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Wagner's disease

Wagner's Disease is a familial eye disease of the connective tissue in the eye that causes blindness. Wagner's disease was originally described in 1938. This disorder is frequently confused with Stickler's syndrome, but lacks the systemic features and high incidence of retinal detachments. Inheritance is autosomal dominant. more...

Waardenburg syndrome
Wagner's disease
WAGR syndrome
Wallerian degeneration
Warkany syndrome
Watermelon stomach
Wegener's granulomatosis
Weissenbacher Zweymuller...
Werdnig-Hoffmann disease
Werner's syndrome
Whipple disease
Whooping cough
Willebrand disease
Willebrand disease, acquired
Williams syndrome
Wilms tumor-aniridia...
Wilms' tumor
Wilson's disease
Wiskott-Aldrich syndrome
Wolf-Hirschhorn syndrome
Wolfram syndrome
Wolman disease
Wooly hair syndrome
Worster-Drought syndrome
Writer's cramp


In 1938 Hans Wagner described 13 members of a Canton Zurich family with a peculiar lesion of the vitreous and retina. Ten additional affected members were observed by Boehringer et al. in 1960 and 5 more by Ricci in 1961. In Holland Jansen in 1962 described 2 families with a total of 39 affected persons. Alexander and Shea in 1965 reported a family. In the last report, characteristic facies (epicanthus, broad sunken nasal bridge, receding chin) was noted. Genu valgum was present in all. In addition to typical changes in the vitreous, retinal detachment occurs in some and cataract is another complication.

Wagner's syndrome has been used as a synonym for Stickler's syndrome. Since there may be more than one type of Wagner syndrome, differentiation from Stickler's syndrome is difficult, and authors disagree as to whether these are the same entity. It may be that Wagner has skeletal effects, but not the joint and hearing problems of Stickler's syndrome. Blair et al. in 1979 concluded that the Stickler and Wagner syndromes are the same disorder. However, retinal detachment, which is a feature of Stickler' syndrome, was not noted in any of the 28 members of the original Swiss family studied by Wagner in 1938 and later by Boehringer in 1960 and Ricci in 1961.

Current Developments

An exhaustive genetics study of blood from 54 patients found everyone with Wagner's disease has the same eight "markers," a genetic fingerprint that sets them apart from those with healthy eyes.

The gene involved helps regulate how the body makes collagen, a sort of chemical glue that holds tissues together in many parts of the body. This particular collagen gene only becomes active in the jelly-like material that fills the eyeball; in Wagner's disease this "vitreous" jelly grabs too tightly to the already weak retina and pulls it away.

Most people with the disease need laser repairs to the retina, and about 60 per cent need further surgery.

Also Known As

  • Wagner’s hyaloid retinal degeneration
  • Wagner’s vitreoretinal herdodegeneration

Reference Links

  • Wagner's disease and erosive vitreoretinopathy
  • University of Ottawa Eye Institute


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Rhapsody in blue: the requiem for classical music is premature
From Weekly Standard, The, 11/7/05 by Kelly Jane Torrance

Critics have been sounding the death knell for classical music for years. Concerts are becoming more sparsely attended; radio stations are dropping the classical format; and new music premieres are becoming less and less frequent. As a Chicago newspaper succinctly concluded, "Experience teaches that the class which enjoys classical music is a small minority."

But that was no recent pronouncement; it was made at the end of the 19th century. Joseph Horowitz teaches us, in his commanding new book, that complaints made today about the public's taste--or lack thereof--are nothing new. Critics, composers, and conductors for at least a century-and-a-half have decried a public more interested in the easy and familiar than the new and challenging.

But as Horowitz would have it, it's not their fault. As can be deduced from its subtitle, this book is a passionate, not an "objective," history. The historian, former New York Times critic, and adviser to a number of American orchestras argues forcefully, and much of the time convincingly, for a very specific thesis: "America's music high culture has at all times (alas) been less about music composed by Americans than about American concerts of music composed by Europeans."

Horowitz surveys a century and a half of musical history in this country, and has found a neverending inferiority complex. We have never considered our own music good enough, instead developing first-class institutions that exist almost solely to celebrate the music of another continent. His story is one of orchestras and opera companies rather than composers.

