Article Text
Abstract
Francisco Goya (1746–1828), a major Spanish artist, became profoundly deaf aged 46 years, following an acute illness. Despite this, his success continued and he eventually died aged 82 years. His illness is sketchily documented in letters written during his convalescence, describing headache, deafness, tinnitus, unsteadiness and visual disturbance with recovery (apart from deafness) over three months. There was a milder similar illness two years before, suggesting a relapsing condition. Vogt–Koyanagi–Harada syndrome, although previously accepted as Goya’s diagnosis, is not supported by the limited evidence. Susac’s syndrome or Cogan’s syndrome, although both rare, are more likely explanations.
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In Francisco Goya’s (1746–1828) self-portrait (fig 1), his intense expression, dark staring eyes, protruding lower lip and uncontrolled hair (popularly a mark of creative genius) evoke memories of his contemporary, Beethoven. In fact, the similarities go beyond their looks—profound deafness had become a significant and tragic influence for both of them in their behaviour and work.
In the winter of 1792, Goya, then aged 46 years, developed a life-threatening illness, which left him profoundly deaf. Despite this major disability, he subsequently lived a long and productive life and died aged 82 years. His illness, still uncharacterised, was a traumatic turning point for him. The traditional view is that deafness changed him from a colourful playboy to a socially isolated and embittered figure, with painting his principal communication with the world. The truth, however, is more complicated: his darker and more threatening artwork was equally the result of him living through the horrors of the French occupation of Spain and its aftermath.
Although little known outside Spain in his lifetime, Goya is now recognised as an extraordinarily talented, courageous and groundbreaking artist. He was true to his beliefs, exceptionally productive (700 paintings, 900 drawings and almost 300 prints) and had artistic ideas and style far ahead of his time.
BIOGRAPHY
Francisco de Goya y Lucientes was born in 1746 in Fuendetodos, a small town in northeast Spain. The young Goya was boisterous, rebellious and promiscuous but his artistic talent was quickly recognised through teenage apprenticeship to José Luzán y Martínez in the nearby regional capital, Saragossa. In 1773, he married Josefa Bayeu (1743–1812); she had 20 pregnancies, but only six delivered and only one child, Francisco Javier, survived to adulthood. When aged 29 years Goya joined Madrid’s Royal Tapestry Factory where he worked industriously for 17 years. He produced more than 60 full sized, coloured cartoons, for weavers to copy as tapestries for the royal palaces. After years of trying, he was accepted into the Royal Academy of San Fernando aged 34 years and, following several aristocratic commissions, he was made a painter to the King in 1786.
In 1790, Goya became unwell, suggesting the subsequent illness might have been part of a relapsing condition. His letters to Martín Zapater from this time1 give some details:
Undated, mid-November 1790: “Now I am taking myself to bed with the shivers for I am not able to do anything, it must be some sort of chill.”
November 1790: “I am standing up, but feel so ill that I do not know if my head is on my shoulders, and I have no wish for food or anything else.”
Undated, probably end-December 1790: “I’m somewhat better now and steadier. Today I went to see the King ….”
In 1792, Goya developed a more severe illness with the subsequent permanent deafness. He had presented a report to the Academy of Fine Arts on 14th October. He had then left Madrid (without the usual permission that a painter to the King should have sought) and in November fell ill in Seville. The details are sketchy and derive mainly from letters written during his convalescence. Goya was extremely fortunate to be cared for in Cadiz by his friend Sebastián Martínez (1747–1800) (fig 2). It is his and Goya’s letters that provide the only meaningful details of his illness:1
17 January 1793 (Goya to Manuel de Cubas, date marked Madrid but written from Cádiz): “I have been confined to my bed for two months ….”
19 March 1793 (Martínez to Pedro Arascot): “he fell ill in Seville … and arrived at my place in a very bad state, where he has stayed without leaving the house … I fear it will be a lengthy business. He … would wish to write to you himself but is unable to on account of pains in his head, where the whole illness rests.”
29 March 1793 (Martínez to Zapater): “Our friend Goya is progressing and while he is showing some improvement, it is very slow … The noises in his head and the deafness are no better but his eyesight has improved and he no longer has the light-headedness that made him lose his sense of balance. He can now go up and down the stairs perfectly well and can at last manage things that he was unable before.”
