Necessity of Revising the Evaluation Protocols of Diseases and Syndromes with Hearing Loss

Electrophysiology tests, nowadays, are one of the important tests in evaluating the function of sensory modalities. Auditory evoked potentials (AEPs) familyare one of the biggest family of evoked potentials (Eps) include more than 10 valuable members as follows: ECochG, ABR, sABR, ASSR, AMLR, P50 (Sensory gating), ALR, MMN, P300, N400, and P600, etc. Unfortunately, these tests do not have their real positions in evaluating the auditory systems of people with diseases and syndromes with hearing problems. During a research in 2015, author of this paper found a patient with Noonan syndrome (NS) with a problem in his middle ear but with delay in speech despite this fact that he was 5 yr old (according to his profile). There was an inconsistency between the results of audiological tests and this fact that the patient had a delay in speech. During 55 yr after discovering NS, there was not any publication as for the using of auditory electrophysiological tests for analyzing the central auditory nervous system in NS. Recall that there are also many diseases and syndromic disorders like NS that hearing loss is one of the signs of those impairments and still many of the aspects in relation with their malformations/malfunctions of CANS have not yet iscovered.Attention to some aspects of syndromes should not lead to a lack of attention to other aspects. Certainly, there is the right of these patients to be able to use the abilities of perceptive and expressive language, but before that, we need to fully understand the functioning of the CANS of these patients and their defects. Delay in speech can be resultant of a delay in the maturation of the nervous system as well. Therefore, the evaluation protocol for the patients with these diseases and syndromes should be revised. Performing auditory electrophysiological tests are required for these patients that they can benefit from all-inclusive services.In this oral presentation, ability of AEPs was described in showing the disorders of auditory system, developmental delay, monitoring the changes in auditory system after auditory rehabilitation and so on

کلیدواژه ها:

Syndrome ، AEPs ، CANS ، Hearing Impairment