The title is a slight misnomer: Horowitz begins his narrative only in the mid-19th century, focusing on the founding of orchestras in Boston and New York. From the beginning, classical music in America was different from its European progenitor. Its founding is a Jamesian tale of Old World decadence versus New World Puritanism--at least from the point of view of the Americans. Theodore Thomas, the German-born, American-raised conductor who founded Chicago's symphony orchestra, declared that concerts were "sermons in tones" that should be a "character-building force" and an "uplifting influence."

Thomas was by no means alone. "Historically, Americans have inclined to judge and understand high culture for its positive moral content, its power to ennoble and uplift," Horowitz notes. "And they have been quick to mistrust or reject art that seems decadent or blasphemous." So 19th-century tastemakers like the Boston critic John Sullivan Dwight celebrated the edifying power of Bach and Beethoven while 20th-century opera boards rejected Richard Strauss's erotic and violent Salome.

Beethoven, in particular, was worshipped by these puritans as a sort of god. His music, more than any other, was thought to have the ability to make us better people. The New York Philharmonic, the country's oldest orchestra, was founded in 1842 (at the same time as Vienna's, and 40 years before Berlin's). Its serious manner--and unadventurous programming--was part and parcel of its role as cultural uplifter: "The Philharmonic musicians tuned offstage and entered with dignity. All but the cellists performed standing. Of the nine programs constituting the first three seasons, two began with Beethoven's Fifth, two with Beethoven's Third, and two with Beethoven's Seventh."


But puritan Americans weren't shy about appropriating other music to their own, morally acceptable, ends. The country's worship of Richard Wagner at the end of the 19th and beginning of the 20th century is a case in point. As Horowitz writes, "Throughout Europe, Wagnerism attracted aesthetes, decadents, and Symbolists who withdrew into troubled realms of the illogical and unconscious, of disease and psychic disturbance." In America, Wagner was a different bird entirely. His Siegfried was remolded into the prototype of the American frontiersman: "He was Davy Crockett, who refused education and roamed the wilderness." American audiences managed to ignore Wagner's "incestuous lovers and unrepentant sinners."

Wagnermania reached such a fever pitch that it took over the country's preeminent opera company, New York's Metropolitan Opera. The German composer's totalitarian acolytes even silenced Met boxholders, the rich elite chronicled so memorably by Edith Wharton who came to the opera to be seen rather than to see--and who provided much of the company's financial support. (It wasn't Wagner's disciples' politics that soured the Met powers-that-be on the composer: The chairman of the opera company himself was actually denied a box because his parents were Jewish.)

Most of New York's Wagnerians were German in origin. In fact, so were most American music-makers in the 19th century. America, as we are often reminded, is a nation of immigrants. And those immigrants brought their love of music with them across the pond. Italian immigrants brought their opera, for example. But it was the Germans, especially, who had a long musical tradition. So it is no surprise that German composers like Beethoven and Wagner reigned supreme. German musicians and conductors were let go during World War I, to this country's loss, but the canon was already set.

One is left with the impression that America was a victim of her virtues. The country that welcomed the world also welcomed its music, overwhelming its own native talent. Most American composers modeled their works after European forms, producing work that was provincial while it was derivative.

It was a Czech--not an American--who tried to show a way out. Antonin Dvorak arrived in Manhattan in 1892 as director of the National Conservatory. The man who based much of his own work on Bohemian folk songs accepted the challenge of showing Americans how to create a national music: "There is more than enough material here and plenty of talent," he assured us. His achingly beautiful Ninth Symphony, From the New World, was the first American masterpiece, based on Native American and black melodies. Indeed, Dvorak declared, "In the negro melodies of America I discover all that is needed of a great and noble school of music." Horowitz calls Dvorak "wonderfully prescient"--it's just that the school of music he predicted turned out to be jazz, not an American classical music.

Why that never came to be is unclear. Horowitz believes that racism accounts for at least part of it--he quotes all too many contemporary critics and musicians who dismissed the Czech composer as a "nigger-lover." He also makes an interesting case that cities like Boston coddled their composers, stifling them while it supported them. Rebellion has been the source of much great art, after all.