31 March 1793 (Martínez to Diego de Gardoqui): “Goya at present absent suffering from paralysis ….”
By 11 July 1793, Goya was well enough to sit again at the Academy of San Fernando. Such were his drive and ambition that he restarted productive work after several months; his first task was a series of 12 small portable oils, his Cabinet Pictures:
4 January 1794 (Goya to Royal Academy): “In order to occupy my imagination which has been so painfully preoccupied with my illness … I have dedicated myself to painting a set of cabinet pictures ….”
Remarkably, despite his disability, Goya reached his career pinnacle as King’s Chief Court Painter aged 53 years. Carlos III (1716–1788), a prudish Catholic but strong supporter of the arts, had nurtured Goya’s career; his benign but less intelligent son Carlos IV (1748–1819), although an ineffectual leader, adored Goya and tolerated his artistic risk-taking and depiction of unconventional themes.
During the Napoleonic occupation (1808–1814) and Carlos IV’s exile, Goya pragmatically remained as Court Painter to the ruling French. The war had begun with the notorious prisoner executions on 2nd and 3rd May 1808, later famously depicted by Goya. The militarily superior French unexpectedly encountered the birth of guerrilla (little war) tactics;2 their frustration as resistance to their rule ran from months into years generated reprisals and terrible cruelties on either side. Sadly, even following the Bourbon monarchy’s restoration with Carlos IV’s son Ferdinando VII, the horrors and devastation continued. Ferdinando’s autocratic regime only encouraged more brutalities—death, torture, rape and famine—legitimised by the Inquisition’s restoration. Goya’s Disasters of War, those print etchings of unspeakable facts of death made at this time, were too shocking for public consumption and were kept from the public view until long after his death.
Even as an old man, Goya’s liberal views threatened the new administration. In 1819, he sought seclusion across the river from Madrid in the Quinta del Sordo (Deaf Man’s House). Withdrawn, elderly, lonely, deaf and probably depressed he gave free rein to a darker style in the Black Paintings, using sinister and supernatural themes. Under pressure from Ferdinando’s regime, old man Goya left Spain for Bordeaux into voluntary exile in 1823,3 taking Leocadia Weiss y Zorrilla, his new partner, and Rosario, her (and possibly Goya’s) daughter. Although stone deaf and non-French-speaking, he found final peace and continued working despite failing health. He died on 16 April 1828 aged 82 years, following a stroke.
WHAT WAS GOYA’S DISEASE?
The limited available information suggests that Goya became bilaterally and profoundly deaf in the context of an acute-onset but prolonged illness characterised by headache, paralysis (possibly ataxia rather than weakness), vertigo, tinnitus, unsteadiness and visual disturbance (possibly oscillopsia rather than ophthalmic disease). The contemporaneously imputed cause—cold exposure while changing a coach axle for the Duchess of Alba4—is clearly wholly inadequate.
Acute deafness with vertigo usually has a peripheral labyrinthine cause, for example, acute viral labyrinthitis, Ménière’s syndrome, autoimmune inner ear disease, or perilymphatic fistula; however, it is almost always (>97%) unilateral. Acute bilateral deafness is mostly idiopathic and often considered viral or autoimmune. The diagnostic possibilities here are inflammatory, vascular or toxic.
Inflammatory causes
Acute viral labyrinthitis, the commonest cause of acute vertigo with deafness, is a syndrome rather than a specific entity. Vestibular neuritis, the commonest cause of sudden disabling vertigo, is often loosely labelled as labyrinthitis but the cochlear nerves are, by definition, unaffected. Ramsay Hunt syndrome, especially without visible rash,5 may present as the labyrinthitis syndrome, with vertigo, vomiting, ataxia, tinnitus and also hearing loss which may be permanent but is rarely bilateral. Goya’s illness does not really fit this description because it lasted for months and headache was a prominent symptom.
Meningococcal or tuberculous meningitis caused deafness long before streptomycin, owing to purulent microvascular cochlear nerve damage. Goya’s acute illness was insufficiently documented to know the speed of onset or severity of his headache, or whether there was photophobia or rash. However, if bacterial meningitis were his illness, Goya was extraordinarily fortunate to survive without specific treatment. Intriguingly, Goya’s self-portrait after 1792 (fig 1) does suggest a mild left facial weakness (note left/right reversal in a self-portrait) implying underlying brainstem or 7th cranial nerve disease, which might complicate meningitis. His facial asymmetry became less evident in a later portrait by his successor as court painter, Vicente López (fig 3).
