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PREVIOUS CASES

The Auditory Agnosias

Jon S. Simons and Matthew A. Lambon Ralph MRC Cognition and Brain Sciences Unit, Cambridge

Auditory agnosia refers to the defective recognition of agnosia, some patients have been described with an apparently auditory stimuli in the context of preserved hearing. There language-specific disorder (Auerbach et al., 1982). Franklin has been considerable interest in this topic for over a hundred ( 1989-see Case P574 below) highlighted five different years despite the apparent rarity of the disorder and potential levels of language-specific impairment that might give rise diagnostic confusion with deafness or even Alzheimer’s to poor spoken comprehension. One of these, word meaning disease (Mendez and Rosenberg, 199 1-see Case P593 deafness, is a form of ‘associative’ auditory agnosia that has below). Following Lissauer’s ( 1890) distinction between fascinated researchers ever since Bramwell first described ‘apperceptive’ and ‘associative’ forms of visual object agno- the disorder at the end of the nineteenth century (see sia, disorders of sound recognition have been divided between Ellis, 1984). impaired perception of the acoustic structure of a stimulus, To be a classic case of word meaning deafness, a patient and inability to associate a successfully perceived auditory should have preserved repetition, phoneme discrimination representation with its semantic meaning (Vignolo, 1982). and lexical decision, but impaired comprehension from Much research has centred on the ‘apperceptive’ form of spoken input alone (comprehension is normal for written auditory agnosia, although the study of such disorders has words and pictures: Franklin et al., 1996; Kohn and Friedman, not been aided by terminological differences in the literature. 1986-see Cases P576 and P589 below). Spoken comprehen- Some authors have used the term ‘auditory agnosia’ to refer sion is graded by imageabilitykoncreteness-words refemng to a generalized disorder affecting the perception of all types to concrete exemplars are more likely to be understood than of auditory stimuli, from non-verbal sounds to speech and abstract concepts-and errors are generally semantically music (e.g., Miceli, 1982). Others have employed separate related (Franklin et al., 1994, 1996-see Cases P575 and names to highlight specific deficits, using ‘auditory agnosia’ P576 below). Word meaning deafness would seem, therefore, to be a language-specific deficit of the highest order although to refer to impairment of non-verbal, environmental sounds Tyler and Moss (1 997-see Case P611 below) have suggested (Albert et al., 1972-see Case P565 below), ‘amusia’ to that, given the speed and accuracy required in speech percep- escribe deficits in the processing of melodies (e.g., Peretz tion, this behavioural pattern may be the natural by-product of et al., 1994-see Case P601 below), and ‘pure’ word deafness a more generalized (earlier) auditory processing impairment. for disorders of speech recognition (e.g., Albert and Bear, Auditory agnosias are often associated with bilateral, 1974; Saffran et al., 1976; Coslett et al., 1984-see Cases or unilateral left, lesions of the superior temporal region Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 P605 and P569 below). While ‘pure’ word deafness is perhaps (Auerbach et al., 1982; Varney and Damasio, 1986) although the most commonly used label, it has been noted for some occasional cases have been described with unilateral right time that the disorder is rarely pure-in the majority of cases temporal involvement (Roberts et al., 1987-see Case P603 there is some concomitant impairment of non-verbal sound below). By far the most common cause is vascular accident processing (Hemphill and Stengel, 1940-see Case P583 but some cases have been reported following encephalitis below; Nielsen, 1946; Wohlfart et al., 1952-see Case P613 (Arias et al., 1995-see Case P566 below), head injury below). Furthermore, many cases are not even free of aphasia (Franklin, 1989-see Case P574 below) and slow progressive (as implied by the original use of the term ‘pure’: Auerbach atrophy (Otsuki et al., 1998-see Case P598 below). Child- et al., 1982-see Case P567 below; Vignolo, 1982). hood auditory agnosia has been described in association with Analysis of impaired speech perception in word deafness epileptogenic, temporal lobe abnormalities on EEG (Kale suggests that certain speech sounds may be affected more et al., 1995; Stein and Curry, 1968-see Cases P586 and than others (Saffran et al., 1976-see Case P605 below). P608) that can improve with anticonvulsant medication Auditory comprehension can be improved by lip-reading (Pearce and Darwish, 1984-see Case P600 below). A (Shindo et al., 1991-see Case P606 below) and context comprehensive review of the current state of the art in this (Saffran et al., 1976). While impaired speech recognition area is provided by Griffiths ef al. (1999) in this issue of normally occurs in the presence of generalized auditory Neurocase. 380 Previous cases: Auditory agnosia

