Smithtr

In the latest ASHA guidelines for CI's to treat Auditory Neuropathy Spectrum Disorder (ANSD), something jumped off the page. From the article:

"There are other conditions involving neuropathy of peripheral nerves that have been associated with hearing loss and ANSD. These include Leber's optic neuropathy, Stevens-Johnson syndrome, Ehlers-Danlos syndrome…"

The Ehlers-Danlos Syndrome is the one that made the hair on the back of my neck stand up: Several weeks ago I screened a young lady for ANSD by measuring her acoustic (stapedial) reflexes. Several years earlier, she had been diagnosed by a school SLP as having (central) auditory processing disorder ((C)APD); but since no audiologic testing was performed, I screened her for ANSD… And she passed with reflex thresholds within normal limits. However, something was telling me that she needed an ABR, as her complaint of music perception didn't quite make sense.

This delightful young lady has Ehlers-Danlos Syndrome -- And she MAY -- and I underscore _MAY_ -- be the first person detected who has ANSD with normal acoustic reflex thresholds. Time to arrange an ABR for her. Fortunately, she is near Johns-Hopkins, which has a world class CI program.



Cochlear Implants in Auditory Neuropathy Spectrum Disorder

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