|Year : 2012 | Volume
| Issue : 2 | Page : 73-74
Duane’s retraction syndrome associated with crocodile tears and hearing loss
Shao-Chun Chen, Chun-Chen Chen, Shiow-Wen Liou
Department of Ophthalmology, Taipei City Hospital, RenAi Branch, 2F., Number 10, Section 4, Ren’ai Road, Da’an District, Taipei City 106, Taiwan, ROC
|Date of Web Publication||19-May-2012|
Department of Ophthalmology, Taipei City Hospital, RenAi Branch, 2F., Number 10, Section 4, Ren’ai Road, Da’an District, Taipei City 106, Taiwan
Source of Support: None, Conflict of Interest: None
Duane’s retraction syndrome (DRS) is a predominately congenital eye movement disorder. We present a 23-year-old female with unilateral DRS, which was diagnosed by magnetic resonance imaging (MRI), and associated symptoms of unilateral crocodile tears and bilateral high-frequency hearing loss. This case highlights that DRS may be associated with various systemic malformations. For children with newly diagnosed DRS, we recommend comprehensive evaluation for any associated abnormalities. For adults with DRS complaining of discomfiture associated with a noncentral head position, we suggest a complete examination, including strabismus tests, the forced duction test, and even MRI, to evaluate the degree of muscle modification and to identify the most suitable treatment strategy.
Keywords: crocodile tears, Duane’s syndrome, strabismus
|How to cite this article:|
Chen SC, Chen CC, Liou SW. Duane’s retraction syndrome associated with crocodile tears and hearing loss. Taiwan J Ophthalmol 2012;2:73-4
|How to cite this URL:|
Chen SC, Chen CC, Liou SW. Duane’s retraction syndrome associated with crocodile tears and hearing loss. Taiwan J Ophthalmol [serial online] 2012 [cited 2020 Jul 9];2:73-4. Available from: http://www.e-tjo.org/text.asp?2012/2/2/73/203727
| 1. Introduction|| |
Duane’s retraction syndrome (DRS) is a predominately congenital eye movement disorder. We present the case of a patient with unilateral DRS, which was diagnosed by magnetic resonance imaging (MRI), and associated symptoms of unilateral crocodile tears and bilateral high-frequency hearing loss.
| 2. Case presentation|| |
A 23-year-old female had horizontal eyeball movement limitation of the right eye since birth. She also had small-angle exotropia of the right eye and abnormal head positioning in the primary position, with the face turned toward the involved eye. She was born by normal delivery at 36 weeks’ gestation. Her body growth and psychomotor development were normal. No family history of either DRS, or similar abnormalities, was reported. On ophthalmological evaluation, her best-corrected visual acuity was found to be 20/20 in both eyes. She had a myopic astigmatism of −0.5 Dsph and −0.5 Dcyl 130° in the right eye and −0.75 Dsph and −1.0 Dcyl 160° in the left eye. She had 8 prism diopters exotropia in the primary position for distance. She had −3 abduction deficit in the right eye on straight right gaze and −1 adduction deficit on straight left gaze. She had retraction of the globe and fissure-narrowing on adduction in the right eye, but did not show typical upshoots or downshoots of either eye on attempted adduction [Figure 1].
|Figure 1: Ocular versions demonstrating −3 abduction deficit in the right eye on straight right gaze and −1 adduction deficit on straight left gaze. Retraction of the globe and fissure narrowing are noted on adduction in the right eye.|
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With the patient under local anesthesia, the forced duction test showed limitation of both adduction and abduction of the right eye. Enophthalmos was also noted, with more severe globe retraction of the right eye during adduction. Her parents had noted profuse lacrimation from the right eye when eating, especially a cool drink or ice cream, since birth. Other neurological examinations were normal. However, audiological examination revealed bilateral high-frequency hearing loss without anatomical malformation.