References Lissauer H. Ein Fall von Seelenblindheit nebst einem Beitrage /ur Theorie derselben. Archiv fur Psychiatrie und Nervenkrankheiteii Albert ML. Bear D. Time to understand: a case study of word deafness 1890; 21: 222-70. with reference to the role of time in auditory comprehension. Brain Mendez MF, Rosenberg S. Word deafness mistaken tor Alrheimer'\ 1974; 97: 373-84. disease: differential characteristics. Journal of the American Albert ML. Sparks R, Von Stockert T, Sax D. A case study of auditory Geriatrics Society I99 1 ; 39: 209- I I. agnosia: linguistic and non-linguistic processing. Cortex 1972; 8: Miceli G. The processing of speech sounds in a patient with corticd 12743. auditory disorder. Neuropsychologia 1982; 20: 5-20, Arias M. Requena I, Ventura M, Pereiro I. Castro A, Alverez A. A Nielsen JM. Agnosia, apraxia, aphasia. Their value in cerebral case of deaf-mutism as an expression of pure word deafness: localization. New York: Hoeber, 1946. neuroiniaging and electrophysiological data. European Journal of Otsuki M, Soma Y, Sato M, Homma A. Tsuji S. Slowly progressive Neur~logy1995: 2: 583-5. pure word deafness. European Neurology 1998: 39: 13540. Auerhach SH, Allard T. Naeser M, Alexander MP, Albert ML. Pure Pearce PS, Darwish H. Correlation between EEG and auditory word deafness. Analysis of a case with bilateral lesions and a perceptual measures in auditory agnosia. Brain and Language IW1; defect at the prephonemic level. Brain 1982; 105: 271-300. 22: 41-8. Coslett HB. Brashear HR, Heilman KM. Pure word deafness after Peretz 1, Kolinsky R, Tramo M. Labrecque R. Hublet C. Demeurissc hilaferal primary auditory cortex infarcts. Neurology 1984; 34: G ef a/. Functional dissociations following bilateral lesions of 347-52. auditory cortex. Brain 1994; 1 17: I 283- I30 I. Ellis AW. Introduction to Bramwell's (1897) case of word meaning Roberts M, Sandercock P, Ghadiali E. Pure word deafness and tleafness. Cognitive Neuropsychology 1984; 1 : 245-58. unilateral right temporo-parietal lesion: a case report. Journal of Franklin S. Dissociations in auditory word comprehension; evidence Neurology, Neurosurgery and Psychiatry 1987: 50: 1708-9. froin 9 fluent aphasic patients. Aphasiology 1989; 3: 189-207. Saffran EMM. Marin 0s. Yeni-Komshian GH. An analysis of speech Franklin S. Howard D, Patterson K. Abstract word meaning deafness. perception in word deafness. Brain and Language 19'76: 3: 209-28. Cognitive Neuropsychology 1994; 11: 1-34. Shindo M, Kaga K, Tanaka Y. Speech discrimination and lip reading Franklin S, Turner J, Lainbon Ralph MA, Morris J, Bailey PJ. A patients with word deafness or auditory agnosia. Brain and distinctive case of word meaning deafness? Cognitive in Neuropsychology 1996; 13: 1 139-62. Language 199 1 ; 40: 153-6 I. Griffiths TD, Rees A, Green GR. Disorders of human complex sound Stein LK, Curry FK. Childhood auditory agnosia. Journal of Speech processing. Neurocase 1999; 5: 365-78. and Hearing Disorders 1968; 33: 361-70. Hrmphill RE, Stengel E. A study of pure word-deafness. Journal Tyler LK, Moss HE. Imageability and category-specificity. Cognitivc Neurology and Psychiatry 1940; 3: 25 1-62. Neuropsychology 1997; 14: 293-3 18. Kale U. El-Naggar M, Hawthorne M. Verbal auditory agnosia with Varney N. Damasio H. CT scan correlates of sound recognition defeci focal EEG abnormality: an unusual case of a child presenting to in aphasia. Cortex 1986; 22: 483-6. an ENT surgeon with 'deafness'. The Journal of Laryngology and Vignolo LA. Auditory agnosia. Philosophical Transactions of thc Otology 1995; 109: 431-2. Royal Society of London B: Biological Sciences 1983: 298: 19-57. Kohn SE. Friedman RB. Word-meaning deafness: a phonological- Wohlfart G, Lindgren A, Jernelius B. Clinical picturt: and morbid semantic dissociation. Cognitive Neuropsychology 1986; 3: 291- anatomy in a case of 'pure word deafness'. Journal of Nervous arid 308. Mental Disease 1952; 116: 818-27. Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Previous cases: Auditory agnosia 38 1

A case study of auditory agnosia: A case of deaf-mutism as an expression linguistic and non-linguistic processing of pure word deafness: neuroimaging and electrophysiological data M. L. Albert, R. Sparks, T. von Stockert and D. Sax M. Arias, I. Requena, M. Ventura, I. Pereiro, Abstract A. Castro and A. Alverez The authors have evaluated at length an individual who had as a major neurological defect an impaired ability to recognize thc nature of heard non- Abstract verbal sounds. that is. auditory agnosia. This case provides additional clinical The authors report a case of pure word deafness. clinically expressed as deaf- support for the theoretical position that there may be two central auditory mutisni in a 17-year-old girl. who was affected with encephalitis when she processing mechanisms. one treating linguistic inputs and the other non- was I8 months old and had not acquired language skills. Actually. physical linguistic inputs. examination revealed buccolingual apraxia and absence of spontaneous speech, auditory comprehension, repetition and denomination, whereas perception of Journal non-verbal sounds was preserved. The seven waves of brainstem auditory Cortex 1972; 8: 12743 evoked reaponses (BAER) were present with normal latencies; middle latency responses (MLR) were also normal. Magnetic resonance imaging (MRI) Neurocase Reference Number: showed bilateral temporoparietal lesions. This case proves that lesions which P565 may give rise to word deafness. when they occur in prelingual age, can determine a peculiar deaf-mutisni clinical picture. Primary diagnosis of interest Auditory agnosia. auditory neglect, aphasia Journal European Journal of Neurology 1995; 2: 583-5 Author’s designation of case Not mentioned Neurocase Reference Number: P5h6 Key theoretical issue 0 The auditory agnosia seen in this patient supporta the view that there are Primary diagnosis of interest separate verbal and non-verbal auditory processing systems Prelingual. bilateral temporoparietal lesions

Key \vord.~auditory agnosia Author’s designation of case Not mentioned Scan, EEG and related measures EEG. radioisotope scan Key theoretical issue 0 The impact of prelingual, bilateral teinporoparietal lesions on the subsequent Standardized assessment development of spoken language comprehension and auditory agnosia Seashore Measures of Musical Talents, Boston Diagnostic Aphasia Examination. Wechsler Adult Intelligence Scale Key UY~/.Y:pure word deafness; deaf-mutism; childhood encephalitis Other assessment Scan, EEG and related measures Audiometric screening. spontaneous speech, naming. comprehension of spoken EEG, MRI. Brainstern Auditory Evoked Responses (BAER) language, Repetition tests, reading. writing, singing. calculation. sound localization, dichotic aounds, recognition of non-verbal sounds Standardized assessment Pure-tone audiogram Lesion location BilateIal posterior cortex Other assessment Clinical assessment of non-verbal sound recognition. and buccolingual apraxia Lesion type Not known Lesion location 0 Bilateral corticosubcortical temporoparietal lesions (MRI)

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Language English Lesion type Encephalitis Language Engliah 382 Previous cases: Auditory agnosia

Pure word deafness. Analysis of a case Chronic auditory agnosia following with bilateral lesions and a defect at the Landau-Kleffner syndrome: a 23 year prephonemic level outcome study

