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Purpose: Auditory neuropathy spectrum disorder, or ANSD, can be a confusing diagnosis to physicians, clinicians, those diagnosed, and parents of children diagnosed with the condition. The purpose of this review is to provide the reader with an understanding of the disorder, the limitations in current tools to determine site(s) of lesion, and management techniques.
Method: This article is a review of what is known about ANSD. It includes descriptions of assessment tools, causes of ANSD, and patient management techniques.
Conclusions: This review is a guide to audiologists, speech-language pathologists, and early interventionists who work with individuals diagnosed with ANSD and/or their families. It highlights the need for more precise tools to describe the disorder in order to facilitate decisions about interventions and lead to better predictions of outcome.
Key Words: electrophysiology, diagnostics, otoacoustic emissions, aural rehabilitation
Brody failed his newborn auditory brainstem re- sponse screening. His follow-up diagnostic evalua- tion, conducted at 1 month of age, revealed present evoked otoacoustic emissions (OAEs) for both ears. Although a cochlear microphonic (CM) was recorded during the audi- tory brainstem response (ABR) test, no ABR was detected foreitherear.
Mr. and Mrs. Johnson, Brody's parents, are relaxed during the evaluation and are not suspecting hearing loss. They were told that babies often fail their newborn hearing screening due to debris in the ear canal or fluid remain- ing in the ears at the time of the screening. Mrs. Johnson had a normal pregnancy and Brody's birth was unevent- ful. In addition, there is no family history of hearing loss. The audiologist who has just completed Brody's evaluation knows that the pattern of results (i.e., OAEs and CM pres- ent with an absent ABR) is consistent with what has been called "auditory neuropathy spectrum disorder" (ANSD). But she wonders, "What do I tell Brody's parents about ANSD? What do I tell them about possible functional out- comes? Will this child be a hearing aid or cochlear implant candidate? The parents will have questions and I am not sure I can give them definitive answers."
ANSD, because of a lack of tests to accurately diag- nose the site of dysfunction and the vast array of functional outcomes, can be a confusing diagnosis. The purpose of this article was to provide...