September 2019 HSC Section 1 Congenital and Pediatric Problems

B.J. Liming et al. / International Journal of Pediatric Otorhinolaryngology 90 (2016) 251 e 258

for GJB2/GJB6 is negative. Diagnostic rates for single gene testing for GJB2/GJB6 vary signi fi cantly based on the patient's ethnicity, and do not outperform the diagnostic rates for comprehensive genetic testing. In cases where CGT is unavailable, single gene testing can be directed by the audiometric phenotype and ethnicity. The economics of genetic testing are complex. Hearing loss panels using NGS technologies are rarely covered by insurance while single gene testing is often covered. Costs vary signi fi cantly between institutions. Ultimately, a sound evidence base will drive insurance company policies towards better coverage for compre- hensive genetic testing.

It should be noted that some authors of this manuscript continue to support imaging as part of the initial diagnostic algo- rithm for bilateral sensorineural hearing loss, primarily to rule out enlarged vestibular aqueduct. However, the majority of authors agreed that it should not be a routine part of the diagnostic algo- rithm for bilateral symmetric sensorineural hearing loss. Most, but not all, of the authors recommend imaging prior to cochlear im- plantation. The majority favor MRI, but some use CT. Some in- stitutions perform MRI during the neonatal period under natural sleep, but most wait until 6 months of age and use sedation or general anesthesia.

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