S. H. Auerbach, T. Allard, M. Naeser, K. Baynes, J.A. Kegl, D. Brentari, C. Kussmaul M. P. Alexander and M. L. Albert and H. Poizner Abstract Abstract 'The authors have demonstrated that the deficit in speech perception in a The authors report on a 27-year-old woman with chronic iiuditory agnosia patient with pure word deafness is secondary to a prephoneinic temporal following Landau-Kleffiier Syndrome diagnosed at age If years. She gIeu auditory acuity disorder. They delineated the nature of the auditory processing up in the hearinghpeaking coinmunity with some exposure to inanually coded delicit in the patient with bilateral lesions and then demonstrated the presence English and American Sign Language. Manually coded (signed) English is of a predicted delicit in phonemic discrimination. This pattern is comparable her preferred mode of communication. Comprehension and production ol' to previous cases with bilateral lesions and distinct from other cases with spoken language remain severely coinpromiaed. Disruptions in auditory unilateral lesions. Review of previous reports suggests that there are two processing can be observed in tests of pitch and duration. suggesting that her distinct types of pure word deafness: type I. in which the deficit is prephonemic disorder is not specitic to language. Linguistic analysis of signed. qmkcn, iind and related to a tempornl auditory acuity disorder, and type 2. a form that is written English indicates her language system is intact. but compromiwd independent of ii temporal auditory acuity disorder. and has a deficit in because of impoverished input during the critical period for acquihititrii ol' linguistic discrimination that does not adhere to a prephonemic pattern [Denes spoken phonology. Specilically. although her sign langungc phonolog) is ;ind Seincnia, 1975 (P.57 I ): Saffran et (I/., 1976 (P60S)l. The former has been intact, spoken language phonology is markedly impaired. The author\ arguc associated with bilateral temporal lobe lesions [Naeser, 1974 (presented at the that deprivation of auditory input during a period critical for the development 17th Annual Academy of Aphasia Meetings, Warrenton. VA, October 1974); of a phonological grammar and auditory-verbal short-term memory has limited Chocholle PI d., Neuropsychologia 1975; 13: 162-721. the latter with left her lexical and syntactic development in specitic ways. unilateral lesions 1 Denes and Semenza, 1975 (PS7 I); Saffran et d.,1976 (P60S)l. The first fornm is an apperceptive disorder. whereas the second Journal represents il higher disorder in phonemic discrimination and may be considered Brain and Language 1998; 63: 3x1425 ii fragment of Wernicke's aphasia. Neurocase Reference Number: Journal PS6X Brain 19x2; 105: 271-300 Primary diagnosis of interest Neurocase Reference Number: Auditory agnosia, Landau-Kleffner syndrome. sign language PSh7 Author's designation of case Primary diagnosis of interest TM Pure word deafness Key theoretical issue Author's designation of case 0 Failure of patient to develop spoken English is due to distorted auditory Not mentioned input during critical period for auditory language acquisiti,.iri. rather than damage to language system itself Key theoretical issue Differcntiation of pure word deafness froin general auditory agnosia Key ~wd.~:auditory agnosia: Landau-Kleffner syndrome: uphdsia

A'rv iiwds: pure word deafne. Scan, EEG and related measures MRI. PET, EEG Scan, EEG and related measures CT. isotope brain scan, EEG Standardized assessment WAIS, Raven Progressive Matrices, WMS-R, WRAT3. Finger Oscillatiori, Standardized assessment Benton Facial Recognition Test, Trail Making Test, Wisconsiii Card Soiling

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Sprecn and Benton Sound Recognition Test. Brainstem Auditory Evoked Test. Pyramids and Palm Trees Test, Boston Naming Test. PPVT-R Kespon\e\ Other assessment Other assessment Audiometric screening, sound discrimination tests. speech discriniination test\. Pure tone threshold audiometry. dichotic listening studies, cortical auditory written grammar and comprehension tests cvoked potentials. temporal auditory acuity measures. natural and synthetic speech mind tests, music tests. sound discrimination, recognition of Lesion location Ti)Vir(iiiiiit'nl;iI sounds 0 PET Bilateral temporal lobe Lesion location Lesion type 0 Right middle cerebral infarct, extending froin the right middle and posterior Hypometabolism temporal region to the parietal lobe, including the postcentral and .;upramarginal gyri: and a sinall. older non-enhancing left posterior temporal Language lohe intarct including part of Wernicke's area. English Lesion type C VA Language English Previous cases: Auditory agnosia 383

Pure word deafness after bilateral Pure word deafness and bilateral primary auditory cortex infarcts posterior perisylvian softenings: report of a case with neuropsychological-CAT H. B. Coslett, H. R. Brashear and correlation K. M. Heilman Abstract M. Dalla Pria, H. Spinnler and G. Vallar In pure word deafness. comprehension and repetition of speech are impaired. but reading, writing. and spontaneous speech are preserved. Pure word Abstract deafness is distinguished from generalized auditory agnosia by the preserved A case of pure word deafness (PWD) with bilateral perisylvian softenings is ability to recognize environmental sounds. The authors examined a patient described. The CAT findings fit the classical anatomical counterpart of PWD: with pure word deafne. .ociated with bilateral infarctions of the primary a nearly complete acoustic disconnection of the Wernicke area. The associated auditory cortex, who could use auditory affective intonation to enhance severe apraxic disorders are discussed. comprehension. The primary auditory cortex seems to be essential for comprehending speech, but comprehension of non-verbal sounds and affective Journal prosody may be mediated by other cerebral structures such as the auditory Archives Suisses de Neurologie, Neurochirurgie et de Psychiatrie 1979; 125: association cortex. 47-58 Journal Neurocase Reference Number: Neurology 1984; 34: 347-52 PS70 Neurocase Reference Number: Primary diagnosis of interest P569 Pure word deafness Primary diagnosis of interest Author’s designation of case Pure word deafness Not mentioned Author’s designation of case Key theoretical issue Not mentioned 0 Neural basis of pure word deafness Key theoretical issue Kr? ~~rds:pure word deafness Preserved recognition of environmental sounds and affective intonation Scan, EEG and related measures Key kvorrl.7: pure word deafness; auditory agnosia; recognition of affective CAT, repeated technetium brain scans prosody Standardized assessment Scan, EEG and related measures Token Test. Raven‘s Coloured Matrices. Poppelreuter-Ghent’s Overlapping CT Figures. Univariate Elementary Patterns, Farnsworth Test, Colour-Figure Matching Test Standardized assessment Boston Diagnostic Aphasia Examination Other assessment Sound discrimination tests, pure tone audiometry Other assessment Pure tone audiometry, competing environmental sounds. staggered spondaic Lesion location words, synthetic sentence index. Ekman faces 0 Bilateral hypodense area in the first temporal and supramarginal gyri and in the pre- and post-rolandic regions. On the right side, additional hypodense Lesion location area in second and third gyri Enhancing infarct in the mid-portion at the left superior temporal gyrus involving Heschl’s area plus an old infarct involving the middle and Lesion type superior temporal gyri: the supramarginal gyrus; inferior portions of Bilateral perisylvian softenings Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 the postcentral. central. and precentral gyri; and the right inferolateral frontal lobe Language English Lesion type CVA Language English 384 Previous cases: Auditory agnosia

