Ophthalmologic disorders in children with syndromic and nonsyndromic hearing loss.
OBJECTIVE
To determine the rate of ophthalmologic anomalies among patients with syndromic and nonsyndromic, congenital sensorineural hearing loss (SNHL) to assess the need for comprehensive ophthalmologic evaluation in these children.


METHODS
Retrospective medical chart review of children with SNHL who underwent comprehensive evaluation by pediatric ophthalmologists and geneticists.


METHODS
Tertiary care pediatric hospital.


METHODS
Seventy-seven patients with SNHL.


METHODS
Degree of hearing loss (HL) and presence of ophthalmologic and genetic disorders.


RESULTS
The overall rate of ophthalmologic disorders was 32% (25 of 77 patients). When children with multisystem genetic disorders known to be related to visual loss were excluded, the rate fell to 23% (12 of 53 vs 13 of 24; P = .006). There was no statistically significant difference in the degree of HL between patients with and without eye disorders (mean [SD], 46.5 [29.9] vs 49.1 [32.3] dB HL; P = .75). Patients with eye disorders were significantly more likely to have a multisystem genetic disorder (13 of 25 [52%] vs 11 of 52 [21%]; P = .006). No patients with ocular abnormalities had isolated otologic disorders, but 9 of 52 (17%) of those patients without ocular abnormalities did.


CONCLUSIONS
Comprehensive ophthalmologic examination revealed a rate of ophthalmologic disorders in children with SNHL in the lower end of the previously reported rates of 31% to 61%. Children with nonsyndromic SNHL have an approximately 2- to 3-fold increase in ocular abnormalities compared with the general pediatric population. Ophthalmologic and genetic consultations are warranted in patients with congenital SNHL.
Johnston
Douglas R
DR
Department of Otolaryngology-Head and Neck Surgery, Alfred I. duPont Hospital for Children, Wilmington, Delaware, USA.
Curry
Joseph M
JM
Newborough
Brian
B
Morlet
Thierry
T
Bartoshesky
Louis
L
Lehman
Sharon
S
Ennis
Sara
S
O'Reilly
Robert C
RC
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000235:genetics; D001299:Audiometry; D002648:Child; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D005128:Eye Diseases / Q000150:complications*; D005260:Female; D005820:Genetic Testing; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000151:congenital*; D006801:Humans; D008297:Male; D009154:Mutation; D010375:Pedigree; D012189:Retrospective Studies
D017630:Connexins; D000072259:Connexin 26