1-year-old boy enjoys sound for the first time after cochlear implant at Columbia Asia Hospital Hebbal.

 

Congenital birth defects are a major cause for infant mortality worldwide. As per the recent

newsletter of WHO, an estimated 2,95,000 newborns die within 28 days of birth every year,

due to this. Identifying the birth defects in the initial stage and treating them early can help

in effective treatment and faster recovery of the child.

Recently, Columbia Asia Hospital Hebbal saw a case of 1-year-old boy Nitin (name changed)

who was suffering from congenital hearing loss due to a rare genetic disorder

Waardenburg-Shah (type 4)syndrome. Since birth, he was not able to hear or

respond to sound and could not speak as well. Successful implementation of a

cochlear implant at the age of 1 helped the boy hear and enjoy sounds for this first time in

his life and develop speech.

Nitin, had attracted a lot of attention from everyone due to his beautiful blue eyes, a

by-product of his rare genetic disorder. Immediately after the birth, Nitin was

diagnosed with Waardenburg-Shah(type4) syndrome.

Waardenburg-Shah (type4)syndrome, is a rare genetic condition less than 10 cases reported

in India, that can cause hearing loss; changes in coloring (pigmentation) of the hair, skin,

and eyes; and Hirschsprung disease, an intestinal disorder that causes severe constipation

or blockage of the intestine. Less than 10 cases of babies with this syndrome are

reported in India. Probably he is the first child to undergo cochlear implant in India

with Waardenburg-Shah syndrome.

Nitin turned 1-year-old and not responding to surrounding sounds, that’s when his

parents consulted Dr. Santosh Shivaswamy, Senior consultant ENT & Cochlear Implant

Surgeon at Columbia Asia Hospital Hebbal for a solution.

Explaining Nitin’s condition, Dr. Santosh Shivaswamy, Senior consultant ENT & Cochlear

Implant Surgeon Columbia Asia Hospital Hebbal said “For one year Nitin could not hear any

sound due to the congenital hearing loss. Since the scans and MRI revealed that his cochlear

and auditory nerve structures were normal, we decided to put superpower digital hearing

aid for both the ears. We also began listening training sessions for him. Since he was not still

responding to sound we counselled the family and they decided to go ahead with a cochlear

implant. Surgery was performed for the right ear and finally the child was able to hear

sound for the first time in his life. He is now undergoing Auditory Verbal Therapy and

recovering.”

While most babies are born healthy, genetic condition, family health history, age of the

mother can cause congenital birth defects. With advancements in health care, many birth

defects can be diagnosed before birth with good accuracy. Birth defects can either be cured

completely through various paediatric surgeries or its impact can be reduced to a large

extent.

Nitin’s parents were happy to see the outcome in their baby. Thanking the efforts of the

doctor, Nitin’s mother said, “Our son is finally responding to us when we call him by his

name for the first time. This is one of the happiest moments for us! Although we were

aware of the genetic condition of our son, we were scared whether he will be able to

overcome his hearing problem. The doctors at Columbia Asia Hospital Hebbal have made it

possible for him.”

Timely surgery helped Nitin to overcome his hearing problem." Performing surgery before

the age of 3 years always helps restore both speech and hearing" in babies with congenital

hearing loss. If treatment is delayed, the results of the surgery and speech outcome may

vary.

Currently Nitin has recovered from his surgery and undergoing the necessary training

sessions. He loves listening to music!