Evidence from a case of pure word Clinical and neuroradiological findings deafness in a case of pure word deafness

G. Denes and C. Semenza M. Di Giovanni, G. D’Alessandro, S. Haldini, Abstract D. Cantalupi and E. Bottacchi ical syndrome of pure word deafness following a Abstract detailed neuropsychological examination showed an Pure word deafness is a clinical syndrome included among the aphasias and idmost absolute inability to name meaningful non-verbal sounds, in spite of is marked by complete deafness of sudden onser with conserved ability to normal recognition as demonstrated by ability to match the sound with the understand and read the written word and with no speech disorders. Thc corresponding nilturd source. This selective anomia. confined to auditory authors report the case of a 61- year-old man in whom pure word de;ifne\\ modality. is discussed in view of previous research in the general held of developed after two episodes of acute cerebral ischaemiu in qiiick succc\slon naming disturbances. which showed that for the vast majority of aphasics, Neuroimaging procedures. that is: computed tomography \c;ui, single photon anoniia wiis not niodality-dependent. Besides that. the contrasting performance emission computed tomography and magnetic resonance imaging. ~rcvenlcd of the patient. who showed complete lack of phonemic analysis matched with the presence of two ischaemic lesions in the temporal cortex bil;itcrally. nornial recognition of some acoustic parameters of speech such as prosody Neurophysiological investigations (electroencephalogram. breinstcm ditory and stress. is discussed. evoked potentials and stapedial reHex) were also srudieil. The ;iuthor\ Journal discuss the outcnine of all these investigations in the light of the rclcv;int published work. Cortex 1075: I I: 401-1 I Neurocase Reference Number: Journal The Italian Journal of Neurological Sciences 1902; 1.7: 507-11) 1’57 I Primary diagnosis interest Neurocase Reference Number: of P572 Pure word deafness Author’s designation of case Primary diagnosis of interest Pure word deafness RK Key theoretical issue Author’s designation of case Not mentioned 0 Comprehension but iinomia for non-verbal sounds in a case of pure word deiifness Key theoretical issue 0 Neurophysiological and structural bases for purc word denlness Kc,! il,ords: pure word deafne. nomia; environmental sounds Scan, EEG and related measures Key 1VfJrdS: pure word deafness; temporal ishemi EEG Scan, EEG and related measures Standardized assessment CT. HMPAO-SPECT. EEC WAIS. Token Test Standardized assessment Other assessment Brainstein auditory evoked potentials Goldman perimctry. audiomctric screening, sound localization, rhyming ability, Lesion location musical sequence discriminatinn. timbre, pitch and loudness discrimination, 0 Bilateral temporal lobe young-old voice discrimination, foreign word discrimination. semantic hearing test. recognition of non-verbal sounds Lesion type Lesion location Cerebral embolism 0 EEG abnormalities over left temporal lobe Language Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Lesion type English Not mentioned Language English Previous cases: Auditory agnosia 385

Right hemisphere: small lesion of posterior part of the superior temporal The functional anatomy of recovery gyrus, including part of the transverse temporal gyrus (Heschl’s gyrus, from auditory agnosia. A PET study of primary auditory cortex) and posterior and ventral part of the insular cortex sound categorization in a neurological Lesion type patient and normal controls CVA Language English A. Engelien, D. Silbersweig, E. Stern, W. Huber, W. Doring, C. Frith and R. S. Frackowiak Abstract H2(15)0-PET was used to investigate the functional anatomy of recovery in a patient (J.B.) with bilateral perisylvian strokes and auditory agnosia, who partially regained the ability to recognize environmental sounds, but remained clinically word-deaf. The patient and a group of six normal volunteers were scanned in the following three conditions: (i) passive listening to environmental sounds: (ii) categorization of environmental sounds; (iii) at rest. In normal subjects. passive listening as compared with rest was associated with significant activations in the auditory cortices and posterior thalami. and in the inferior parietal lobe and anterior insulzdfrontal opercular region on the right. In J.B., activations were observed in the spared auditory cortex and inferior parietal lobe of the right hemisphere and in regions adjacent to the perisylvian lesion in the left hemisphere (anterior insuldfrontal opercular region, middle temporal gyrus and inferior parietal lobe). The recovered function, as measured by categorization olsounds compared with passive listening, in J.B. was associated with bilateral activation of a distributed network comprising (prefrontal. middle temporal and inferior parietal cortices, as well as the right cerebellum and the right caudate nucleus. In addition. there was a left-sided activation of the anterior cingulate gyrus. In normal subjects, the same categorization task led to activation of a network comprising (pre)frontal, middle temporal and inferior parietal cortices in the left hemisphere only. These results suggest that bilateral activation (with recruitment of areas homologous to those known to be responsible for normal function). the engagement of peri-infarct regions, and the involvement ofa more widespread neocortical network, are mechanisms of functional reorganization after injury that may enable recovery from. or compensation for. cognitive deficits. Journal Brain 1995; 118: 1395-409 Neurocase Reference Number: P573 Primary diagnosis of interest Auditory agnosia Author’s designation of case JB Key theoretical issue 0 Functional anatomy of recovery in a case of auditory agnosia Downloaded by [The University of Manchester Library] at 13:18 22 June 2012

Key work auditory agnosia; recovery: audition: cognition: PET Scan, EEG and related measures PET, MRI, SPM maps Standardized assessment Leistungsprufsystem, Corsi Block Tapping, Nonverbaler Lerntest, Verbaler Lerntest, Aachen Aphasia Test Other assessment Audiometric screening, logical reasoning task. auditory tasks with environmental sounds, linguistic and paralinguistic auditory tasks. passive listening versus rest. categorization versus listening Lesion location Left hemisphere: lesioning of third frontal gyrus, inferior parts of the pre- and post-central gyri, superior temporal gyrus, supramarginal gyrus, insula and deep basal ganglia. Primary auditory cortex completely destroyed. White matter underlying middle temporal gyrus and inferior parietal lobe partly destroyed 386 Previous cases: Auditory agnosia

