Optic Nerve Hypoplasia, Retinopathy of Prematurity and Musical Savants
By Darold Treffert, MD
The 60 Minutes program of September 28, 2003 — “Musically Speaking” — brings to the fore once again the recurring, fascinating link between vision loss, mental disability and musical genius seen in certain prodigious musical savants over more than a century.
Musical talent as a special ability in savant syndrome has been reported repeatedly and consistently since Dr. J. Langdon Down first described savant syndrome in 1887. Indeed he commented that music was a frequent special skill among savants he had seen in his years as Superintendent at Earlswood Asylum. He noted these savants readily acquired tunes and rarely forgot them. He had one boy in his care who, after attending an opera, would come away with perfect recollection of all the arias, and would continue indefinitely to hum or sing them correctly.
Particularly striking, though, in the reports of musical savants over these past 116 years, and in a number of cases described in detail on this site — see right sidebar — is the very conspicuous, disproportionate regularity with which the triad of blindness, mental disability and musical genius occurs in savant syndrome, particularly when one considers the rarity of each of those components in an already rare condition. That linkage and triad is so frequent that a special school — Soundscape — solely devoted to the musically gifted student with visual impairment opened recently in London and is described in more detail elsewhere on this site. A separate section in Extraordinary People (pages 244-249) discusses this intriguing, interesting triad in considerable depth, but some of those observations are summarized here.
One of the first cases reported in the savant literature with this triad of blindness, mental handicap, and conspicuous musical ability was a woman described by Dr. Alfred Tredgold in his classic chapter on Savant Syndrome in his 1914 textbook Mental Deficiency. There he recounted the instance of a woman brought to his attention by Dr. Ulysses Trelat, at the Salpetriere (a French mental hospital), who had been born blind, had mental retardation in addition to being crippled by rickets, and great musical talent. “Her voice was very correct and whenever she had sung or heard some piece she knew perfectly well the words and the music. As long as she lived they came to her to correct the mistakes in singing of her companions; they asked her to repeat a passage, which had gone wrong, which she always did admirably. One day, Geraldy, Liszt, and Meyerbeer came to the humble singing class of our asylum to bring her encouraging consolations.”
Then in 1849 begins the remarkable story of Thomas Greene Wiggins, better known throughout the remainder of that century as “Blind Tom” Bethune, whose musical talent had already taken him, at age 11, to the White House. After the Civil War his piano concert tours took him around the country and to Europe, where he played for all the musical luminaries of the day. His vocabulary was less than 100 words, but his musical repertoire was over 5,000 pieces. In Philadelphia a panel of 16 outstanding musicians of the day signed a statement with their conclusions about “Blind Tom”: “Whether in his improvisations of performances of compositions by Gottschalk, Verdi, and others; in fact, in every form of musical examination — and the experiments were too numerous to mention — he showed a capacity ranking him among the most wonderful phenomena in musical history.”
Born a little more than a century later, in 1952, Leslie Lemke became themost prominent musical savant of his time. Other persons described on this site — Ellen, Tony, Derek — have become very well knownin recent years. The 60 Minutes piece introduces us to yet another such person — Rex — and others continue to come to attention as well.
Types of Visual Loss in Musical Savants
There are generally two types of visual loss in musical savants with this triad of abilities and disabilities. Until recently the more common source of vision loss was retinopathy of prematurity (ROP). ROP was first described and so-named in 1942. This condition is limited usually, but not always, to infants born prematurely at about 6 months and weighing 3 lbs or less. The retina is most vulnerable at about the sixth month of gestation, and in the prematurely born infant this eye condition, when it occurs, begins 3 to 5 weeks after birth. For a number of years following its discovery, the cause of ROP remained a mystery. Then, in 1951, a clear causal association was made between it and the use of high concentration oxygen in the newborn nursery, especially with premature infants. Since that time strict monitoring and curbs on oxygen levels for premature infants have been instituted and the incidence of ROP has dropped, but not disappeared. Children with ROP show a disproportionately high incidence of a number of developmental disability problems, including autism. There is speculation as to whether these problems are linked directly to ROP, or whether they might be simply another accompaniment of prematurity. As early as 1964 Dr. Bernard Rimland speculated that perhaps the same neurotoxic and neuropathological events that led to ROP in premature infants might be occurring elsewhere in the brain of the developing child to produce some of the typical and characteristic signs and symptoms of early infantile autism so often seen in children with ROP. How those changes relate to the frequency of musical talent in this sub-group of autistic with this triad of musical genius, autism and blindness remains to be explained. This relationship of ROP to autism, and musical savantism, is explored in much more depth in a section of Extraordinary People noted above.
