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CS - AN/AD

Auditory Neuropathy or Auditory Neuropathy/Dys-synchrony (AN/AD) was first recognised in the 1980s and is a comparatively new and a potentially confusing diagnosis. It is also referred to as Auditory Neuropathy Spectrum Disorder (ANSD). 

Children diagnosed with AN/AD may function as totally deaf or (in about 7% of cases) may experience no problem and acquire language naturally and normally.  Other children fit into this continuum anywhere between the two extremes. 

For some AN/AD children their hearing may fluctuate from day to day or even hour to hour based on fluctuations in body temperature or background noise.  A minority outgrow the disorder.  Children tested in clinical conditions in a sound-proof booth may appear to have virtually normal hearing but when noise is added hearing for speech is described as ‘universally very poor’.

It is now estimated that 10% of the deaf population may have AN/AD.  It is widely recommended that Cued Speech is used with AN/AD babies and children for a number of reasons; one of which is that about 7% of the children with this problem ultimately need NO intervention. Therefore, one does not want to do harm with hearing aids or implants, but can ensure language development regardless of the outcome by using Cued Speech.

Auditory Neuropathy/Dys-synchrony (AN/AD)

Increasingly specialists in the USA are recommending Cued Speech for children diagnosed with AN/AD. We are very grateful to Charles Berlin Ph.D., Professor of Hearing Science and Clinical Professor of Otolaryngology Head and Neck Surgery, LSU Health Services Center, USA, both for his permission to quote him in the following information and for checking the remaining text for accuracy.

Introduction

Auditory Neuropathy or Auditory Neuropathy/Dys-synchrony (AN/AD) was first recognised in the 1980s and is a comparatively new and a potentially confusing diagnosis. Children are described as having AN/AD when results of two so-called ‘objective tests’ conflict. Thus, AN/AD is diagnosed when tests of outer hair cells (Otoacoustic emissions) suggest normal hearing but tests of the inner hair cells and nerve fibers (ABR) suggest poor hearing and/or deafness (see overleaf for more information). In other words, the child’s ears appear to be ‘hearing’ by the emissions test but brainstem tests to ascertain whether there is enough neural synchrony to allow speech to be ’heard’ within the brain, is grossly abnormal.

Children diagnosed with AN/AD may function as totally deaf or (in about 7% of cases) may experience no problem and acquire language naturally and normally. Other children fit into this continuum anywhere between the two extremes. 


For some AN/AD children their hearing may fluctuate from day to day or even hour to hour based on fluctuations in body temperature or background noise. A minority outgrow the disorder. Children tested in clinical conditions in a sound-proof booth may appear to have virtually normal hearing but when noise is added hearing for speech is described as ‘universally very poor’.

It is now estimated that 10% of the deaf population may have AN/AD. It is widely recommended that Cued Speech is used with AN/AD babies and children for a number of reasons; one of which is that about 7% of the children with this problem ultimately need NO intervention. Therefore, one does not want to do harm with hearing aids or implants, but can ensure language development regardless of the outcome by using Cued Speech.

What is AN/AD?

It was originally thought that the auditory nerve was always to blame so the condition was described as Auditory Neuropathy.

It now appears that additionally the sound being received by the child’s brain can be muddled or ‘dys-sychronised’ because of the absence or breakdown of a specialised cell in the inner ear, the Inner Hair Cell. It sounds as if the child is listening through a very poorly tuned radio. Sound may be distorted and sometimes sound like little more than static or white noise. This is one of the reasons why some professionals prefer to label the condition Auditory Neuropathy/Dys-synchrony, because often the nerve is perfectly intact.

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