Dissociations in auditory word Abstract word meaning deafness comprehension; evidence from 9 fluent aphasic patients S. Franklin, D. Howard and K. Patterson Abstract The authors report the case of a patient. DRB, who shows irnpaired auditory S. Franklin comprehension of abstract hut not concrete words. in the context of preserved Abstract comprehension of all types of written words. Good performance on auditory Traditional aphasia classifications do not allow for a detailed description of lexical decision for abstract words indicates that DRB is ablr to access these auditory comprehension impairments. A cognitive neuropsychological model lexical entries. This pattern may be termed 'abstract word meaning dcafnesh'. Repetition is also more impaired for abstract words than concrete words. of lexical processing allows us to distinguish at least five different levels of DKH impairment in single word auditory comprehension. It also specifies a is unable to repeat non-words or to write them to dictation. He makes seiiiiintic more complex relationship between impairments in repetition and auditory errors in real word repetition, which is compatible with the view that his comprehcnsion. Nine fluent aphasic patients. with auditory comprehension repetition is semantically mediated. Exactly the same pattt:rn of seniantic disorders. were accessed using tests of phoneme discrimination, lexical errors and a particular difficulty with abstract words is found in tasks where decision. synonym matching and word and non-word repetition. The results DRB is required to write to dictation. DRB's auditory comprehension and of these tests. as predicted, indicated that there were five dissociable levels repetition impairments are not affected by word frequency. Performance on of impairment. although there was some evidence for interaction between specific words is inconsistent. and is improved following a writtcn cue. levels. The patients showed qualitative differences between auditory and written comprehension. Four of the patients had impairments in repetition Journal despite having no impairment in phoneme discrimination tests. No two patients Cognitive Neuropsychology 1994; I I: 1-34 showed exactly the same pattern of impairment across all the tests administered. Neurocase Reference Number: Journal P575 Aphasiology 1989: 3: 189-207 Primary diagnosis of interest Neurocase Reference Number: Word meaning deafness P574 Author's designation of case Primary diagnosis of interest DRB Forms of word deafness Key theoretical issue Author's designation of case 0 Clinical presentation of word meaning deafness ES. FM. NIi EC, AH, MK. DRB, CJ. D1 Key words: word meaning deafness: imageability: repetition Key theoretical issue Scan, EEG and related measures 0 Clinical presentation of five different forms of word deafness CT Kc,y iiwdx: pure word deafness; word-form deafness; word meaning deafness; auditory agnosia; repetition Standardized assessment Raven's Standard Progressive Matrices, British Picture Vocabulary Scale. Test Scan, EEG and related measures for the Reception of Grammar Not mcntioned Other assessment Standardized assessment Pure tone audiometry. Pyramid and Palm Trees test. spoken and written word Not mentioned comprehension, phoneme discrimination, lexical decision. dictarion. re petit it)^^ Other assessment Lesion location Phoneme discrimination. lexical decision, synonym matching, repetition Left middle cerebral infarct Lesion location Lesion type Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Ncit mentioned CVA Lesion type Language All CVA except AH - head inury English Language English Previous cases: Auditory agnosia 387

A distinctive case of word meaning Auditory sound agnosia without aphasia deafness? following a right temporal lobe lesion

S. Franklin, J. firner, M. A. Lambon Ralph, T. Fujii, R. Fukatsu, S. Watabe, A. Ohnuma, J. Morris and P. J. Bailey K. Teramura, I. Kimura, S. Saso and K. Kogure Abstract Abstract The authors report the case of a patient DrO. who has impaired auditory A 55-year-old right-handed man showed inability to recognize the meaning comprehension but intact written comprehension. His ability to repeat words of non-verbal sounds without impairment of language comprehension after a that he cannot comprehend. and his good performance on auditory lexical ular accident. His auditory acuity was intact and no other sign of decision, suggest that DrO has an impairment of the mappings between the agnosia. apraxia or aphasia was detectable. His errors on a test of sound lexical representations of spoken words and their corresponding semantic recognition were acoustic rather than semantic. Brain CT scan showed a small representations; a word meaning deafness. DrO's ability to understand heard lesion in the posterior part of the right temporal lobe. This case suggests that words depends on their imageability and length, such that he is worse at auditory sound agnosia without language disorder can ensure a lesion confined comprehending words that are both short and abstract. This is interpreted in to the right hemisphere. and that the deficit is discriminative rather than terms of phonological and semantic distinctiveness. associative in nature. Journal Journal Cognitive Neuropsychology 1996; 13: I I3942 Cortex 1990; 26: 263-8 Published erratum appears in Cortex 1990: 26: 672 Neurocase Reference Number: Neurocase Reference Number: P576 PS77 Primary diagnosis of interest Primary diagnosis of interest Word meaning deafness Auditory agnosia Author's designation of case Author's designation of case DrO Not mentioned Key theoretical issue Key theoretical issue Description of a disorder in which impaired comprehension of spoken Auditory agnosia for non-verbal sounds in context of preserved verbal words occurs without generalized semantic or phonological impairment comprehension may be associated with selective damage to right hemisphere

Key ~'urds:word meaning deafness; imageability Key \vort/.r: auditory agnosia; right hemisphere: aphasia Scan, EEG and related measures Scan, EEG and related measures CT CT Standardized assessment Standardized assessment Not mentioned Standard Language Test of Aphasia. WAIS. Seashore Measure of Musical Talents Other assessment Pure tone audiometry. spoken and written word comprehension, sound and Other assessment phoneme discrimination, repetition. lexical decision Audiometric screening. recognition of non-verhal sounds Lesion location Lesion location Left middle cerebral infarct Posterior part of right temporal lobe Lesion type Lesion type CVA Hyperdensity Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Language Language English English 388 Previous cases: Auditory agnosia