Thus completes the first half of Classical Music in America: its rise. Out of nothing, scrappy Americans created symphony orchestras and opera houses to rival those of Vienna and Berlin. In 1902, Richard Strauss called the Boston Symphony the "most marvelous in the world." Dvorak forged a path to a real original American music. Everything seemed possible.

American art music really began to collapse, Horowitz believes, after the start of World War I. Oddly, that's just when America began to get composers whose names are still very well known--Samuel Barber, Aaron Copland, George Gershwin. But it's the even bigger names with which Horowitz is concerned--Vladimir Horowitz, Leonard Bernstein, and, most especially, Arturo Toscanini.

The Italian-born Toscanini became the music director of the New York Philharmonic in 1929 and the conductor of the NBC Symphony in 1937. The conductor, who was also the subject of an earlier Horowitz book, epitomizes for the author all that went wrong in American classical music. He started the now-ubiquitous tradition of the part-time principal conductor. Toscanini had little interest in championing or even performing new music: "Forty percent of his Philharmonic repertoire comprised music by Beethoven, Brahms, and Wagner." Toscanini's first European tour with the Philharmonic did not include a single work by an American.

But he was wildly popular. This may be where our much maligned celebrity culture began. The Luce press, Time and Life, reported in great detail on the maestro's personal life. The contents of his bag were revealed to a world waiting with baited breath. He became a model for the superstar conductor who devoted himself to interpreting the best music of the past--men like Herbert von Karajan and Leonard Bernstein.

Performers don't fare any better under Horowitz's pen. He indicts the Russian transplant pianist Vladimir Horowitz with a telling quotation. Horowitz once urged a student to exaggerate his playing: "If you play Classic music in correct style on a big piano and in a big hall, it will bore most of the audience." As a Viennese music critic said in another context, "America was truly the promised land, if not of music, at least of the musician."

While Horowitz sees this as a low point, classical music in this country may never have been more popular. "As many Americans know that Toscanini conducts an orchestra as know that Joe DiMaggio plays center field," Life reported in 1939. One wonders how many know the name of the current Philharmonic music director (Lorin Maazel) and that of, say, Kobe Bryant. Neither can one imagine today's cities in as much grief as Chicago was on the death of its beloved orchestra founder, Theodore Thomas: "Chicago's telephone operators were instructed to tell callers, 'Theodore Thomas is dead' before asking, 'Number, please.'"

If Toscanini is Horowitz's villain, the scourge of American art music, then the Boston Symphony's Serge Koussevitzky and the Philadelphia Orchestra's Leopold Stokowski are his heroes. According to Horowitz, these were about the only two conductors interested in American music. (He does a wonderful job of elucidating their charms: "Stokowski's ... is the most feline Beethoven on record. It races or glides on cat's paws, a marvel of rippling musculature, of poised power and energy.")

But Horowitz provides many details that contradict his own complaint that American music wasn't given a chance in its own country. George Whitefield Chadwick, born in 1854, "swiftly became the most performed Boston composer (prior to the arrival of Serge Koussevitzky in 1924, his music was played by the Boston Symphony 78 times)." Of Charles Martin Loeffler, Horowitz writes, "his success as a composer was such that the Boston Symphony performed his music 117 times during his lifetime; his friend Heinrich Gebhard appeared as piano soloist in Loeffler's A Pagan Poem 66 times in 20 years with the orchestras of Boston, Chicago, Cleveland, New York, Philadelphia, Pittsburgh, and St. Louis, among others."

Even in the postwar period, where Horowitz locates classical music's decline, we find examples of orchestras showing the world what home-grown composers could do. A recent Washington Post story on the National Symphony Orchestra's 75th anniversary notes, "The NSO spent the entire summer of 1959 in Latin America, playing 68 concerts in 19 countries, with at least one American composition on every program."

There is plenty of evidence that American orchestras programmed American music. The problem seems to be that audiences weren't that interested. So many of the composers Horowitz tells us about, like Loeffler, are barely known today. The situation only seems to have gotten worse as the 20th century progressed.

