Eye Abnormalities Associated With Sensorineural Hearing Loss

Abstract

Otolaryngology - Head & Neck Surgery| July 01 2009 Eye Abnormalities Associated With Sensorineural Hearing Loss AAP Grand Rounds (2009) 22 (1): 4. https://doi.org/10.1542/gr.22-1-4 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Eye Abnormalities Associated With Sensorineural Hearing Loss. AAP Grand Rounds July 2009; 22 (1): 4. https://doi.org/10.1542/gr.22-1-4 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: eye abnormalities, sensorineural hearing loss Source: Sharma A, Ruscetta MN, Chi DH. Ophthalmologic findings in children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 2009;135(2):119–123; doi:10.1001/archoto.2008.546 To determine the prevalence of ophthalmologic abnormalities in children with sensorineural hearing loss (SNHL), investigators at the University of Washington, Seattle, and University of Pittsburgh retrospectively reviewed the charts of children aged 18 years and younger seen at a tertiary care university children’s hospital between November 2000 and June 2007 for SNHL. All children with SNHL for whom results of ophthalmologic evaluation were available were included in the study. Severity of hearing loss assessed by audiometric evaluation was defined as follows: mild, 26 to 40 dB; moderate, 41 to 55 dB; moderately severe, 56 to 70 dB; severe, 71 to 90 dB; and profound, greater than 90 dB. Patients in the study were offered genetic testing for mutations in the GJB2 gene, which codes for connexin 26, a gap junction protein found within the spiral ligament of the cochlea, and is responsible for about 50% of all case of autosomal recessive, non-syndromic hearing loss. The study population consisted of 226 children with a mean age of 37 months. Males and females were equally represented. Hearing loss was mild in 37 patients (16.4%), moderate in 58 (25.7%), moderately severe in 20 (8.8%), severe in 36 (15.9%), and profound in 75 (33.2%). SNHL was bilateral in 174 patients (76.9%). Of the 226 children, 49 (21.7%) were diagnosed as having an ophthalmologic abnormality. Refractive errors (ie, myopia, hyperopia, and/or astigmatism) were present in 23 patients (10.2%). Non-refractive conditions such as strabismus and retinitis pigmentosa were diagnosed in 29 patients (12.8%). There was no difference in the prevalence of ocular anomalies based on severity of SNHL, nor between children with unilateral or bilateral SNHL. Of the 144 patients who underwent GJB2 testing, 38 had abnormal results. No patients with single allele mutation (11/144) and only one patient with biallelic mutation (27/144) had any ophthalmologic abnormalities. The 106 patients with normal GJB2 test results (n=106) were more likely to have identifiable ophthalmologic abnormalities than those patients with biallelic GJB2 mutations (P=.04). Imaging by CT or MRI of the skull base was available in 216 patients. The most common inner ear anomaly detected was enlarged vestibular aqueduct (10.6%). No differences in prevalence of eye findings were noted between those with or without abnormalities on imaging. The authors noted that the prevalence of ophthalmologic findings in their cohort was significantly greater than that observed in the Baltimore Vision Screening Project (14% vs 21.7%; P =.02), which assessed a cohort of 285 elementary school children from inner-city Baltimore, MD, underscoring the importance of ophthalmologic examination of children with SNHL. The authors conclude that ophthalmologic findings in children with SNHL are common and that ophthalmologic examination can be beneficial in the evaluation of children with SNHL. Dr Gottschall has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a... You do not currently have access to this content.