Patient's Query
Hello doctor,
I am writing regarding the newborn screening of my baby. He was prematurely born at 29 weeks and spent 40 days in NICU. During his stay, we tested his hearing via the OAE method. At first, both the ears did not respond, and in the second screening, the left ear was perfect. But, there was no response from the right ear. After three consecutive failures of OAE in the right side, we went for BERA. However, the BERA test showed no response in the right ear even at 90 dB. Hence, the audiologist recommended a review after one month. Now, I am a bit worried. What could be the cause for BERA failure? And what should be done to overcome this? Please help.
Thank you.
Hello,
Welcome to icliniq.com.
I read your query and understand your concern.
I have reviewed the files (attachment removed to protect patient identity).
The BERA (brainstem evoked response audiometry) report shows a profound hearing loss in the right ear. OAE (otoacoustic emissions) shows an absence of OAE in the right which means the inner ear hair cells have got damaged. Your baby has had a tough course till now. He has suffered hyperbilirubinemia, ICU (intensive care unit) stay, and neonatal sepsis. All these are strong reasons for hearing impairment. We encounter children with such neonatal histories who develop hearing loss. If the hearing loss is unilateral and the other side, that is, the left ear, is fine, then we can wait and proceed. We may use hearing aids or cochlear implants in the defective ear in another year's time so that the baby can have normal growth and development. However, if the other ear is not fine, then we need to give the baby a cochlear implant or bilateral implant as soon as possible. After four to five years of life, the neural system loses its plasticity, and thereafter an implant will not function as well as one that is put in now. As the child grows, the nervous system involved in hearing gradually loses its plasticity. When the baby cannot hear, he does not develop speech. The sooner we rehabilitate him, the better his development will be. To identify if the baby hears, check the following:
In the reports you have attached, where is the report for the left ear? The BERA report for the left is not seen. Also, I would like to see OAE reports as well. When the baby is better, you will have to take him to an otolaryngologist (ENT (ear, nose and throat) specialist) or a neuro-otologist (neuro-specialized ENT). We must check his outer and middle ears for any pathology. Also, I hope the baby does not have any other abnormalities. We must rule out a syndromic association.
I hope this helps.
Please revert so I can assist you further.
Thank you.
Patient's Query
Hello doctor,
Thanks for the response.
It is extremely sad, and we are unable to digest this fact. My baby is otherwise healthy and does not seem to have any issues. Could you please advise if any medicines used in the NICU might have caused this? When his left ear is fine, why does his right ear have such a problem? The left ear BERA was not done because he woke up during the test, and they could not complete it. However, OAE was successful. (but the report is not yet available). Will this be cured by any surgery or treatment without any implant or hearing aid? It is worrying to put such aids for such a small baby, and it will be a challenge to maintain it on the go. Since his left ear seems fine, can we maintain it without such aids? Will this affect the quality of his life?
Thank you.
Hello,
Welcome back to icliniq.com.
I would suggest you repeat the BERA (brainstem evoked response audiometry) for both ears once again. At least the left one. If for sure we know that the left ear is functioning, then we can hold the implant or aid and wait and watch. If in the course of a child's development, you feel that he does not hear, then we can repeat the BERA and manage accordingly. Please observe his milestones ardently and carefully for about a year. Regular visits to his pediatrician and discussing the milestones will be helpful. Please observe the baby regarding his waking up to sounds, his responding to calling his name, his being startled by loud sounds, etc. These will give you a good idea about the hearing of the child.
Amikacin injection given during his hospital stay post-birth could have resulted in damage to his hearing. However, Amikacin is an excellent aminoglycoside antibiotic that has good action against gram-negative organisms in sepsis. Amikacin given for short durations will not cause hearing damage. In some individuals, Amikacin does not cause damage. A hearing aid will not involve surgery, but as you said, it will be difficult for a baby. A cochlear implant is fixed during surgery and switched on after a few weeks. A cochlear implant also has an external component that will be visible to people. Hearing aids and cochlear implants have different indications. Before we do the cochlear implant, we run a battery of tests to check the hearing. BERA is just one of them. By all means, I feel you should get BERA done for the left ear and report to us. The management will depend on that. Any surgery we attempt will not be now; we shall wait a few more months.
I hope this helps.
Please revert so I can assist you further.
Thank you.
Patient's Query
Hi doctor,
Thank you for your reply.
Hope you are doing well. I refer to the conversation we had a month back about the issue related to my newborn hearing screening.
Summarising below:
My newborn (preterm birth at 29.5 weeks) was getting his hearing test failed via the OAE method on the right side. Hence went for BERA for the right side, but that was a failure. as per the audiologist's advice, we did a repeated BERA.
This time, we have done OAE for both the ear and BERA for the right side. However, we could not complete the BERA for the right ear, and the left ear was not even started because the baby kept on waking up during the test.
I have attached the updated OAE and BERA report (right - half done) herewith. As per the audiologist's opinion. There is a response till 60DbNhL; however, there is latency for the right ear. Also, they could not complete the test below 60 DB because he kept waking up. But OAE is still absent in the right side. They also did impedance audiometry which shows congestion in the middle ear for the right side.
My baby has been suffering from cold from birth, and it is still not recovered completely.
Left ear OAE is present. The audiologist has mentioned that there seems to be hearing ability; however, since there is middle ear congestion and the test was not completed, they cannot assure and asked for an ENT review.
1. Could you please check the updated result and assist us in guiding whether there is a hearing loss and whether it could be corrected?
2. Since he has had a cold from birth, does it lead to any congestion in the middle ear? What could be the treatment for middle ear block (if any)?
3. Whether the BERA test bypasses the middle ear functions and stimulates the sound directly to the inner ear? I wanted to know whether the issue was because of the middle ear or because of the inner ear.
4. If the fluid inside the middle ear could cause this issue? In that case, how long will it take to drain it out? He is now 3.5 months from birth.
5. When there is a loud sound, he gets startled, but that is not consistent. In that case, can we assume he has normal hearing?
Thank you for the advise.
Hello,
Welcome back to icliniq.com.
I have gone through the updated reports (attachments have been removed to protect the patient's privacy).
OAE: OAE (otoacoustic emissions) is normal on the left and absent on the right. We can conclude that his left side inner ear mechanisms are fine. Regarding the right, middle ear pathology can cause the absence of OAE. OAE is sound produced by hair cells that should be heard in the outer ear by a probe. If the middle ear has fluid, OAE would not be normal.
Regarding cold and treatment for middle ear block, decongestants would help. You need to rule out any infection in the middle ear. Middle ear problems can affect OAE results.
BERA (brainstem evoked response audiometry) evades the middle ear and tests only the inner ear. I think you can assume that he has normal hearing in at least one ear. But you need to wait. If one ear is functioning, we are not going to operate in the non-functioning ear.
I hope this helps.
Please revert so I can assist you further.
Thank you.
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Answered byDr. Shyam Kalyan N
Medically reviewed byiCliniq medical review team
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