High-resolution T2-weighted MRI of the brainstem revealed that the left abducens nerve entered Dorello’s canal and that the right abducens nerve was absent. The MRI also showed a smaller muscle volume of the right lateral rectus muscle compared to that in the left eye in the orbital view [Figure 2]. The patient was diagnosed with unilateral DRS, based on the following findings: limited abduction in the right eye, with retraction of the eyeball and narrowing of the palpebral fissure on adduction; dysgenesis of the right abducens nerve; and abundant lacrimation and high-frequency hearing loss.
|Figure 2: A magnetic resonance image shows the whole path of the left abducens nerve (arrow), as a linear dark structure emerging from the level of the pontomedullary junction. The right abducens nerve is not identified.|
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| 3. Discussion|| |
DRS is a predominately congenital eye movement disorder characterized by marked limitation or absence of abduction, restriction of adduction, retraction of the globe, contraction of the palpebral fissure, and upshoots or downshoots of the affected eye on adduction. These movements were originally believed to be caused by extraocular muscle fibrosis, but they have now been linked to abnormal innervation of the extraocular muscle, or absence or hypoplasia of the abducens nucleus with anomalous innervation of the lateral rectus muscle by branches of the oculomotor nerve. Absence of the abducens nerve on MRI can help distinguish DRS from clinical mimickers, such as chronic abducens palsy, and may assist in presurgical planning for strabismus correction. In some patients, particularly children, characteristic diagnostic signs of DRS may not be manifested, and thus MRI is utilized for the diagnosis of DRS. In our patient, a limitation, but not restriction, of right eye horizontal movement was observed. It seems that the right lateral rectus muscle still had some function in this patient, which may be paradoxically innervated by the branches of the oculomotor nerve.
Approximately 30% of individuals with DRS have other congenital anomalies, particularly of the ear, kidney, heart, upper limbs, and skeleton. These associated anomalies are typically reported in simplex cases, but also occur together with DRS as familial malformation or genetic syndromes. Abducens nuclei are known to develop between approximately the 5th and 8th week of human embryogenesis. Malformations of many kinds of organs that develop during this period are known to be associated with DRS. The auricle, external canal, and auditory ossicles develop at approximately the 8th week of embryogenesis from the second branchial arch. Kirkham found that 11% of 112 patients with DRS showed unilateral or bilateral sensorineural deafness. In the same study, a dominant trait with poor penetrance and variable expressivity was also found.
Congenital aberrant (anomalous) tearing describes unexpected alterations in lacrimation, such as tearing when eating, absence of emotional lacrimation, or unusual late-onset tearing. DRS, combined with crocodile tears, is more prevalent in Chinese than Western populations. A possible reason for the crocodile tears in DRS may be a lesion causing nuclear degeneration in the brain stem, or dysgenesis in the immediate vicinity of the abducens nucleus. The paradoxical effects of DRS and the lacrimal disorder are the result of substitute innervation of the lateral rectus by fibers from the oculomotor nerve and the lacrimal gland by the nerve fibers subserving salivation.
Although DRS combined with crocodile tears or hearing loss is not uncommon, DRS associated with both crocodile tears and hearing problems has only been reported once previously. In 1984, Tachibana reported the case of a 19-year-old woman with bilateral DRS associated with bilateral symmetrical mixed hearing loss and crocodile tears. The patient described here is the second reported case with concurrent DRS, crocodile tears, and hearing loss.
In conclusion, this report describes a patient with DRS combined with crocodile tears and bilateral high-frequency hearing loss. This case highlights that DRS may be associated with various systemic malformations. For children with newly diagnosed DRS, we recommend comprehensive evaluation for any associated abnormalities. For adults with DRS, complaining of discomfiture associated with a noncentral head position, we suggest a complete examination, including strabismus tests, the forced-duction test, and even MRI, to evaluate the degree of muscle modification, and to identify the most suitable treatment strategy.
| References|| |
Duane A. Congenital deficiency of abduction, associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol
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Tachibana M, Hoshino A, Oshima W, Nishimura H, Mizukoshi O. Duane’s syndrome associated with crocodile tear and ear malformation. A case of stapes anomaly and its reconstruction. Arch Otolaryngol
[Figure 1], [Figure 2]