My premature baby has failed his hearing test. What to do next?

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 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:

1. Does he wake up in case there is a loud noise?
2. Does he get startled by loud sounds or sudden sounds?
3. Does he respond to his name or loud sounds (he needs more time to turn to his name being called)?
4. 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,

Welcome back to icliniq.com.

I would suggest you 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 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.

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 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 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.