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Arun Sharma, MD; Melissa N. Ruscetta, PhD; David H. Chi, MD

Arch Otolaryngol Head Neck Surg. 2009;135(2):119-123.

Objectives  To determine the prevalence of refractive and nonrefractive ophthalmologic abnormalities in children with sensorineural hearing loss (SNHL) and to evaluate the overall utility of routine ophthalmological examination in children with SNHL.

Design  An institutional review board–approved retrospective analysis of ophthalmologic findings in children (18 years and younger) with SNHL seen between November 6, 2000, and June 4, 2007.

Setting  Tertiary care university children's hospital.

Patients  Children (18 years and younger) with SNHL.

Main Outcome Measures  Ophthalmologic findings and SNHL.

Results  There were 226 patients with SNHL who underwent ophthalmologic examination. Of these patients, 49 (21.7%) had an ophthalmologic abnormality. Refractive errors were present in 23 patients (10.2%), whereas nonrefractive conditions were present in 29 patients (12.8%). The cause of SNHL was syndromic in 11 patients (4.9%), of whom 5 (2.2%) had syndromes with associated ophthalmologic abnormalities. No statistically significant differences were found in ophthalmologic findings based on severity or laterality of SNHL. The prevalence of ophthalmologic findings was not significantly different between patients whose conditions were diagnosed on the basis of newborn screening and other patients. Ophthalmologic abnormalities were found in 1 of 27 patients (3.7%) with biallelic GJB2 mutations and 22 of 106 patients (20.7%) without any GJB2 mutations (P = .04).

Conclusions  In this study, the overall prevalence of ophthalmologic findings in children with SNHL was 21.7%. Ophthalmologic anomalies were present in 3.7% of children with GJB2 mutations. Routine ophthalmologic examination can be beneficial in the evaluation of children with SNHL.

Author Affiliations: Departments of Otolaryngology, University of Washington, Seattle (Dr Sharma), and The University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania (Drs Ruscetta and Chi).

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