Brainstem encephalitis, either viral or autoimmune, is a possible explanation. However, deafness from intrinsic brainstem disease is very uncommon because hearing is represented bilaterally in the ascending pathways (lateral lemnisci). The more usual explanation for permanent bilateral deafness rarely accompanying acute demyelinating encephalomyelitis, is either associated peripheral labyrinthine damage, for example, zoster involvement of the cochlear nerves (“Ramsay Hunt syndrome plus”)5 or bilateral cochlear nerve root damage at the entry zones.6
Goya’s wild adolescent lifestyle may have exposed him to the risk of syphilis but his subsequent recovery and good functional health for 40 more years makes untreated syphilis highly unlikely.
Vogt–Koyanagi–Harada syndrome, a predominantly ophthalmological and dermatological syndrome, results from autoimmunity targeting the melanin of inner ear, eyes, hair and skin, causing patches of white hair (poliosis), vitiligo, vertigo, hearing loss and tinnitus, but rarely manifests solely as deafness.7 Although Cawthorne (1965)8 proposed this as Goya’s diagnosis, and this has been widely accepted,9 it is difficult to sustain without supporting features. Goya retained good eyesight (evidenced by his continued unrivalled artistic ability) and his detailed portraits (figs 1 and 3) show no poliosis or vitiligo.
Cogan’s syndrome is a rare non-syphilitic interstitial keratitis with vestibulo-auditory dysfunction primarily affecting young adults.10 The acute illness may first appear following apparent recovery from a flu-like illness, possibly from chlamydia. The eye symptoms usually predominate, with acute pain, photophobia and lacrimation but sometimes the auditory effects occur in isolation. The ear symptoms are usually bilateral, with tinnitus, acute episodic vertigo and sensorineural hearing loss. Cogan’s syndrome is a possible explanation of Goya’s illness, although presumably without the usual predominant ocular features (keratitis, uveitis, episcleritis) since Martínez would otherwise likely have mentioned red eyes when describing the symptoms.
Susac’s syndrome is an autoimmune microangiopathic endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It occurs mostly in young women (3:1) with mean age of onset at 30 years.11 Its characteristic triad is encephalopathy, branch retinal artery occlusions and hearing loss. The encephalopathy presents as headache and confusion and may progress to dementia. Inner ear involvement (hence deafness) develops gradually over several weeks, and although initially unilateral, invariably becomes bilateral and permanent, though rarely complete. Retinal arteriolar occlusions are almost invariable but mostly asymptomatic and improve over time (nowadays with immunosuppression). The prognosis is possibly better in men than women,12 though some are left with neuropsychological sequelae. Susac’s syndrome seems a likely explanation for Goya’s disease.
Vascular causes
Stroke damaging the vestibulo-cochlear nerve root entry zones is rare because this area has a rich collateral blood supply. However, anterior inferior cerebellar artery (AICA) occlusion is, after labyrinthitis, the main differential diagnosis of acute vertigo with deafness, especially where there are vascular risk factors. In addition there is vomiting, tinnitus and brainstem signs including ataxia, bilateral facial weakness and crossed hemianaesthesia. Usually patients are permanently disabled, although some have only hearing loss and vertigo.13 Rarely, complete basilar artery occlusion may present only as isolated bilateral deafness with vertigo, provided anterior basilar retrograde flow is well maintained from the carotid artery via posterior communicating arteries.14 As far as we know, however, Goya’s illness was not of sudden onset (and so not “stroke-like”) and severe headache is not common in stroke.
Mitochondrial cytopathies characteristically cause sensorineural hearing impairment, and occasionally acute presentations may mimic viral encephalitis. However, mitochondrial disease as the cause of a monophasic illness in a middle-aged man with no subsequent recurrence makes this diagnosis unlikely.
Toxic causes
Paint pigments in 18th century Spain contained both red lead and highly toxic white lead, often with added mercury. Hand-grinding and mixing of pigments created dust that artists would inhale and ingest. Lead is neurotoxic through its binding to and denaturing of protein sulfhydryl groups; distal cochlear nerve and the lower brainstem auditory pathway damage may cause sensorineural deafness.15 Sudden bony release of lead during immobilisation from another illness may have contributed to Goya’s deafness. Goya’s wife, Josefa, would also have inhaled leaded paint dust; maybe this (rather than the more likely lupus or antiphospholipid syndrome) partly explained her frequent miscarriages, stillbirths, and medically unexplained illnesses documented in Goya’s letters.
