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? Kindly assist us in ruling out the issue. And what should be done to overcome this?
Hello,
Welcome to icliniq.com.
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 stay, and neonatal sepsis. All these are strong reasons for hearing impairment. We encounter children with such neonatal history 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 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:
- Does he wake up in case there is a loud noise?
- Does he get startled by loud sounds or sudden sounds?
- Does he respond to his name or loud sounds (he needs more time to turn to his name being called)?
- If you stand away from his sight and clap loudly, does he try and see where the sound came from?
- 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 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.
Do revert back with all the details. Hoping to see your son healthy and good soon.
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?
Hello,
Welcome back to icliniq.com.
- I would suggest you to repeat the BERA 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 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 startling with 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 the damage to hearing. However, Amikacin is an excellent aminoglycoside antibiotic that has a 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 the damage.
- A hearing aid will not involve surgery but like you said it will be difficult for a baby. A cochlear implant is fixed during a 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. In 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 few more months.
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Hope you are doing well. I refer the conversation we had a month back about the issue related to my new born hearing screening.
Summarising below:
My new born (preterm birth at 29.5 weeks) was getting his hearing test failed via OAE method on the right side. Hence went for BERA for right side but that was a failure. as per audiologist advise we did a repeated BERA.
This time, we have done OAE for both the ear and BERA for the right side. However we couldn't complete the BERA for right ear and left ear was not even started because baby keeps on waking up during the test.
I have attached the updated OAE and BERA report (right - half done) herewith. As per the audiologist opinion. There is a response till 60DbNhL however there is a latency for right ear. Also they couldn't complete the test below 60 DB because he kept on waking up. But OAE is still absent in the right side.They also did a impedance audiometry which shows a congestion in middle ear for right side.
My baby is suffering from cold from birth and it is still not recovered completely.
Left ear OAE is present
Audiologist has mentioned that there seems to be hearing ability however since there is a middle ear congestion and test was not completed they cannot assure and asked for an ENT review.
1. could you please check the updated result and assist us to guide whether there is a hearing loss and whether it could be corrected?
2. Since he is having cold from birth, does it lead to any congestion in middle ear? what could be the treatment for middle ear block (if any).
3. Whether BERA test bypasses the middle ear functions and stimulates the sound directly to inner ear? i wanted to know whether the issue is because of middle ear of because of inner ear.
4. IF the fluid inside middle ear could cause this issue? in that case how long it will 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?
Thanks for the advise.
Sorry for the delayed reply.
I have gone through the updated reports.
OAE : OAE is normal on left and absent on right. We can conclude that his left side inner ear mechanisms are fine. Regarding right, middle ear pathology can cause absence of OAE.
OAE is sound produced by hair cells that should be heard in the outer ear by probe. if middle ear has fluid oae won't be normal.
regarding cold and treatment for middle ear block, decongestants would help. you need to rule out any infection in middle ear. middle ear problem can affect OAE result.
BERA evades middle ear and tests only inner ear. I think you can assume that he has normal hearing in a t least one ear. But you need to wait. If one ear is functioning we are not going to operate in the non functioning ear.
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