Pure word deafness and hemispheric Psychoacoustical deficits related to dynamics: a case history bilateral subcortical haemorrhages. A case with apperceptive auditory agnosia M. S. Gazzaniga, A. V. Glass, M. T. Sarno and J. B. Posner 0. Godefroy, D. Leys, A. Furby, J. DeReuck, Abstract C. Daems, P. Rondepierre, B. Debachy, The authors report on the effects of a presumed unilateral brain lesion on J. Deleume and A. Desaulty language functions in ii case of 'pure word deafness'. The patient was totally iinp;iircd in his auditory perception of speech while retaining much written Abstract coiiiprehension. spontaneous speech and writing. As is usually the case, the The authors report a case of acute deafness secondary to bilateral haeniorrhnge\ word tlr;ilness was contaniinated by some indication of auditory agnosia and involving the external capsule and extending to both tenipord isthmi. The \om specific aphaisc deficits. The patient's performance on auditory-tactile lesions probably disrupted both auditory radiations. Deafne!.s disappeared matching tests provides a striking contrast with the performance of split brain within 2 weeks leading to a transient auditory agnosia for environmental and patients. The right hemisphere in these patients is able to make both a visual- verbal sounds. Perforniance on audiological and neurolingui!dic tests were tactile and ;in auditory-tactile match between word and object. This case. consistent with the hypothesis of a deficit of non-specific auditory processes. Iioucver. wiis unehle to make an auditory-tactile match. A possible explanation Psychoacoustical deficits related to subcortical lesions are very rare and might ltir the I;wh of right hemispheric language function in this case is offered. differ from those due to conical lesions by the lack of nphasi,i. The present The damaged left hemisphere may have an interfering effect on the potential case and both previous cases with subcortical lesions might suffer froni lingui\tic c;ipacity of the non-domimant hemisphere. auditory agnosia of apperceptive type. Its characteristics and tlie role of non- specific auditory processes are discussed using the data ohtaincd troin thi\ Journal third case. ('ortcr 1972: 0: 13643 Journal Neurocase Reference Number: Cortex 1995; 31: 149-59 PS7X Neurocase Reference Number: Primary diagnosis of interest P.579 Pure word tle;it'ness Primary diagnosis of interest Author's designation of case Apperceptive auditory agnosia WH Author's designation of case Key theoretical issue Not mentioned 0 Hilateral functional impairment froni a unilateral left lesion producing pure word de;ltness Key theoretical issue Auditory agnosia without aphasia can result from huhcortical Icsions spiiring h'c,,~ii,orc/,\: auditory agnosia; pure word deafness the temporal cortex

Scan, EEG and related measures Kq words: auditory agnosia; deafness; subcortex: apperceptive agnosia Carotid nrteriogram Scan, EEG and related measures Standardized assessment CT. MRI, PET Henton Sound Recognition Test. Benton and Spreen Neurosensory Center Comprehensive Examination for Aphasia. Token Test Standardized assessment MMSE, WAIS, PM38. Peterson Paradigm, Battery 144. Gestural Apraxia. Other assessment Cancellation Test. Rey's Figure copying, Shortened Token Test. BDAE Audiometric testing, speech discrimination. lateralization of sound. delayed auditory feedhnck. word discrimination. short-term memory Other assessment Audiometric screening, brainstem auditory evoked potentials. aound Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Lesion location recognition test, neurolinguistic assessment, oral comprehension. repetition. "it inentionecl naming, fluency Lesion type Lesion location C'VA MRI: Bilateral external capsule extending to teniporal isthmuy Language Lesion type ilnglish Haemorrhage Language English Previous cases: Auditory agnosia 389

A 66-year-old woman with a rapidly Auditory agnosia and cortical deafness: progressing dementia and basal ganglia analysis of a case with 3-year follow-up involvement M. N. Goldstein, M. Brown and J. Hollander M. Gold, A. Rojiani and R. Murtaugh Abstract Studies of a patient with an auditory recognition disorder are presented. These Abstract are discussed in light of their relation with decreased auditory acuity. Auditory A 66-year-old woman presented with a rapidly progressive dementia initially agnosia is not necessarily a static sign: improvement may occur. Auditory characterized by an auditory agnosia. She experienced a rapid progression of agnosia may not be a pure disturbance in cerebral associative function. her aphasia and developed ataxia and myoclonus. An initial neurological evaluation suggested a left parieto-temporal lesion, however, neuroiinaging Journal did not reveal any. An MRI of her brain demonstrated highly focal T2 Brain and Language 1975; 2: 324-32 hyperintensitiea in her basal ganglia. The patient was diagnosed with Creutzfeldt-Jacob disease (CJD) on the basis of the presence of two proteins Neurocase Reference Number: in the cerebrospinal fluid that are highly sensitive and specific for CJD. P58 I Pathological examination confirmed the diagnosis. The differential diagnosis and utility of MRI in patients with CJD is discussed. Primary diagnosis of interest Journal Auditory agnosia Journal of Neuroiinaging 1997; 7: 171-5 Author's designation of case Neurocase Reference Number: JS PS80 Key theoretical issue 0 Patient presented with generalized auditory agnosia which improved over Primary diagnosis of interest time until speech comprehension was the only major deficit Auditory agnosia: Creutzfeldt-Jacob disease Author's designation of case Kr! bvordv: auditory agnosia: pure word deafness Not mentioned Scan, EEG and related measures Key theoretical issue EEG Auditory agnosia may be a presenting feature of CJD Standardized assessment Porteus Maze. Bender Gestalt Test. Standard Achievement Test, Picture Key words: auditory agnosia; CJD; aphasia Interest Inventory. Differential Aptitude Tests, WAlS Scan, EEG and related measures Other assessment MRI. EEG, lumbar puncture Audiometric screening. speech .sound discrimination. ineinory tasks, sound Standardized assessment localization. pulse tone patterns reproduction. rhythmic stimuli reproduction None mentioned Lesion location Other assessment 0 Bilateral frontal and temporal regions. more pronounced on right Tests of verbal and visual memory, oral comprehension. repetition, reading, Lesion type object naming. writing Possible necrosis secondary to encephalitis Lesion location Language MRI: basal ganglia English Lesion type Hyperintensity Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Language English 390 Prcvious cases: Auditory agnosia

