How a Single Genetic Mutation Led to the Quietest Village on Earth
As I entered Dadkhai—a remote, picturesque village on top of a hillock surrounded by the mighty Himalayas—the first thing I noticed was the silence. There are no children playing games and screaming loudly, no adults gossiping and laughing and haggling, and none of the sort of bustling noises you’d typically hear in any other village in the Indian-administered side of Kashmir.
That’s because Dadkhai—inhabited by about 300 families, according to locals—is known as the “silent village.” It has a higher proportion of deaf and mute people within its population than any other community on Earth. A 2014 investigation by the government-funded Indian Council of Medical Research (ICMR) found that Dadkhai has the world’s highest prevalence of non-syndromic deaf mutism (meaning the loss of hearing and speech is not associated with conditions affecting other parts of the body). Villagers say many of the children born here are unable to speak or hear.
Naturally, the village operates quite differently from the rest of the world. It’s common to see people staring at each other without saying a word. Villagers are identified not by names but by marks on their bodies. Even those able to hear and speak normally have adapted to the quiet nature of communication.
“Every family is hit by this curse,” Choudhary Mohammad Hanief, the village chief, told me when I visited Dadkhai in February. “At least 83 people are still deaf and mute. I requested the government for a school for these children but our pleas fell on deaf ears. These people have been kept away from basic education.”
The remote village is impoverished by most metrics. Many houses have verdant roofing made of birch bark and earth, and families supplement their incomes by growing vegetables. There are no paved roads. Houses are connected through crisscrossing dirt trails. A small health center exists, but has no doctor. Drinking water is drawn from nearby springs. The villagers largely depend on agriculture, livestock, and daily wage labour for their livelihoods.
Yet the community remains peaceful. Crime is very rarely reported, according to police records.
However, the high prevalence of deaf-mutism has brought Dadkhai international notoriety. Villagers are now suspicious of visitors, often wielding cameras and taking notes, and calling themselves doctors. They don’t want to cooperate anymore, saying they have given enough blood for samples.
That includes Hanief, who owns a prominent three-story house in the village and is better off than most of the inhabitants. Hanief has taken it upon himself to advocate for the needs of the village’s disabled. In the past, he has invited doctors from all across Jammu and Kashmir and parts elsewhere to find some kind of cure for village children born deaf and mute here.
But now, he said, he has given up on doctors.
“Over the years teams of doctors visited the village, taking blood samples of our deaf and mute children,” said Hanief. “And then we didn’t hear anything from them.”
When I visited his home, his children (Shabir Ahmad, 34; Aftab Ahmad, 18) were outside building a wall around a vegetable garden. Hanief told me they are master masons but they cannot speak or hear. “They are deaf and mute,” he said. “You have to know their language and as a parent, you die every day thinking about what will happen to them after our death.”
Both Shabir and Aftab looked toward their father and began to speak to each other in sign language. “They are asking why these doctors are here. They are saying they will not give blood samples again,” said Hanief.
“Doctors came here. News reporters came here and shot our videos but we didn’t find any solution on how to stop the spread of this disease to our future generation,” he said. At the very least, the villagers hoped to get a special school dedicated to educating the deaf and mute children who grew up here. But no such school was built, and the village has been once again forgotten.
For decades, the cause behind deaf-mutism in Dadkhai was a mystery. But scientists strongly suspected there was a genetic link. According to Sunil Kumar Raina, an Indian neurologist who led the 2014 ICMR investigation, the village is mostly comprised of Gujjars, an ethnic nomadic community spread across India, Pakistan, and Afghanistan. His investigation’s genetic tests on the village’s population revealed the wide prevalence of a mutation that causes a deficiency in a protein called otoferlin, which can lead to hearing loss. That mutation is hereditary.
And so experts believe that consanguineous marriages within the village over decades mean the hereditary mutation has quickly spread down from generation to generation. Government data reveals that at least 83 people, mostly females, are affected by the defective gene, which has torn through 55 families in the village.
To change the village’s future, authorities are now encouraging residents of this historically remote village to break their isolation and search for partners who hail from other communities. Sushil Razdhan, is a well-known neurologist from the Indian provinces of Jammu and Kashmir who leads a research team studying the prevalence of deaf mutism in Dadkhahi. “Initially [in 2014] we saw 120 patients, but the number has decreased now because many families have stopped intra-community marriages,” he said.
Locals say the first case of deaf and mute was reported in 1901 when a person named Meran Baksh was born to a family who migrated to the village from a nearby Jammu district. He lived a long life, dying some time in the early 1990s.
But it turns out the deaf-mutism originates from at least one other source as well. According to the ICMR genetic mapping investigation, there is more than one lineage responsible. As Dadkhai’s reputation as a “silent village” spread, doctors theorize that others with hearing and speech loss who carried the otoferlin gene mutation may have moved into the village.
As I spent more time in Dadkhai, I spoke to another villager, Gulam Nabi, who has two deaf and mute children. “We can’t go outside the village because if our kids need anything, how would they ask?” he told me.
The lack of assistance from authorities means residents in Dadkhai are on their own to figure things out. That’s despite pleas from villagers for some kind of help.
“The government should open special schools for deaf and mute children so they could get a basic education,” Nabi added. “They would be able to use and understand sign language to communicate. They should open some working centers where they can work, learn and earn their livelihood.”
Razdhan agreed. While there are ways to help reduce the rate of deafness and muteness in the village, there still needs to be some help for those who are living with these disabilities in Dadkhai.
“The deaf-mutism is affecting their social life and the government needs to rehabilitate them,” Razdhan said.
Source: The Daily Beast
