Vintage Sacks (6 page)

28 July.
Miss R. sought me out this morning—the first time she had done so in almost two weeks. Her face has lost its jubilant look, and she looks anxious and shadowed and slightly bewildered: “Things can't last,” she said. “Something awful is coming. God knows what it is, but it's bad as they come.” I tried to find out more, but Miss R. shook her head: “It's just a feeling, I can't tell you more. . . .”

1 August.
A few hours after stating her prediction, Miss R. ran straight into a barrage of difficulties. Suddenly she was ticcing, jammed, and blocked; the beautiful smooth flow which had borne her along seemed to break up, and dam, and crash back on itself. Her walking and talking are gravely affected. She is impelled to rush forward for five or six steps, and then suddenly freezes or jams without warning; she continually gets more excited and frustrated, and with increasing excitement the jamming grows worse. If she can moderate her excitement or her impulsion to run, she can still walk the corridor without freezing or jamming. Analogous problems are affecting her speech: she can only speak softly, if she is to speak at all, for with increased vocal impetus she stutters and stops. I have the feeling that Miss R.'s “motor space” is becoming confined, so that she rebounds internally if she moves with too much speed or force. Reducing her L-DOPA to 3 gm. a day reduced the dangerous hurry and block, but led to an intensely severe oculogyric crisis—the worst Miss R. has had since starting L-DOPA. Moreover, her “wiping” tic—which reappeared on the 28th—has grown more severe and more
complex
with each passing hour. From a harmless feather-light brush of the chin, the movement has become a deep circular gouging, her right index finger scratching incessantly in tight little circles, abrading the skin and making it bleed. Miss R. has been quite unable to stop this compulsion
directly,
but she can override it by thrusting her tic-hand deep in her pocket and clutching its lining with all of her force. The moment she forgets to do this, the hand flies up and scratches her face.

AUGUST 1969

During the first week of August,
¹³
Miss R. continued to have oculogyric crises every day of extreme severity, during which she would be intensely rigid and opisthotonic, anguished, whimpering, and bathed in sweat. Her tics of the right hand became almost too fast for the eye to follow, their rate having increased to almost three hundred per minute (an estimate confirmed by a slow-motion film). On August 6, Miss R. showed very obvious palilalia, repeating entire sentences and strings of words again and again: “I'm going round like a record,” she said, “which gets stuck in the groove. . . .” During the second week of August, her tics became more complex, and were conflated with defensive maneuvers, counter-tics, and elaborate rituals. Thus Miss R. would clutch someone's hand, release her grip, touch something nearby, put her hand in her pocket, withdraw it, slap the pocket
three
times, put it back in the pocket, wipe her chin
five
times, clutch someone's hand . . . and move again and again through this stereotyped sequence.

The evening of August 15 provided the only pleasant interlude in a month otherwise full of disability and suffering. On this evening, quite unexpectedly, Miss R. emerged from her crises and blocking and ticcing, and had a brief return of joyous salacity, accompanied with free-flowing singing and movement. For an hour this evening, she improvised a variety of coprolalic limericks to the tune of “The Sheikh of Araby,” accompanying herself on the piano with her uncontractured right hand.

Later this week, her motor and vocal block became absolute. She would suddenly call out to Miss Kohl: “Margie, I . . . Margie, I want . . . Margie! . . . ,” completely unable to proceed beyond the first word or two of what she so desperately wanted to say. When she tried to write, similarly, her hand (and thoughts) suddenly stopped after a couple of words. If one asked her to try and say what she wanted, softly and slowly, her face would go blank, and her eyes would shift in a tantalized manner, indicating, perhaps, her frantic inner search for the dislimning thought. Walking became impossible at this time, for Miss R. would find her feet completely stuck to the ground, but the impulse to move would throw her flat on her face. During the last ten days of August, Miss R. seemed to be totally blocked in all spheres of activity; everything about her showed an extremity of tension, which was entirely prevented from finding any outlet. Her face at this time was continually clenched in a horrified, tortured, and anguished expression. Her prediction of a month earlier was completely fulfilled: something awful
had
come, and it was as bad as they came.

1969–72

Miss R.'s reactions to L-DOPA since the summer of 1969 have been almost nonexistent compared with her dramatic initial reaction. She has been placed on L-DOPA five further times, each with an increase of dose by degrees to about 3.0 gm. per day. Each time the L-DOPA has procured
some
reduction in her rigidity, oculogyria, and general entrancement, but less and less on each succeeding occasion. It has
never
called forth anything resembling the amazing mobility and mood change of July 1969, and in particular has never recalled the extraordinary sense of 1926-ness which she had at that time. When Miss R. has been on L-DOPA for several weeks its advantages invariably become overweighed by its disadvantages, and she returns to a state of intense “block,” crises, and tic-like impulsions. The form of her tics has varied a good deal on different occasions: in one of her periods on L-DOPA her crises were always accompanied by a palilalic verbigeration of the word “Honeybunch!” which she would repeat twenty or thirty times a minute for the entire day.

