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. 2016 Sep;2(3):256-265.
doi: 10.1007/s40746-016-0056-6. Epub 2016 Jul 8.

Diagnosis and Management of Congenital Sensorineural Hearing Loss

Michelle M Chen, John S Oghalai

Diagnosis and Management of Congenital Sensorineural Hearing Loss

Michelle M Chen et al. Curr Treat Options Pediatr. 2016 Sep.

Abstract

Hearing loss is the most common sensory disorder in the USA. The diagnosis of congenital hearing loss starts with newborn hearing screening, which is best performed with auditory brainstem evoked responses in order to avoid the risk of missing auditory neuropathy spectrum disorder. A careful history and physical exam can occasionally help reveal the etiology for congenital hearing loss. Imaging studies, either CT temporal bones or MRI of the internal auditory canals without gadolinium, and genetic testing, in particular for connexin 26, connexin 30, and Pendred syndrome, are the most useful diagnostic tests. Management of congenital hearing loss involves early fitting of amplification. Early cochlear implantation, preferably before 2 years of age, should be strongly considered for children with bilateral severe hearing loss.

Keywords: Cochlear implantation; Congenital hearing loss; Deafness; Diagnosis; Hearing loss; Sensorineural hearing loss.

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Conflict of interest statement

Compliance with Ethical Standards: Conflict of Interest: John S. Oghalai reports grants from the NIH (R01 DC010075), during the conduct of the study. Michelle M. Chen declares that she has no conflict of interest.

Figures

Fig. 1
Fig. 1
Diagnostic algorithm for congenital hearing loss.
See this image and copyright information in PMC

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