Hearing Loss in the Cervico-oculo-acoustic (Wildervanck) Syndrome

Cremers, C.W.R.J.; Hoogland, Gerrit A.; Kuÿpers, W.

doi:10.1001/archotol.1984.00800270058015

Cited by 35 publications

(8 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…1 There has been no report of chromosomal aberrations in Wildervanck syndrome, and the small percentage of 45X observed in the chromosomes of this patient are probably age-associated aneuploidy. 2 Wildevanck syndrome consists of Klippel-Feil syndrome, Duane syndrome, and congenital sensorineural hearing disturbance.…”

Section: Discussionmentioning

confidence: 71%

Ruptured Aneurysm of the Sinus of Valsalva With Wildervanck Syndrome (Cervico-Oculo-Acoustic Syndrome), Blepharoptosis and Short Stature Case Report

Mori

Konno

et al. 2007

Circ J

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 71%

Ruptured Aneurysm of the Sinus of Valsalva With Wildervanck Syndrome (Cervico-Oculo-Acoustic Syndrome), Blepharoptosis and Short Stature Case Report

Mori

Konno

et al. 2007

Circ J

View full text Add to dashboard Cite

“…Wildervanck, in an extensive review of the syndrome, favoured polygenic inheritance with limitation to females ( Wildervanck, 1978). Although this disorder is to a large extent limited to females, males with Wildervanck syndrome (with and without optic nerve head coloboma) have been reported ( Cremers et al ., 1984 ; Regenbogen and Godel, 1985). The deafness in Wildervanck syndrome (which may account for up to 1% of female deafness and can be unilateral or bilateral) is congenital, usually severe and sensorineural in type but can also be due to ossicular chain anomalies ( Cremers et al ., 1984 ).…”

Section: Familial and Environmental Factorsmentioning

confidence: 99%

Duane’s syndrome

Gutowski

2000

Euro J of Neurology

View full text Add to dashboard Cite

Duane's syndrome is an unusual congenital form of strabismus where there is paradoxical anomalous lateral rectus innervation of the affected eye due to misdirection of axons destined for the medial rectus. Three types of Duane's syndrome are recognized. Most cases of Duane's syndrome are sporadic but up to 10% are familial, usually with autosomal dominant inheritance. Several autosomal dominant syndromes with dysmorphic features are associated with Duane's syndrome. Chromosomal loci for genes contributing to Duane's syndrome have been suggested at 4q, 8q and 22q. Duane's syndrome is heterogeneous at multiple levels with variations in its ocular manifestations, accompanying systemic manifestations and in the chromosomal loci with which it may be associated. The definition clinically and genetically of the various subgroups of Duane's syndrome will provide a valuable insight into brainstem axonal guidance to the extraocular muscles during human development.

show abstract

“…The hearing loss may be sensorineural, conductive, or mixed in Wildervanck syndrome, and these patients may have malformations of external auditory canal, external acoustic meatus, ossicles and bony labyrinth (9,10). The present case also had atresia of right external auditory canal and anomalies of mastoid bone and ossicles, whereas the hearing in the right ear was normal.…”

Section: Discussionmentioning

confidence: 57%

An Uncommon Cause of Duane Syndrome in a Child: Wildervanck Syndrome

Anık¹,

Dursun²,

Polat³

et al. 2016

jpr

View full text Add to dashboard Cite

Duane syndrome is a congenital eye movement disorder characterized by limitation of abduction and/or adduction accompanied with lid fissure narrowing, and globe retraction in attempted adduction. Duane syndrome is mostly an isolated entity, but systemic anomalies have been reported in 6-10% of patients. Wildervanck syndrome (cervico-oculo-acoustic syndrome) is a very rare disease comprised of the triad of Klippel-Feil deformity (congenitally fused cervical vertebrae), Duane syndrome, and hearing loss. The disorder is limited, or almost completely limited, to females, raising the question of sex-linked dominance with lethality in the hemizygous male. Herein, we describe an adolescent girl who was admitted to our clinic for obesity and diagnosed with Wildervanck syndrome due to bilateral abducens paralysis (Duane syndrome), Klippel-Feil deformity and hearing loss.

show abstract

Hearing Loss in the Cervico-oculo-acoustic (Wildervanck) Syndrome

Cited by 35 publications

References 15 publications

Ruptured Aneurysm of the Sinus of Valsalva With Wildervanck Syndrome (Cervico-Oculo-Acoustic Syndrome), Blepharoptosis and Short Stature Case Report

Ruptured Aneurysm of the Sinus of Valsalva With Wildervanck Syndrome (Cervico-Oculo-Acoustic Syndrome), Blepharoptosis and Short Stature Case Report

Duane’s syndrome

An Uncommon Cause of Duane Syndrome in a Child: Wildervanck Syndrome

Contact Info

Product

Resources

About