March 2021 The New Indian Express

One-year-old Nitin (name changed) suffered from congenital hearing loss due to a rare genetic disorder — Waardenburg-Shah (type 4) syndrome. But, with timely treatment through cochlear implant surgery, Nitin can hear and enjoy sounds for the first time in his life and develop speech.  He is also the first child in India with Waardenburg-Shah syndrome to undergo cochlear implant.

babyWaardenburg-Shah (type-4) syndrome is a rare genetic condition that can cause hearing loss, change in colouring (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 have been reported in India. 

Nitin would not respond to sounds and after he turned one. His parents then consulted a private hospital. “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 training sessions in listening for him. Since he was not still responding to sounds, 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 sounds for the first time in his life on January 2. He is now undergoing Auditory Verbal Therapy and is recovering. The parents have decided to go for the cochlear implant for the left ear later. For now, he is wearing a hearing aid on  the left ear,” said Dr Santosh  Shivaswamy, Senior consultant ENT, Cochlear Implant, Surgeon at Columbia Asia Hospital, Hebbal.

Dr Shivaswamy added, “Timely surgery helped Nitin overcome his hearing problem. Performing surgery before the age of 3 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.”Congenital birth defects  are a major cause for infant mortality worldwide. As per a recent World Health Organisation newsletter, 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. Nitin’s parents were happy with the outcome. Nitin’s mother said, “Our son is finally responding to us when we call him by his name. This is one of the happiest moments for us! Although we were aware of the genetic condition of our son, we were anxious whether he will be able to overcome his hearing problem. The doctors at Columbia Asia Hospital Hebbal have made it possible for him.”

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