Mutism and auditory agnosia due to A study of pure word-deafness bilateral insular damage-role of the insula in human communication R. E. Hemphill and E. Stengel Abstract The authors describe a case in which there was a syndrome cori\isting ol piire M. Habib, G. Daquin, L. Milandre, word deafness and what has been described as nsyniholia for pain. followiii~ M. L. Royere, M. Rey, A. Lanteri, G. Salamon a head injury. The inter-relationship of these two disorders has hecn di\cussed and it has been pointed out that pure word deafness and nsyinholia lor pirill and R. Khalil have the comnion feature of a disturbance in the relationship bc!wcen the Abstract individual and his environment. The localization of the responsible lesion\ has been discussed. Consideration has been given to the problem ol thc A c:iw is reported of transient muiism and persistent auditory agnosia due to position of pure word deafness in the systems of the aphasias IH(I succc5sivc i\chneniic infarct\ mainly involving the insular cortex on both Iiciiii\phcIe\. During tlic 'mutic' period, which lasted about I month, the Journal pitien1 did n(11 rcspond to any ;iuditory stimuli and made no effort to Journal Neurology and Psychiatry 1940: 3: 25 1-62 coiiiiiiiiiiiciite. On lollow-up examinations. language competences had re- aplie;ircd ;iIiiio\t intiict. hut :I massive auditory agnosia for non-verbal sounds Neurocase Reference Number: %:I\ ohscrved. From closc inpection of lesion site. as determined with brain PSX3 rwiiiancc imaging, and from ;I study of auditory evoked potentials, it is cunclutlcd !hiit hilateral insular damage was crucial to both expressive and Primary diagnosis of interest rcccpti\c c(rmponcnts of the syndrome. The role of the insula in verbal and Pure word deafness non-verbal c.oiiimunicatioii is discussed in the light of anatomical descriptions (11the piltlcrn of connectivity of !he insular cortex. Author's designation of case Journal Henry C Ncur(rp\ycli~rlo~i;i19%; 3.3: 327-39 Key theoretical issue Neurocase Reference Number: Pure word deafness and its relationship to asymholia for pain 1'5x7 Key w(J~~,Y:pure word deafness Primary diagnosis of interest Scan, EEG and related measures Muli\ni. ;iuditory agnosia Not mentioned Author's designation of case Standardized assessment 'fM Not mentioned Key theoretical issue Other assessment 0 This patient's gcneraliied auditory agnosin developed into an agnosia for Audiometric screening nori-vcrh;il sounds only. The insula may have a critical role in attentional a\pcct\ of coiiiiiiuiiiciiti(rn Lesion location Not mentioned Kcv II,(I~A:speech: niutism: insular cortex; auditory agnosia Scan, EEG and related measures Lesion type Head injury ('T. MRI Standardized assessment Language English HIIAE, Rcy-Osterricth Complcx Figure. Trail Making Test. Stroop Test, Wi\con\in C;ird Sorting Test. Wcchaler Memory Scale. Rey's 15 word auditory I c;i rni ng. WA I S- K

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Other assessment Atiilioiiictric \crcening I,esion location 13ilatcral insul;ir region Lesion type I\ch;icniic inlhrction Language l%gIi\h Previous cases: Auditory agnosia 39 1

Cortical auditory disorder caused by Defective auditory recognition after bilateral temporal infarctions small haemorrhage in the inferior co 11 i c u1 i K. Ishii, Y. Ueda, N. Ohkoshi, H. Mizusawa and S. Shoji K. Johkura, S. Matsumoto, 0. Hasegawa and Abstract Y. Kuroiwa The authors present a 55-year-old man who suddenly became afflicted with a bilateral auditory disturbance. Auditory acuity was preserved relatively well. Abstract Brainstem auditory evoked potentials (BAEPs) were normal. Middle latency We report the rase of a inale patient with a traumatic small haemorrhage auditory evoked potentials (MLAEPs) revealed the total absence of all waves. partially involving the bilateral inferior colliculi without evidence of a temporal Brain computed tomography (CT) showed infarctions of the temporal lobes lobe lesion. He was unable to comprehend spoken words although he had bilaterally, involving the superior temporal and transverse temporal gyri. The intact speech production, reading and writing abilities. Comprehension of recognition of‘ verbal and non-verbal sounds was also impaired. Brain CT. environmental sounds was also affected. Among the receptive musical abilities. MLAEPs and auditory recognition tests were useful in diagnosing the cortical discrimination of intensity. tone and rhythm were preserved, while recognition auditory disorder. of melody was impaired. Audiometry showed normal thresholds for pure tone. Waves I-IV of brainstem auditory evoked potentials were elicited normally. Journal whereas the wave V was elicited with reduced amplitude and prolonged Internal Medicine 1995; 34: 801-5 latencies on both sides. The main component of middle latency auditory evoked potentials, which is evoked over both hemispheres by monaural Neurocase Reference Number: stimulation to either side in nornial subjects. was elicited only over the P584 hemisphere contralateral to the ear receiving stiinulation. The patient’s auditory tindings were similar to those usually found in generalized auditory agnosia. Primary diagnosis of interest Auditory agnosia is usually considered as a sign of a hitemporal cortical or Cortical auditory disorder subcortical disorder, but, in the patient. a brainstem disorder caused a disturbance of‘ auditory recognition similar to auditory agnosia due to a Author’s designation of case hiteinporal lesion. The patient’s auditory findings may belong to the category Not mentioned ofa brainstem auditory-processing disorder brought on by a small haemorrhage in the inferior colliculi. In addition, thc impairment in the patient implies that. Key theoretical issue in the neural processing of musical parameters. the decoding of intensity. tone oniplished at the level of inferior colliculus. whereas further 0 Cortical auditory agnosia is a distinct syndrome from auditory agnosia and pure word deafness. Recognition of verbal and non-verbal stimuli are cortical processing is necessary for the appropriate recognition of melody. similarly impaired Journal Key wrtls: auditory agnosia; deafness; evoked potentials; temporal lobe Journal of the Neurological Sciences 1998; 161: 914 Scan, EEG and related measures Neurocase Reference Number: CT, ECG P585 Standardized assessment Primary diagnosis of interest WAIS-R. WMS-R. Token Test, Western Aphasia Battery. audiometric screening Auditory agnosia Other assessment Author’s designation of case Brainstem auditory evoked responses, auditory recognition tests Not mentioned Lesion location Key theoretical issue CT bilateral temporal lobe (Heschl’s and superior temporal gyri) Case of generalized auditory agnosia resulting from brainstem pathology Lesion type uditory agnosia; inferior colliculus; word deafness; amusia Infarct Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Scan, EEG and related measures Language EEC. CT. MRI, HMPAO-SPECT English Standardized assessment WAIS-R, Western Aphasia Battery. Standard Language Test of Aphasia Other assessment Audionietric screening Lesion location Bilateral inferior rolliculi Lesion type Haemafonia Language English 391 Previous cases: Auditory agnosia