But there is a second cause of visual impairment or loss more recently linked to musical savant abilities. Optic Nerve Hypoplasia (ONH), or underdevelopment of the optic nerves, was once considered rare but is now the leading cause of pediatric congenital vision loss in the United States. Septo-Optic Dysplasia (SOD), sometimes known as DeMorsier’s Syndrome, is a form of ONH with many associated structural abnormalities of the brain, including absence of the Corpus Callosum and/or Septum Pellucidum, abnormalities of the pituitary gland and other endocrine abnormalities. A website at www.focusfamilies.org, hosted by the Focus Families organization, with groups in the United States, United Kingdom., Australia, Canada and Germany serves as a valuable resource for information and education about ONH and SOD, and serves as well as valuable support resource for affected families “to learn more about these conditions and come to a better understanding about out children.” On that website parents describe their observations about these special children-their abilities as well as disabilities-and many have noted increased musical inclination and expertise in these youngsters (much as in William’s Children). They have also noted some autistic-like behaviors and those are described by them on this site as well. A particularly valuable side bar on the United States portion of the Focus Families website is a link to a chart entitled “Autism Spectrum Disorder in Learners with Blindness/Vision Impairments — A comparison of characteristics.” This chart usefully compares and contrasts “typical development,” “Blind/Vision Impaired Development” and “Autism Spectrum Disorder Development” on several scales: Impairments in Social Interactions; Impairments in Communication; Responses to Sensory Information; and Restricted, Repetitive and Stereotyped Patterns of Behavior. These comparisons are important because there are a number of skills and behaviors that children with blindness or visual impairment can have that are similar to, but not the same as, skills and behaviors seen in autistic spectrum disorders. The overlap of certain behaviors in children with vision impairment or loss with those of autism are well known, and reported, and in comprehensive evaluations children with vision impairments or loss it is important to make those differentiations.
Music As a Healer
The music of the savant is delightful in its own right, and we are its beneficiaries. But it has a healing aspect to it also for the savant himself or herself. The 60 Minutes program title “Musically Speaking” is an apt one, for as that program demonstrates, and as Soundscape documents, music provides for the savant the “conduit toward normalization,” particularly language acquisition, that I address elsewhere on this site. Recent research documents that musical exposure can increase verbal memory, and with it, language acquisition. With that increased language skill comes increased social skills as well, all of which are certainly evident in Leslie, Derek, Ellen and Tony and now Rex.
But the musical savant requires some unique and especially tailored approaches, given the several disabilities coupled with some very unique abilities. Information about these special approaches needs to be more widely disseminated, and more widely applied to persons with these special skills and abilities. The Savant Academy, recently established by David Mehnert, whose work with Rex is featured in the 60 Minutes piece, is dedicated to exploring and supporting education of persons with savant syndrome, along with encouraging awareness and research of the condition. It is beginning its work especially with musical savants, with whom Mr. Mehnert is closely involved.
In the original 60 Minutes program in 1983 that featured Leslie the musician, Alonzothe sculptor, and George the calendar calculator — all of whom so many persons remember so well — Morley Safer referred to savant syndrome as a “marvelous mystery.” It is. He also indicated it was a remarkable phenomenon “no one can explain.” Gradually, though, we are making some progress in that regard. There are so many intriguing questions raised by this striking contrast of ability and disability. Those questions become even more intriguing as we question why this particular triad of vision loss, mental disability and prodigious musical ability has continued to appear so conspicuously, with disproportionate regularity, throughout the past century’s musical savants. Newer techniques for study, and newer strategies of teaching and intervention, promise to propel us further along than we have ever been before in helping to explain, and benefit from, the marvelous mystery of the musical savant.
For me, though, whatever scientific interest musical savants hold is paralleled by, and in fact exceeded by, the human interest and inspiration these special persons, and their remarkable families, caretakers and teachers. They provide not only a better understanding not just the musical savant, but a better understanding and appreciation of the specialness and potential and A-bilities of all persons with Dis-abilities. They inspire our diligent search and concerted effort to achieve the full potential within all persons — including ourselves.
Return to Savant Articles
For more information, please contact:
Darold A. Treffert, MD
St. Agnes Hospital, Fond du Lac, Wisconsin
Clinical Professor, Department of Psychiatry
University of Wisconsin Medical School, Madison
Personal website: http://www.daroldtreffert.com