However deep and strange her pathological state, Miss R. can invariably be “awakened” for a few seconds or minutes by external stimuli, although she is obviously quite unable to generate any such stimuli or calls-to-action for herself. If Miss A.—a fellow patient with dipsomania—drinks more than twenty times an hour at the water fountain, Miss R. cries, “Get away from that fountain, Margaret, or I'll clobber you!” or “Stop sucking that spout, Margaret, we all know what you really want to suck!” Whenever she hears my name being paged she yells out, “Dr. Sacks! Dr. Sacks!! They're after you again!” and continues to yell this until I have answered the page.

Miss R. is at her best when she is visited—as she frequently is—by any of her devoted family who fly in from all over the country to see her. At such time she is all agog with excitement, her blank masked face cracks into a smile, and she shows a great hunger for family gossip, though no interest at all in political events or other current “news”; at such times she is able to say a certain amount quite intelligibly, and in particular shows her fondness for jokes and mildly salacious indiscretions. Seeing Miss R. at this time one realizes what a “normal” and charming and alive personality is imprisoned or suspended by her ridiculous disease.

On a number of occasions I have asked Miss R. about the strange “nostalgia” which she showed in July 1969, and how she experiences the world generally. She usually becomes distressed and “blocked” when I ask such questions, but on a few occasions she has given me enough information for me to perceive the almost incredible truth about her. She indicates that in her “nostalgic” state she
knew
perfectly well that it was 1969 and that she was sixty-four years old, but that she
felt
that it was 1926 and she was twenty-one; she adds that she can't really imagine what it's like being older than twenty-one, because she has never really experienced it. For most of the time, however, there is “nothing, absolutely nothing, no thoughts at all” in her head, as if she is forced to block off an intolerable and insoluble anachronism—the almost half-century gap between her age as felt and experienced (her
ontological
age) and her actual or official age. It seems, in retrospect, as if the L-DOPA must have “de-blocked” her for a few days, and revealed to her a time-gap beyond comprehension or bearing, and that she has subsequently been forced to “re-block” herself and the possibility of any similar reaction to L-DOPA ever happening again. She continues to look much younger than her years; indeed, in a fundamental sense, she
is
much younger than her age. But she is a Sleeping Beauty whose “awakening” was unbearable to her, and who will never be awoken again.

A DEAF WORLD

We are remarkably ignorant about deafness, which Dr. Johnson called “one of the most desperate of human calamities”—much more ignorant than an educated man would have been in 1886, or 1786. Ignorant and indifferent. During the last few months I have raised the subject with countless people and nearly always met with responses like: “Deafness? Don't know any deaf people. Never thought much about it. There's nothing
interesting
about deafness, is there?” This would have been my own response a few months ago.

Things changed for me when I was sent a fat book by Harlan Lane called
When the Mind Hears: A History of the
Deaf,
which I opened with indifference, soon to be changed to astonishment, and then to something approaching incredulity. I discussed the subject with my friend and colleague Dr. Isabelle Rapin, who has worked closely with the deaf for twenty-five years. I got to know better a congenitally deaf colleague, a remarkable and highly gifted woman, whom I had previously taken for granted.
¹⁴
I started seeing, or exploring for the first time, a number of deaf patients under my care. My reading rapidly spread from Harlan Lane's history to
The Deaf Experience,
a collection of memoirs by and about the first literate deaf, edited by Lane, and then to Nora Ellen Groce's
Everyone Here
Spoke Sign Language,
and to a great many other books. Now I have an entire bookshelf on a subject that I had not thought of even as existing six months ago, and have seen some of the remarkable films that have been produced on the subject.

The text of this selection has been edited slightly from its original form in
Seeing Voices.

One more acknowledgment by way of preamble. In 1969 W. H. Auden gave me a copy, his own copy, of Deaf
ness,
a remarkable autobiographical memoir by the South African poet and novelist David Wright, who became deaf at the age of seven. “You'll find it fascinating,” he said. “It's a wonderful book.” It was dotted with his own annotations (though I do not know whether he ever reviewed it). I skimmed it, without paying more attention, in 1969. But now I was to rediscover it for myself. David Wright is a writer who writes from the depths of his own experience—and not as a historian or scholar writes about a subject. Moreover, he is not alien to us. We can easily imagine, more or less, what it would be like to be him (whereas we cannot without difficulty imagine what it would be like to be someone born deaf, like the famous deaf teacher Laurent Clerc). Thus he can serve as a bridge for us, conveying us through his own experiences into the realm of the unimaginable. Since Wright is easier to read than the great mutes of the eighteenth century, he should if possible be read first—for he prepares us for them. Toward the close of the book he writes:

Not much has been written about deafness by the deaf. Even so, considering that I did not become deaf till
after
I had learned the language, I am no better placed than a hearing person to imagine what it is like to be born into silence and reach the age of reason without acquiring a vehicle for thought and communication. Merely to try gives weight to the tremendous opening of St. John's Gospel: In the beginning was the Word. How does one formulate concepts in such a condition?

