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The term auditory neuropathy is more recently referred to as auditory dys-synchrony. Auditory dys-synchrony is a more accurate term because "neuropathy" implies a problem with the eighth nerve Lukasz Piszczek Dortmund Trikot , when in fact the nerve may not be affected. It is a dysfunction in the auditory system in which a person has normal outer hair cell function, but heshe has a dys-synchronous connection between the inner hair cells and the eighth nerve. In general, this means that patients display normal OAEs (otoacoustic emissions) and a measurable cochlear microphonic, but they have absent ABRs (auditory brainstem response) and acoustic reflexes (Hood, 2002). The responses could be caused by a problem with the inner hair cells, select outer hair cells, the synapse between the hair cells and the auditory nerve Shinji Kagawa Dortmund Trikot , the neural axon or dendrite, the myelin sheath, the spiral ganglion cells, or a combination of all of these (Mason, De Michele, Stevens, Ruth Christian Pulisic Dortmund Trikot , & Hashisaki, 2003).

Clinical Test Results

According to Dr. Chuck Berlin, patients with auditory neuropathy have absent acoustic reflexes, present OAEs, present cochlear microphonic, an absent ABR, and normal radiographic results. A full test battery is required to accurately detect and identify auditory dys-synchrony. These tests include tympanometry Maximilian Philipp Dortmund Trikot , acoustic reflexes, OAEs, cochlear microphonic, and ABR.

After confirming normal middle ear status with tympanometry, then the otoacoustic emissions and cochlear microphonics are used to assess outer hair cell function (which should be normally functioning in these patients), and acoustic reflexes and ABR are used to assess the synchrony of the eighth nerve (which would be abnormal in these patients). Radiologic testing can also be done to rule out eighth nerve lesions or multiple sclerosis. Persons with true auditory dys-synchrony would not show any abnormalities on radiographic testing (Hood, 2002).

Diagnosis

According to Dr. Chuck Berlin Mahmoud Dahoud Dortmund Trikot , a diagnosis cannot be based on a patient's audiogram or speech scores alone. Pure-tone thresholds and speech testing can vary greatly among these patients. Patients will often complain that they have difficulty hearing or understanding speech, especially amidst background noise. However, this is common among people with typical cochlear loss, and therefore a whole range of testing is needed before an accurate diagnosis can be made. The first clinical sign (after confirming normal middle ear status) would be present OAEs and absent reflexes. These results indicate a need to conduct further testing (ABR and ECochG).

The ABR portion of the complete test battery will provide the clinician with defining information. When doing an ABR, the cochlear microphonic will invert when the clinician changes the polarity. With auditory dys-synchrony, the whole waveform will completely invert with a change in polarity. This is because there is no neural response, and there is only a cochlear microphonic. The cochlear microphonic will be extended in time and may even resemble waves Sebastian Rode Dortmund Trikot , but the way to detect this is by comparing condensation runs to rarefaction runs. If the entire response inverts, then the clinician can be sure it's the cochlear microphonic and not neural activity. This finding, in correlation with all other test results, will confirm the diagnosis that someone has auditory dys-synchrony (Hood, 2002).

Risk Factors and Treatment

Infants at risk are those with hyperbilirubinemia, premature birth weight, perinatal asphyxia Sergio Gomez Dortmund Trikot , and transfusions. Children at risk are those who fail to develop speech and language or who are thought to have an auditory processing disorder. However, auditory dys-synchrony does occur in children with no risk factors. This can run in families and gene identification is currently being pursued (Hood, 2002).

Patients with may have other peripheral neuropathies. Some people demonstrate hereditary sensory-motor neuropathy, while others have less apparent or no symptoms of neuropathy. Auditory dys-synchrony is generally bilateral and most patients complain that they have difficulty hearing or understanding speech, which is especially problematic in noise. Hearing aids provide help with the detection of sound, but they offer minimal help to these patients in terms of discrimination (Hood, 2002).

Cochlear implantation has been successful with these patients Manuel Akanji Dortmund Trikot , which further confirms the need to use the word "dys-synchrony" rather than "neuropathy". "Neuropathy" implies a problem with the eighth nerve, when in fact the nerve may not be affected. If the problem did lie with the eighth nerve, then this would imply that cochlear implants are not an option. However, cochlear implants have been proven to be very beneficial to patients with auditory dys-synchrony (Hood, 2002). Retha Demaine
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