| Description |
Neither ABR nor OAEs are objective infallible hearing tests. There are conditions when they could be mis-leading and/or conflict with one another. One such condition is AN/AD. AN/AD is present when the ABR is absent or de-synchronized but emissions are (or once were) present. Proper diagnostic techniques and etiologies will be discussed. Etiologies range from prematurity, hypoxia and BIND (Bilirubin Induced Neural Damage) to genetic absence of Inner Hair Cells (OTOFERLIN) or neural maldevelopment (Pejvakin) and MPZ (Myelin Protein Zero), among others. Seven percent of our sample of more than 300 patients have REQUIRED NO INTERVENTION to develop usable speech and language despite their continually absent ABRs. Management should be conducted with an eye toward language development and in conjunction with Speech-Language Pathologists and/or educational audiologists and Teachers of the Deaf. We will review the value of Cued Speech, and cochlear implants as an especially powerful combination and show how managing the patient from the audiogram is not always productive. Care should also be taken to distinguish Auditory Immaturity (where the ABR and the abnormal behavior will all resolve spontaneously) from AN/AD where language might develop but the ABR will remain de-synchronized throughout life.
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