Auditory Neuropathy: Frequently Asked Questions

Charles I. Berlin, PhD

Q. I have a six-month-old who has just been diagnosed with auditory neuropathy at the 60decibal level. What is the difference between this and nerve deafness? Why is it so controversial? What are the chances of it getting better? -worse? What percentage of patients do hearing aides work for? If it gets worse, are cochlear implants an option? I greatly appreciate any and all information that can be provided!!

A. This isn't controversial as much as it is unfamiliar to many physicians and audiologists. If you e-mail me your snail mail address I will send you some reprints and suggestions for management. cberli@lsumc.edu You can also look at the LIST SERVE below which is made up of parents whose children have been so diagnosed.www.onelist.com/AuditoryNeuropathy...Read their archives...or you can get there from our web page below. We also have a link to a demonstration of what your child might hear like. That will be very useful to explain to your audiologists why hearing aids would not really be useful. You will find link to Fang Gang Zeng's web page at the University of Maryland (Click Here )as you work through our web page at LSU. We recommend Cued Speech and/or American Sign Language first, and then, if you want your child to be a verbal user of the phone etc., consider a cochlear implant. But NOT before he/she learns some language because He/she won't be able to talk if they don't have much to say.

The condition is better named AUDITORY DYS-SYNCHRONY because it is a disorder of the timing of the auditory nerve. There are many possible causes but in newborns the most common is a mild hyperbilirubinemia (12-16 cc/dl). It manifests itself when the ABR looks absent or abnormal and the otoacoustic emissions (sounds which come from the ear) are normal if not robust.

Some children have a genetic condition of either no inner hair cells or a predisposition to jaundice effects. Some chidlren actually get better...or at least they begin to hear more although their ABRs may never improve. Don't fret over that since the ABR is NOT a test of hearing, only a test of neural synchrony (precision of timing) and many children can have poor synchrony without being deaf. Does your child actually behave deaf? If so, start to learn Cued Speech (Language) or signs so that you and your household can all use them and the child can eavesdrop on you visually.

Cochlear implants DO help these children but it is well to watch them for a while to see if they are making spontaneous recovery. During that time you should not be waiting for a miracle, but should be pouring language into them visually.