Biomaterial Database

Congenital horizontal gaze paralysis and ear dysplasia--a syndrome. 1983

A B Safran, and A Roth, and C A Haenggeli

A 9-year-old boy with cyanotic congenital heart disease demonstrated congenital horizontal gaze paralysis and dysplasia of the left external and middle ear. Another patient associating congenital horizontal gaze paralysis and left hemifacial atrophy including ear dysplasia had previously been reported. The coincidence of such rare dysgenetic phenomena as congenital horizontal gaze paralysis and ear dysplasia in 2 patients seems to be significant. Since patients with congenital horizontal gaze paralysis often replace lateral gaze by convergence spasms, gaze paralysis sometimes remains undiagnosed. Therefore, when ocular motor disturbance is associated with ear dysplasia, the possibility of congenital horizontal gaze paralysis should be considered.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009886	Ophthalmoplegia	Paralysis of one or more of the ocular muscles due to disorders of the eye muscles, neuromuscular junction, supporting soft tissue, tendons, or innervation to the muscles.	Oculomotor Paralysis,External Ophthalmoplegia,Internal Ophthalmoplegia,Ophthalmoparesis,External Ophthalmoplegias,Internal Ophthalmoplegias,Ophthalmopareses,Ophthalmoplegia, External,Ophthalmoplegia, Internal,Ophthalmoplegias,Ophthalmoplegias, External,Ophthalmoplegias, Internal,Paralysis, Oculomotor
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D004431	Ear, External	The outer part of the hearing system of the body. It includes the shell-like EAR AURICLE which collects sound, and the EXTERNAL EAR CANAL, the TYMPANIC MEMBRANE, and the EXTERNAL EAR CARTILAGES.	External Ear,Outer Ear,Ear, Outer,Ears, External,Ears, Outer,External Ears,Outer Ears
D004432	Ear, Middle	The space and structures directly internal to the TYMPANIC MEMBRANE and external to the inner ear (LABYRINTH). Its major components include the AUDITORY OSSICLES and the EUSTACHIAN TUBE that connects the cavity of middle ear (tympanic cavity) to the upper part of the throat.	Tympanic Cavity,Tympanum,Middle Ear,Cavities, Tympanic,Cavity, Tympanic,Ears, Middle,Middle Ears,Tympanic Cavities,Tympanums
D005133	Eye Movements	Voluntary or reflex-controlled movements of the eye.	Eye Movement,Movement, Eye,Movements, Eye
D006330	Heart Defects, Congenital	Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life.	Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015	Abnormalities, Multiple	Congenital abnormalities that affect more than one organ or body structure.	Multiple Abnormalities
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes