Duanes Syndrome 0 Increasing Awareness of a Rare

Duane’s Syndrome 0 Increasing Awareness of a Rare Visual Impairment Anne Papalia-Berardi, Ph. D Associate Professor of Special Education Millersville University

Definition 1 Duane’s Syndrome (DS) is an eye movement disorder present at birth “Miswiring” of medial and lateral rectus muscles that move the eyes

Definition 2 DS is caused by improper development of sixth cranial nerve controlling lateral rectus muscle Irregular structure of a branch of third cranial nerve controlling medial rectus muscle DS is classified as strabismus Varies from other forms of strabismus Neurological rather than muscular origin

3 Classifications of Duane’s Syndrome Type 1 (70 -80%): Inability to moves eyes outward away from nose Type 2 (7%): Inability to moves eye inward toward nose Type 3 (15%): Inability to move eyes inward or outward Subtype A: Affected eye turned inward toward nose in primary gaze Subtype B: Affected eye turned outward toward ear in primary gaze Subtype C: Eyes in a straight primary position

Associated Conditions 4 In 70% of cases DS occurs alone, however other conditions are associated with DS Malformation of skeleton, ears, eyes, kidneys, and nervous system Sensorineural hearing loss Incidence of sensorineural hearing loss occurring with DS ranged from 1% to 11% Rate comparable to reported incidences of hearing impairment in infants at high risk and from neonatal intensive care units

Symptoms 5 Clinical presentation of DS is varied Characterized by Limited ability to move an eye inward towards nose, outward towards ear, or both Eyeball pulls into socket, and eye opening narrows Eye may move upward or downward during vertical movement in an A, V, or X pattern Head turn to maintain single binocular vision

Duane’s Syndrome- Type 3 6

Related Syndromes 7 DS can occur as part of several syndromes Okihiro's Forearm Syndrome malformation and hearing loss Wildervanck Fusion of neck vertebrae and hearing loss Holt-Oram Syndrome Abnormalities of upper limbs and heart Morning Syndrome Glory Syndrome Abnormalities of optic disc or "blind spot” Goldenhar Jaw, Syndrome cheek, and ear malformation usually on one side of face

Causes 8 Exact cause of DS is not known Many researchers believe DS results from a disturbance, either genetic or environmental, during embryonic development between third and eighth week of pregnancy DS is sporadic in 90% of cases and familial in up to 10% of cases In familial cases a genetic causes is suspected Both dominant and recessive forms of DS have been found

Prevalence 9 Rare disorder existing in 0. 1% of the general population Responsible for 1 -5% of strabismus cases More common in girls (60%) than boys (40%) Left eye three times more likely to be impacted than right eye Both eyes affected in 15% to 20% of cases

General Impact 10 DS cover a wide spectrum Symptoms and extent of impact vary Each case must be considered individually DS does not progress with age Impact may increase with age as other physical and visual factors change

Visual Impact 11 Impact similar to other forms of strabismus Intermittent double vision, depth perception difficulties, and limited peripheral vision Loss of vision in affected eye (amblyopia) Most people with DS eyes are aligned in primary position during childhood and infancy May contribute to misdiagnosis or under diagnosis Most childhood vision screening check acuity in each eye but do not assess binocular vision

Psychological & Behavioral Impact 12 Psychological Low self esteem Teasing/bullying due to physical appearance Behavioral Refusal to participate in certain activities Perceived lack of motivation Irritability Lack of direct eye contact during conversation Can be interpret as rudeness or defiance

Physical Impact 13 Head Turn Causes neck and back pain May result in lack of direct eye contact when interacting with others Eye strain Leads to headaches and perceptual fatigue Mobility Difficulty navigating environment due to double vision and limited peripheral vision

Treatment 14 There is no cure for DS Surgery Recommended when eyes are misaligned in primary position and/or a significant head turn is noted Does not significantly impact vision Most people learn to compensate by ignoring double images

Accommodations 15 Children with DS benefit from a variety of accommodations depending on extent of impact Teacher and peer education about DS Preferential seating Sit on the side of the room of the non-affected eye Enlarged print and easy to read font Extra time during reading and tests involving reading Ruler or guide under text while reading Placement of book, worksheet, or computer when sharing with another student or reading to teacher Assistance technology Print to speech features

For More Information on DS 16 Genome. gov – Learning About Duane’s Syndrome www. genome. gov/11508984 Duanes: Duane’s Retraction Syndrome groups. yahoo. com/list/duanes/ Engle Laboratory – Learn About Duane’s Syndrome http: //www. childrenshospital. org/cfapps/researc h/data_admin/Site 339/mainpage. S 339 P 18 sublevel 15. html