The influence of illness on art
One can only imagine Goya’s anguish and isolation, being permanently deaf in a society generally intolerant of disability and with no opportunity for specific rehabilitation. He did learn rudimentary sign language but essentially remained isolated, with painting his main means of communication. Martínez’s close support probably prevented any worse psychological outcome.
However, Goya was determined to show that he could rise above the miseries of his illness. He needed to prove that he could work despite his illness and prove wrong the director of the Royal Tapestry Factory who in March 1794 had written, “Goya finds it absolutely impossible to paint, as a result of serious illness”.2
Goya’s predicament readily explains his focus on disaster and chaos in his first works after recovery. Depression and frustrations inevitably found expression in his work. His Cabinet Pictures (1793–4) depict house fires, shipwreck, robbery, imprisonment and madness. His themes of isolation, imprisonment, being robbed and going mad were powerful metaphors for his predicament.16 Figure 4 illustrates a striking change in his handling of the same theme, the Pilgrimage to the Church of San Isidro (Madrid’s Patron Saint). The first, in his 40s before becoming deaf, is bright, happy and colourful; the second, in his 70s, is dark, sad and macabre.
Goya’s Los Caprichos (1799) (capricho = whim, fancy), a series of 80 etchings satirising the worst of Spanish society, epitomise the thoughts and attitudes of a man coming to terms with his devastating disability. Two in particular illustrate his plight.
The Blowers, Caprichos 48 shows three people being tortured by the noises and blowing of animals and witches; this may reflect Goya’s ongoing troublesome tinnitus. His text reads, “The blowing witches are the most revolting and most stupid in the devil’s league, if they had any sense they would not blow.”
The Chinchilla Rats, Caprichos 50 (fig 5) reflects Goya’s experience of an unsympathetic society. Two noblemen have had their brains removed and have locks covering their ears. Goya’s accompanying quotation reads, “People who hear nothing, people who are ignorant and never do anything useful, belong to the family of the Chinchilla rats. They are good for nothing.” Goya must have known that certain Chinchilla rat strains are congenitally deaf; to him they reflected people in society whom he believed were ignorant, did not listen, and could do nothing useful for him or his disability.17
Although at first glance, Goya’s paintings reflect a change from bright and light-hearted before his deafness, to darker and more unsettling afterwards, it is clear that his illness had not significantly damaged his artistic ability. In his soundless world he focused more on the macabre and became preoccupied with fantasies. But equally important to his altered painting style was the psychological damage of living through the bloody and brutal war of terror during and after the Napoleonic occupation of Spain, and learning of, and even witnessing, the unimaginable atrocities of that time.
Despite the obvious negatives, Goya’s deafness might in some ways have enhanced his art, since without noise distractions he could more easily immerse himself in the visual medium. Sight was his main sense, and without distracting sounds Goya may have been able better to focus on the visual content of his art. He used his art to express emotions and vent frustrations, particularly on the impact of his deafness on his life. Interestingly, Joshua Reynolds and David Hockney, each with hearing impairment, also noted this as a positive effect of their condition.18
Goya was already well known, well-paid and well-respected at the time of his illness; his profound deafness only increased his notoriety. In this sense his deafness may have helped with his success. It is possible he used his eccentric reputation to push boundaries, encouraging a bolder and more experimental approach to his commissions, and in so doing took considerable personal risks in his dealings with a repressive regime and the notorious Inquisition.
CONCLUSION
The scanty contemporaneous documentation of Goya’s illness means we shall probably never have a definite diagnosis. Any unifying diagnosis must explain several weeks of headache, confusion, ataxia and deafness in a middle-aged man, with subsequent permanent deafness but an otherwise complete recovery over several months, possibly with a previous milder episode two years before, and followed by an artistically productive life to late old age. Labyrinthitis complicated by Ramsay Hunt syndrome is an appealing possibility but permanent complete bilateral deafness would be very unusual. Bacterial meningitis is a possibility but with a remarkable recovery unassisted by antibiotics. The previously accepted diagnosis of Vogt–Koyanagi–Harada syndrome is unlikely given the lack of ocular and dermatological features. An anterior inferior cerebellar artery stroke has to be considered but would be an unlikely cause of subsequent complete deafness without other neurological sequelae. Of the possibilities discussed, Cogan’s syndrome or more likely Susac’s syndrome—although both are extremely rare—seem the most likely explanations for Goya’s disease.
Acknowledgments
This article was reviewed by José Luís Martí-Vilalta, Barcelona, Spain.
REFERENCES
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