It is this—the relation of language to thought—that forms the deepest, the ultimate issue when we consider what faces or may face those who are born, or very early become, deaf.

The term “deaf” is vague, or rather, is so general that it impedes consideration of the vastly differing degrees of deafness, degrees that are of qualitative, and even of “existential,” significance. There are the “hard of hearing,” fifteen million or so in the U.S. population, who can manage to hear some speech using hearing aids and a certain amount of care and patience on the part of those who speak to them. Many of us have parents or grandparents in this category—a century ago they would have used ear trumpets; now they use hearing aids.

There are also the “severely deaf,” many as a result of ear disease or injury in early life; but with them, as with the hard of hearing, the hearing of speech is still possible, especially with the new, highly sophisticated, computerized, and “personalized” hearing aids now becoming available. Then there are the “profoundly deaf”—sometimes called “stone deaf”—who have no hope at all of hearing any speech, whatever imaginable technological advances are made. Profoundly deaf people cannot converse in the usual way—they must either lip-read (as David Wright did), or use sign language, or both.

It is not merely the degree of deafness that matters but—crucially—the age, or stage, at which it occurs. David Wright, in the passage already quoted, observes that he lost his hearing only after he had acquired language, and (this being the case) he cannot even imagine what it must be like for those who lack or have lost hearing before the acquisition of language. He brings this out in other passages.

My becoming deaf when I did—if deafness had to be my destiny—was remarkably lucky. By the age of seven a child will have grasped the essentials of language, as I had. Having learned naturally how to speak was another advantage—pronunciation, syntax, inflexion, idiom, all had come by ear. I had the basis of a vocabulary which could easily be extended by reading.
All of these would have been denied
me had I been born deaf or lost my hearing earlier than I did.
[Italics added.]

Wright speaks of the “phantasmal voices” that he hears when anyone speaks to him provided he can
see
the movement of their lips and faces, and of how he would “hear” the soughing of the wind whenever he saw trees or branches being stirred by the wind.
¹⁵
He gives a fascinating description of this first happening—of its
immediate
occurrence with the onset of deafness:

[My deafness] was made more difficult to perceive because from the very first my eyes had unconsciously begun to translate motion into sound. My mother spent most of the day beside me and I understood everything she said. Why not? Without knowing it I had been reading her mouth all my life. When she spoke I seemed to hear her voice. It was an illusion which persisted even after I knew it was an illusion. My father, my cousin, everyone I had known, retained phantasmal voices. That they were imaginary, the projections of habit and memory, did not come home to me until I had left the hospital. One day I was talking with my cousin and he, in a moment of inspiration, covered his mouth with his hand as he spoke. Silence! Once and for all I understood that when I could not see I could not hear.
³

Though Wright knows the sounds he “hears” to be “illusory”—“projections of habit and memory”—they remain intensely vivid for him throughout the decades of his deafness. For Wright, for those deafened after hearing is well established, the world may remain full of sounds even though they are “phantasmal.”
¹⁶
intensely vivid for him throughout the decades of this deafness . For Wright, for those deafened after hearings is well established, the world may remain full of sounds even though they are “phantasmal.”
¹⁷

It is another matter entirely, and one that is essentially unimaginable, by the normal (and even by the postlingually deafened, like David Wright), if hearing is absent at birth, or lost in infancy before the language is acquired. Those so afflicted—the prelingually deaf—are in a category qualitatively different from all others. For these people, who have never heard, who have no possible auditory memories, images, or associations, there can never be even the illusion of sound. They live in a world of utter, unbroken soundlessness and silence.
¹⁸
These, the congenitally deaf, number perhaps a quarter of a million in this country. They make up a thousandth of the world's children.

It is with these and these only that we will be concerned here, for their situation and predicament are unique. Why should this be so? People tend, if they think of deafness at all, to think of it as less grave than blindness, to see it as a disadvantage, or a nuisance, or a handicap, but scarcely as devastating in a radical sense.

