae of the disorder it implies that myxoedematous cretins have an alternative mechanism. Further, the nature of the neurological deficit associated with both types of endemic cretinism has received scant attention in recent times considering that it remains a common disorder in many parts of the world. The nature and extent of the neurological deficit found in endemic cretinism was investigated in 104 cretins from a predominantly myxoedematous endemia in western China and in 35 cretins from central Java, Indonesia, a predominantly neurological endemia. We found a similar pattern of neurological involvement in nearly all cretins from both endemias, regardless of type (myxoedematous or neurological), and of current thyroid function. Hallmarks of the neurological features included mental retardation, pyramidal signs in a proximal distribution and extrapyramidal signs. Many patients exhibited a characteristic gait. This probably reflected pyramidal and extrapyramidal dysfunction, although joint laxity and deformity were important contributing factors. Other frequently encountered clinical features were squint, deafness, and primitive reflexes. Cerebral computerized tomography (CT) revealed basal ganglia calcification in 15 of 50 subjects. The presence of basal ganglia calcification was confined to cretins with severe hypothyroidism. Otherwise, cerebral CT scanning demonstrated only minor abnormalities which did not contribute to the localization of the clinical deficits. We conclude that the same neurological disorder is present in both types of endemic cretinism reflecting a diffuse insult to the developing fetal nervous system. These clinical findings support the concept of maternal and fetal hypothyroxinaemia, arising from severe iodine deficiency, as the primary pathophysiological event in endemic cretinism. Differences between the two types of cretinism may be explained by continuing postnatal thyroid hormone deficiency in the myxoedematous type, which results in impaired growth, skeletal retardation and sexual immaturity.
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PMID:The neurology of endemic cretinism. A study of two endemias. 204 52

Two patients with Moebius' syndrome are described, a brother and a sister. In addition to bilateral congenital facial nerve paralysis, both had deafness, an external deformity of the auricles, and a latent squint. The deafness in both cases was sensorineural in type; although there was paralysis of the motor part of the facial nerve, taste and lacrimation were intact. This report shows that not all components of the facial nerve are always effected in this syndrome.
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PMID:Moebius' syndrome: a report of two cases. 338 27

A retrospective study of oculovisual assessment records of the population of three Ontario schools for deaf children assessed the differential effects of various causes of deafness on the prevalence of vision anomalies. Inherited deafness (ID) appears to be associated with the fewest visual anomalies. Congenital rubella (CR) had the greatest prevalence and was associated with the broadest spectrum of ocular and visual problems. It appears to have a significant effect on corneal curvature as well as being associated with anomalies of other organ systems. Children who had had CR, neonatal sepsis (NS), and Rh incompatibility (Rh) all showed higher rates of strabismus and amblyopia. Those who were deaf from meningitis (MEN) or NS showed a tendency to be more hyperopic, whereas the sample with Rh showed a trend toward myopia. The CR children had the broadest range of spherical refractive errors of all the causes of deafness. Children in schools for the deaf are generally those with substantial or profound hearing loss and thus are more likely to have an accompanying vision impairment. Knowledge of the effect on vision of the causes of deafness should aid practitioners in identifying and detecting similar effects on vision when mild or moderate deafness does not require a child's attendance at schools for the deaf.
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PMID:Differential effects of various causes of deafness on the eyes, refractive errors, and vision of children. 376 97

We experienced two cases of "Osteosclerosis" who were 12 and 15 year old sisters. Previous reported cases of this disease are 50 cases and among them only one patient was reported in Japan. Osteosclerosis seems to be inherited as an autosomal recessive trait. Parental consanguinity is also observed. The peculiar facies are evident in infancy, characterized by broad, flat nasal bridge, ocular hypertelorism and prognathic, broadened mandible. Commonly, they have cutaneous syndactylies in bilateral hands and feet, especially between the second and third finger and toe. Roentgenographically, hyperostosis with osteosclerosis can be observed in systemic bones, particularly the calvarium is greatly thickened. Since such a bony change occurs most severely at the base of the skull, important clinical symptoms of this disease are cranial nerve palsies resulting from obliterations of unilateral or bilateral several cranial nerve foramina. In many cases deafness due to progressive encroachment upon the middle ear cavities and auditory nerve canals appears early in infancy. Transient palsy of the facial nerve occurs somewhat later, and bilateral facial paralyses are usually permanent in adulthood. In some cases optic atrophy and visual field defect due to compression of the optic nerves are late complications. Other ocular symptoms are strabismus, nystagmus and exophthalmos. Anosmia and trigeminal nerve palsy are less common. Lower cranial nerve symptoms can not be noted but the reason is unclear. Chronic headache, convulsion and mental retardation are occasionally present. They are considered as a result from increased intracranial pressure due to progressive diminution of the cranial capacity. By same mechanism, several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum in early adulthood. Then, some reporter puts emphasis on prophylactic opening of the foramen magnum in all adult cases.
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PMID:[Sibling case of osteosclerosis with cranial nerve symptoms]. 629 11

Wildervanck syndrome is a combination of congenital anomalies characterized by deafness, Klippel-Feil deformity, and an unusual ocular motility disturbance called Duane retraction syndrome. This syndrome is seen infrequently in the general population and may not be recognized since the external appearance of the ears may be normal, and the unusual form of strabismus may not be obvious to the non-ophthalmologist. Patients with the full extent of this triad have a unique appearance. CT examination in these patients delineates the cervical anomalies as well as the inner ear deformity.
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PMID:Wildervanck syndrome--the external appearance and radiologic findings. 648 Feb 41

The sound localisation ability of four groups of severely deaf children is reported. It was found that those children who had some early experience of binaurally presented sound were likely to retain the ability to localise sounds, although if such experience was via binaural hearing aids, it was not certain to lead to retention of the ability. Children with early experience of sound which lacked interaural cues were likely to have lost the ability to localise, whether the experience was due to a severe asymmetric deafness or early single aid provision. The parallel with the loss of stereopsis in children with an uncorrected strabismus is drawn.
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PMID:Sound localization and early binaural experience in the deaf. 738 87

To determine the nature and prevalence of ophthalmologic abnormalities in children with congenital or prelinguistic sensorineural deafness, we performed complete ophthalmologic examinations on 54 children aged 2 to 14 years from the University of Miami Ear Institute's Cochlear Implant Program. Of 54 children, 33 (61.1%) had some form of ophthalmologic abnormality, with the majority (24 patients; 44.4%) being refractive errors. Of 54 patients, two (3.7%) had strabismus and two (3.7%) had external adnexal anomalies. One child (1.8%) had cataracts. The cause of the deafness, which had previously been unknown, was determined in three of 54 cases (5.6%) as a result of ocular examinations documenting rubella retinopathy, tapetoretinal degeneration (Usher's syndrome), and iris heterochromia (Waardenburg's syndrome). Routine ophthalmologic examination must be mandated for this population, and the ophthalmologist should be encouraged to play a broader role in the care of children with deficiencies of both vision and audition.
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PMID:Ophthalmologic abnormalities in the pediatric cochlear implant population. 802 78


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