Friday, 12 August 2016

Island of the Deaf...

(New England Historical Society)
On Martha's Vineyard.  The tale of the island deaf is compelling not only because it so beautifully illustrates a genetic founder effect, but it is also an unusual blurring of disabled versus abled. 

The story has been told well in many places, including Arthur R. Railton’s excellent “The History of Martha’s Vineyard“, Nora Groce’s “Everyone Here Spoke Sign Language: Hereditary Deafness on Martha’s Vineyard“, and a 3-part series by audiologist Bob Traynor.

The deafness on the Vineyard traces its roots to Weald, in the County of Kent in England. The pilgrims brought the recessive allele here an unknown number of times.

The first deaf person known to live on Martha’s Vineyard was Jonathan Lambert, born in 1657 into a prominent family on Cape Cod. He had one deaf sibling and five siblings who could hear. Jonathan married Elizabeth Eddy, who was from the island, and they moved there in 1692. Two years later he bought what is today Lambert’s Cove from a Wampanoag. A diary entry from April 5, 1714, from a Judge Sewall wrote of Jonathan Lambert that he “spake not a word to us, and it seems he is deaf and dumb.” Other accounts call him a “deaf mute.”

Jonathan and Elizabeth had 7 children. The two born deaf, the first cases known to have been born on the island, didn’t have children, but the condition appeared again because the others were obligate carriers – each had to have a recessive allele because their father had 2 copies of the mutation.

The numbers of deaf grew as the few and large founding families intermarried, and a unique local sign language evolved. At one point the town of Chilmark actually required residents to know and use it. The deaf people held the same types of jobs as everyone else, even serving in public office, and town meetings and other get-togethers used sign language.

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