Horowitz blames the lack of a vital American school of composition almost solely on the culture of performance--and the market. He is right, of course, to bemoan the situation we now find ourselves in, where most performers and conductors have little connection to the music of their own time. Few of these music-makers champion new American music; fewer still commission any. They still play primarily Bach, Beethoven, and Mozart. And it is also true, of course, that profit-seeking businesses often favor the tried and true over the unknown.

But these aren't the only reasons classical music is, as Horowitz says, "the American performing art most divorced from contemporary creativity." As in other fields, the increase in the number of musical artists working in the academy has had a deleterious effect. Here it is the lack of a real market that is the problem. Writing for each other rather than the public, academic composers have created music that audiences have found to be unlistenable. Listeners--who on the whole prefer tonality to atonality--have, in many cases, rightly rejected music coming out of the ivory tower.

Neither does Horowitz spend much time on the question of music education. There was a time when almost every middle-class home contained a piano. Music was a part of everyday life, not something you listened to out of duty. Today, schools facing budget cuts and increased pressure to prepare students for the "information society" are dropping their music programs at an alarming rate. It's no wonder that, at classical concerts, most of the heads are gray. Optimistic critics tell us not to worry--when people get older and richer, they become more interested in attending concerts. But it's hard to believe that the people who are listening now to Shakira and Snoop Dogg are suddenly going to start listening to Schubert and Shostakovich when they turn 65.

Don't look to classical radio to pick up the slack. Washington, D.C.'s WGMS claims to be "the most-listened-to classical station nationally." Too bad, then, that it is pioneering a dumbing-down of classical radio in America. The station's programming includes "Mozart in the Morning ... guaranteed to perk up your morning commute and get you to work fully charged," and "Rush Hour Relief [with] classical selections perfectly suited to calming your frazzled nerves." Stations used to play entire symphonies and concertos so audiences could hear how a work develops. Gone, too, are the days when classical radio exposed its listeners to the best of new music. Now, stations like WGMS, "your at-work station," tout classical music as something good only for background music.

Because Horowitz has difficulty seeing all the reasons for decline, he can't really point the way forward. There are constant hints here at the need for a government bureaucracy "dispassionately to sort things out." Yet one of the joys of his book is the fascinating collection of stories of very American entrepreneurs who pursued their own vision, tastemakers be damned. These are men like the impresario Oscar Hammerstein, cigar trader and grandfather of the Broadway lyricist, whose Manhattan Opera was much more amenable to modern repertoire than the Met's. Hammerstein offered Salome while the Met banned it after a single performance.

Most telling, perhaps, is Horowitz's phrase for the world in which we live now--"post-classical times." One wonders if he has actually given up on art music in America. He urges the traditional music schools to teach jazz and improvisation; but at the same time, he decries the fact that the Three Tenors have become "the dominant symbol of American classical music." None of the "crossover" acts have strengthened classical music in America. They only dilute and trivialize the music they claim to champion.

To find a solution, you need to understand the problem. And despite some blind spots, Joseph Horowitz has a pretty good grasp of the problem. The critic may emphasize the work of other critics, to the detriment of important composers and conductors. For such a big book, he spends little time on composers like Charles Ives, a versatile composer with a uniquely American sound. And Michael Tilson Thomas, one of this country's preeminent advocates of contemporary American composers, merits only a paragraph. He may ignore how Europe is dealing with a similar problem--there, programmers enjoy some success attracting younger audiences with later, shorter concerts. He may complain about aspects of contemporary performance--the showmanship, the pandering to audiences--that have been there since the beginning.

But Horowitz understands that leaders must challenge their audiences, setting taste themselves rather than allowing it to be set for them. His sprawling, ambitious, insightful book should be read by anyone who cares about art music in America.

Kelly Jane Torrance is books columnist for the American Enterprise Online, arts and culture editor of Brainwash, and fiction editor of Doublethink.

Classical Music in America

A History of Its Rise and Fall

by Joseph Horowitz

Norton, 606 pp., $39.95

COPYRIGHT 2005 News America Incorporated
COPYRIGHT 2005 Gale Group

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