Comprehensive diagnostic battery for evaluating sensorineural hearing loss in children.
OBJECTIVE
Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child.


METHODS
Retrospective review.


METHODS
Tertiary care university hospital.


METHODS
A total of 270 children referred for severe to profound SNHL between January 2002 and June 2009.


METHODS
Results of the following were reviewed: magnetic resonance imaging, computed tomography, renal ultrasound, electrocardiography, fluorescent treponemal antibody absorption test, connexin 26 sequencing, genetic consultation, and ophthalmologic consultation.


METHODS
Diagnostic yield of each test was determined.


RESULTS
Each diagnostic test or consultation was completed by at least 95% of patients for whom it was ordered. Magnetic resonance imaging revealed abnormalities explaining SNHL in 24% of patients. Computed tomography showed inner ear anomalies in 18% of patients. Biallelic connexin 26 mutations were found in 15%. Renal ultrasound found anomalies in 4% of patients. Electrocardiography found 1% of patients with prolonged QT intervals. Fluorescent treponemal antibody absorption test result was positive in 0.5%. Genetic consultation found a genetic cause for hearing loss in 25%. Ophthalmologic consultation found abnormalities associated with hearing loss in 8%.


CONCLUSIONS
Diagnostic radiologic imaging is the highest yielding test for evaluating children with SNHL. Connexin 26 sequencing identifies a nearly nonoverlapping subset of children compared with imaging. Specialty consultations, particularly from a clinical geneticist, can improve diagnostic yield. Other tests, although of lower diagnostic yield for SNHL, can identify important diseases that significantly affect patient health.
The Bobby R. Alford Department of Otolaryngology - Head and Neck Surgery, Baylor College of Medicine and The Hearing Center at Texas Children's Hospital, Houston, Texas, USA.
Jerry W
JW
Lin
Naweed
N
Chowdhury
Avni
A
Mody
Ross
R
Tonini
Claudia
C
Emery
Jody
J
Haymond
John S
JS
Oghalai
D001921:Brain / Q000473:pathology; D002648:Child; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D004424:Ear Canal; D004562:Electrocardiography; D005260:Female; D016508:Fluorescent Treponemal Antibody-Absorption Test; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D006320:Hearing Tests; D006801:Humans; D007668:Kidney / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014465:Ultrasonics; D014463:Ultrasonography; D014786:Vision Disorders / Q000150:complications; D014787:Vision Tests
D017630:Connexins; D000072259:Connexin 26