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Reasons for Congenital hearing loss - Online Doctor Chats

Date : 21-Feb-2012
User rating for this question
Very Good Posted in: Ear, Nose and Throat Problems
Answered by

General & Family Physician
Practicing since : 2004
Answered : 40 Questions
User :   Hi
Doctor :   Hi,
User :   I have a daughter who is turning 4 in July
User :   she has been diagnoised of hearling loss of about 80% in the past year
User :   the doctors are saying that it is a progressive loss
User :   we are sure that the loss was not there from the birth as she was checked for the same at the time of her birth
Doctor :   ok,
User :   although the doctors have conducted multiple BERA tests on her, no one is telling us the reason as to why the loss has happened
User :   although she has now been fitted with hearing aids, we would like to know if there is a way in which we can know what is the reason of her loss
User :   so that if possible it can be cured
User :   is there a way we can find it out ?
Doctor :   There are two major components of the hearing sense
Doctor :   one is sensory and the other conducting system
Doctor :   the sensory component of the hearing apparatus is made or the analyzing and interpreting brain , nerves and down to the receptors in the cochlea ( the hearing apparatus )
Doctor :   any damage in this cannot be cured
User :   ok .. but how do we know if the damage is in the sensory part or the conducting system ?
Doctor :   and the other part constituted by the Ear pinna , auditory canal , the ear ossicles ( small bones )
Doctor :   there are tests with tuning forks and also audiometry test can determine this
User :   we have the reports from these tests as well as BERA, some doctors also suggested that we get an MRI done, would you suggest that ?
User :   also how can we share these reports with you and other doctors on the panel here
Doctor :   yes, MRI would give a detailed insight into the brain nerves and their development
Doctor :   You can mail them
User :   so you would suggest that we go for an MRI as well
Doctor :   yes,
User :   one of the doctor was saying that it might be due to the forcep delivery ... can that be a cause of progressive hearing loss as well ?
Doctor :   if the choclea ( sensory part ) not developed and the nerve ( 8th nerve ) is good , then there is an option for Choclear implants
Doctor :   yes, the forceps delivery can do rarely affect
User :   ok ... if she is picking up words easily with hearing aids does that suggest something ? i mean does that suggest that the inner year is probably ok ?
Doctor :   There can be damage either to the ear itself or to the brain centers that process the aural information conveyed by the ears.
User :   i guess cause she is picking up words we can rule out issue with the brain centers can we ?
Doctor :   yes, we do can consider that they are working
Doctor :   yes, you are right
Doctor :   as the analysis of the sounds is done by the brain
User :   so if that is the case, that the brain is able to analyse the sound and hearing aids are able to make her hear properly can the condition be cured without the choclear implants ?
User :   or as the doctors in bangalore is suggesting that sooner or later we have to get the implants done
Doctor :   yes, there is a chance . BERA can give some clue into that possibility
User :   we noticed one strange thing, even the doctors conducting the tests (BERA) noticed it, before the BERA, when they wore conducting the audiometery, when they played some drum noices etc , my daughter was able to respond to them without the hearing aids on
User :   but in the BERA they said that the loss is 80% and that she could have not have responded like the way she did without any aids
Doctor :   that's a good input
User :   can it be that the during the sleep the hearing is more effected then when she is wide awake
Doctor :   Sleep inactivates the brain completely
Doctor :   so it cannot be differentiated
User :   but in BERA child is made to sleep right
Doctor :   yes, there the brain stem evoked responses are recorded , which acts at the subconscious level
User :   so can it be that the child reacts completely differently to sounds when she is asleep and when she is awake ?
Doctor :   the cortex ( the inactive part during sleep ) which makes the baby to realize the sounds and their meaning
Doctor :   when asleep the brain is inactive , and in BERA the subconscious parts of the brain are checked for the impulse generation
User :   so that means the response when asleep and active can be different i.e. BERA cannot be take as a conclusive proof for the hearing loss
Doctor :   once when the brain is active ( awake ) the impulses are generated there causing a sense in the child
Doctor :   the baby's responses are cut by making her sleep , but at the subconscious level the brain functioning is evaluated in BERA
Doctor :   the sleep is induced not to pick any other impulses created by other sensory inputs to the brain example like vision when awake
Doctor :   Are you able to read me ?
User :   in that case how is the response of the child so different when asleep and when awake ?
Doctor :   since the BERA says she has loss of 80%
Doctor :   then the problem could be anywhere from the conducting system to the brain stem .
Doctor :   and the well being of the conducting system is determined by the tuning fork tests or pure tone audiometry
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