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Many Children With Sensorineural Hearing Loss Also Have Ocular Disorders CHICAGO -- February 16, 2009 -- About one-fifth of children with sensorineural hearing loss also have ocular disorders, according to a study published in the February issue of Archives of Otolaryngology-Head & Neck Surgery. "Especially early in life, sensorineural hearing loss is associated with delays in language, speech, cognitive and social development," the authors write. "Given the effects of hearing impairment, children with sensorineural hearing loss are particularly dependent on other means of information acquisition. If these children were to have unrecognized ophthalmologic abnormalities that limited visual acuity, there could be further detrimental effects on development." Arun Sharma, MD, University of Washington, Seattle, Washington, and colleagues reviewed ophthalmologic findings in 226 patients with sensorineural hearing loss who were seen at a children's hospital between 2000 and 2007. Of these, 49 (21.7%) had an ophthalmologic abnormality, including 23 (10.2%) with refractive errors (including nearsightedness, farsightedness, and astigmatism), and 29 (12.8%) with non-refractive errors. The cause of sensorineural hearing loss was syndromic in 11 patients (4.9%, and 5 (2.2%) had syndromes with related eye problems. All participants were offered genetic testing for mutations in GJB2. Of the 144 patients who underwent this screening, 27 (18.8%) had 2 mutated copies of the GJB2 gene, and 1 (3.7%) of those had ophthalmologic abnormalities. This compares with none of the 11 patients with a single copy of the mutated gene and 22 of 106 patients (20.8%) with no mutations. "This is consistent with the impression that GJB2 mutations result in sensorineural hearing loss but not in additional anomalies or syndromes," the authors wrote. "A multidisciplinary approach is important in the evaluation and treatment of children with sensorineural hearing loss to ensure that their medical, education, and social needs are met." "Ophthalmologic evaluation can be beneficial for patients by allowing ophthalmologists to diagnose co-existing disorders that affect vision and by helping otolaryngologists to determine the cause of sensorineural hearing loss," they concluded. SOURCE: Archives of Otolaryngology-Head & Neck Surgery E-mail this page to a friend or colleague! To print, use this version