Whether deafness is “preferable” to blindness, if acquired in later life, is arguable; but to be born deaf is infinitely more serious than to be born blind—at least potentially so. For the prelingually deaf, unable to hear their parents, risk being severely retarded, if not permanently defective, in their grasp of language unless early and effective measures are taken. And to be defective in language, for a human being, is one of the most desperate of calamities, for it is only through language that we enter fully into our human estate and culture, communicate freely with our fellows, acquire and share information. If we cannot do this, we will be bizarrely disabled and cut off—whatever our desires, or endeavors, or native capacities. And indeed, we may be so little able to realize our intellectual capacities as to appear mentally defective.

It was for this reason that the congenitally deaf, or “deaf and dumb,” were considered “dumb” (stupid) for thousands of years and were regarded by an unenlightened law as “incompetent”—to inherit property, to marry, to receive education, to have adequately challenging work—and were denied fundamental human rights. This situation did not begin to be remedied until the middle of the eighteenth century, when (perhaps as part of a more general enlightenment, perhaps as a specific act of empathy and genius) the perception and situation of the deaf were radically altered.

The
philosophes
of the time were clearly fascinated by the extraordinary issues and problems posed by a seemingly languageless human being. Indeed, the Wild Boy of Aveyron, when brought to Paris in 1800, was admitted to the National Institution for Deaf-Mutes, which was at the time supervised by the Abbé Roch-Ambroise Sicard, a founding member of the Society of Observers of Man, and a notable authority on the education of the deaf. As Jonathan Miller writes:

As far as the members of this society were concerned the “savage” child represented an ideal case with which to investigate the foundations of human nature. . . . By studying a creature of this sort, just as they had previously studied savages and primitives, Red Indians and orangutans, the intellectuals of the late eighteenth century hoped to decide what was characteristic of Man. Perhaps it would now be possible to weigh the native endowment of the human species and to settle once and for all the part that was played by society in the development of language, intelligence, and morality.

Here, of course, the two enterprises diverged, one ending in triumph, the other in complete failure. The Wild Boy never acquired language, for whatever reason or reasons. One insufficiently considered possibility is that he was, strangely, never exposed to sign language, but continually (and vainly) forced to try to speak. But when the “deaf and dumb” were properly approached, i.e., through sign language, they proved eminently educable, and they rapidly showed an astonished world how fully they could enter into its culture and life. This wonderful circumstance—how a despised or neglected minority, practically denied human status up to this point, emerged suddenly and startlingly upon the world stage (and the later tragic undermining of all this in the following century)—constitutes the opening chapter of the history of the deaf.

But let us, before launching on this strange history, go back to the wholly personal and “innocent” observations of David Wright (“innocent” because, as he himself stresses, he made a point of avoiding any reading on the subject until he had written his own book). At the age of eight, when it became clear that his deafness was incurable, and that without special measures his speech would regress, he was sent to a special school in England, one of the ruthlessly dedicated, but misconceived, rigorously “oral” schools, which are concerned above all to make the deaf speak like other children, and which have done so much harm to the prelingually deaf since their inception. The young David Wright was flabbergasted at his first encounter with the prelingually deaf.

Sometimes I took lessons with Vanessa. She was the first deaf child I had met. . . . But even to an eight-year-old like myself her general knowledge seemed strangely limited. I remember a geography lesson we were doing together, when Miss Neville asked,

“Who is the king of England?”

Vanessa didn't know; troubled, she tried to read sideways the geography book, which lay open at the chapter about Great Britain that we had prepared.

“King—king,” began Vanessa.

“Go on,” commanded Miss Neville.

“I know,” I said.

“Be quiet.”

“United Kingdom,” said Vanessa.

I laughed.

“You are very silly,” said Miss Neville. “How can a king be called ‘United Kingdom'?”

“King United Kingdom,” tried poor Vanessa, scarlet.

“Tell her if you know, [David].”

“King George the Fifth,” I said proudly.

“It's not fair! It wasn't in the book!”

Vanessa was quite right of course; the chapter on the geography of Great Britain did not concern itself with its political setup. She was far from stupid; but having been born deaf her slowly and painfully acquired vocabulary was still too small to allow her to read for amusement or pleasure. As a consequence there were almost no means by which she could pick up the fund of miscellaneous and temporarily useless information other children unconsciously acquire from conversation or random reading. Almost everything she knew she had been taught or made to learn. And this is a fundamental difference between hearing and deaf-born children—or was, in that pre-electronic era.

Vanessa's situation, one sees, was a serious one, despite her native ability; and it was helped only with much difficulty, if not actually perpetuated, by the sort of teaching and communication forced upon her. For in this progressive school, as it was regarded, there was an almost insanely fierce, righteous prohibition of sign language—not only of the standard British Sign Language but of the “sign-argot”—the rough sign language developed on their own by the deaf children in the school. And yet—this is also well described by Wright—signing flourished at the school, was irrepressible despite punishment and prohibition. This was young David Wright's first vision of the boys: