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9/26/2014  |   4:30 PM - 6:00 PM   |  

Ophthalmologic Findings in Patients with Cytomegalovirus-Induced Sensorineural Hearing Loss

Introduction: Cytomegalovirus (CMV) is the most common congenital viral infection in the United States affecting 30-40,000 neonates annually. The most common disability from congenital CMV infection is sensorineural hearing loss (SNHL), but patients can also experience neurodevelopmental delay and ophthalmologic sequelae. Recent evidence suggests that SNHL due to congenital CMV may be more common than previously thought. There is no consensus on ophthalmologic screening of patients with CMV-induced SNHL. This study looks at the ophthalmologic findings in patients with CMV-induced SNHL. Methods: A retrospective review of the charts of children 3 years and younger who presented to one otolaryngologist between May 2008 and September 2013 with SNHL was performed. Eighty-three of 111 children underwent CMV testing, imaging and genetic evaluation. Twenty-five (30%) patients were diagnosed with confirmed or probable CMV-induced SNHL. Results: Sixteen (64%) patients underwent ophthalmologic examination. All sixteen had normal in-office examinations without evidence of active or prior chorioretinitis or optic atrophy. Age at time of examination ranged from 2 months to 5 years. All but one patient had normal visual behavior (fixation or optotype acuity) for age. One patient had intermittent strabismus that improved with time and glasses wear. One patient had poor visual behavior secondary to cortical visual impairment and a large-angle exotropia. Conclusion: Severe bilateral vision impairment secondary to CMV is most commonly caused by optic atrophy and cortical visual impairment. Retinal scarring and strabismus can also lead to vision loss in patients with congenital CMV. This review of 16 patients who presented for evaluation of CMV-induced SNHL found only one patient with visual loss secondary to CMV. Given reports of late-onset of chorioretinitis and the known risk of progressive SNHL, parents should be advised of these risks and followed over time.

Marielle Young (Primary Presenter,Author), marielle.young@hsc.utah.edu;
Marielle Young graduated from Yale University with a dual degree in History and International Studies. A native of Salt Lake City, she returned to pursue a medical degree at the University of Utah School of Medicine. She went on to complete ophthalmology residency training at the University of California, San Francisco and a fellowship in pediatric ophthalmology and adult strabismus at the Children’s Hospital Boston where she was a Heed Fellow. Her academic interests include the study of amblyopia and infantile cataracts.

ASHA DISCLOSURE:

Financial - No relevant financial relationship exist.

Nonfinancial - No relevant nonfinancial relationship exist.

Robert Hoffman (Author), robert.hoffman@hsc.utah.edu;
Robert O. Hoffman is Chief of the Division of Pediatric Ophthalmology and Eye Muscle Disorders. Dr. Hoffman has special interests in retinopathy of prematurity, ocular problems associated with genetics, craniofacial disorders, cataracts in infants and children and complicated strabismus.

Albert Park (Author), albert.park@imail.org;
Albert Park, MD, serves as the section chief of pediatric otolaryngology and professor within the Department of Surgery and the Department of Pediatrics at the University of Utah. Dr. Park obtained his MD at the Washington University School of Medicine. He then completed his residency training at Loyola University Medical Center and pediatric otolaryngology fellowship training at the Hospital for Sick Children in Toronto, Canada. His primary research interest focuses on understanding the pathophysiology of congenital cytomegalovirus (CMV) induced hearing loss and developing novel assays for diagnosis.

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