Verbal auditory agnosia with focal EEG Subcortical auditory agnosia abnormality: an unusual case of a child presenting to an ENT surgeon with S. Kazui, H. Naritomi, T. Sawada, N. Inoue and ‘deafness’ J. I. Okuda Abstract A case of generalized auditory agnosia without aphasia sccond:iry 111 U. Kale, M. El-Naggar and M. Hawthorne cardiogenic cerebral embolism is reported. The infarct5 in this patient u‘crc Abstract localized within the biteniporal subcortices as confirmed by coniputeriid axial tomography and magnetic resonance imaging. The tintlings suggested Vcrhiil auditory agnosia implies the failure to recogniLe sounds in a patient that interruption of hoth auditory radiations by bilateral suhcortical Ichion\ who is ~icverthelessnot deaf. The authors present a child referred to the ENT owpatient department for a suspected hearing loss investigation. At one point may play an important role in the occurrence of ‘cerebral auditory disorder\’ \he had grommets inserted on the basis of a flat tynipanogram. hut with no Journal el lect. All hearing tcsts. including electrocochleography and distraction tests. revealed nornial hearing thresholds. At the age of 3 years and 3 months, and Brain and Language 1990: 38: 176-87 on direct questioning of the parents, it was discovered that the child responded Neurocase Reference Number: reni;irkahly well to music. Paediatric neurologists made the diagnosis of verbal auditory iignosia. The condition is very similar to another acquired language P587 disorder called Landau-Kleffiier syndrome. It is unusual for such a case to Primary diagnosis of interest present priinarily to an ENT surgeon. Auditory agnosia Journal Author’s designation of case The Journnl (if Laryngology and Otology 1995; 109: 431-2 Not mentioned Neurocase Reference Number: Key theoretical issue PSXh Case of generalizcd auditory agnosia without ophxsia duc to sni;ill Primary diagnosis of interest subcortical lesions Childhood verbal auditory agiiosia Key words: auditory ngnosia; temporal lobe; subcortex Author’s designation of case Scan, EEG and related measures Not nicntioned EEG. CT. MRI, cerebral angiography. clectrocochleo~raphy Key theoretical issue Standardized assessment 0 Vcrhal auditory ;igno\ia in ii case of possible Landau-Kleffner syndrome Standard language test of aphasia Kcv 11 on/.\:agno\iii; verhal: auditory: Landau-Kleffner syndrome Other assessment Scan, EEG and related measures Right-left discrimination, tinger identi tication. colour reciqiition. \ iwil object recognition. unilateral spatial neglect. constructional capacity. rcniote CT. EEG and recent memory. environmcntal sounds. verbid identitication of melodie\. Standardized assessment loudness discrimination. frequency discrimination. click fusion test\., clich detection threshold, discrimination lists with varying interwls. discriniin;itiim Not mentioned tests with varying number of clicka. vowel and phonemic discrimination -- Other assessment natural speech Pui-c tone audiometry Lesion location Lesion location CT right temporal and left parietal subcortex 0 MRI: additionally left temporal stem 0 No ahnornialities noted Lesion type Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Lesion type No ahnornialities noted Infarction Language Language English Engl i\h Previous cases: Auditory agnosia 393

The problem of agnosia in the light of a Word-meaning deafness: a case of pure word deafness phonological-semantic dissociation

R. Klein and J. Harper S. E. Kohn and R. B. Friedman Abstract Abstract A 45-year old man showed, after probable v ular lesion of the brain, a Word-meaning deafness represents an auditory comprehension disturbance marked aphaxic disorder and absence of response to auditory stimuli. After a that is due to a dissociation between rate phonological and semantic ahon period lasting only a few days, the aphasic disturbance had almost information. The patient is unable to u tand a spohen word that he can completely disappeared; the picture which followed was that of a pure word repeat and can understand when read. Two cases of word-meaning deafness deafness associated with disturbance in rhythm, musical expression and some are presented to demonstrate that this disorder can be due either to a breakdown disturbance in writing. On audiometric testing, carried out 6 weeks after the prior to the accessing of stored phonological representations (’pre-access’) or start of the illness, the air and bone conduction of the left ear was normal to a breakdown following the accessing of stored phonological representations and there was a moderate mixed deafness in the right ear. At this period. the (’post-access’ 1. word deafness was still almost complete. The verbal sounds were experienced as an undifferentiated continuous humming noise without any rhythm. Journal Subsequently a gradual limited improvement in the word sound perception Cognitive Neuropaychology 1986: 3: 29 1-308 occurred, but there was still a considerable defect after 18 months of observation. The sequence in which perception of various qualities of speech Neurocase Reference Number: recovered was followed up. and a number of phenomena associated with the PS89 word deafness were studied. Some of these phenomena considered to be significant for the mechanism of the disorder have been discussed in detail. Primary diagnosis of interest Word-meaning deafness Journal Journal of Mental Science 1956: 102: I 12-20 Author’s designation of case HN Neurocase Reference Number: LL P588 Key theoretical issue Primary diagnosis of interest Word-meaning deafness represents either a deficit Pure word deafness in the phonological lexicon, or a disconnection between the phonological lexicon and semantic system Author’s designation of case RC iuditory agnoaia: word-meaning deafness: phonological lexicon: xeiiiantic memory Key theoretical issue 0 Nature of pure word deafness Scan, EEG and related measures CT Key wonk pure word deafness: agnosia Standardized assessment Scan, EEG and related measures Boston Diagnostic Aphasia Examination Not mentioned Other assessment Standardized assessment Testa of repetition. word-picture matching, word-body part matching. Terman-Merrill Vocabulary Tests generation of definitions Other assessment Lesion location Audiometric screening, repetition tasks HN: Wernicke’s area LL: Wernicke’s area extending to body of left lateral ventricle Lesion location 0 Lesion type

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Not mentioned Not specified Lesion type CVA assumed Language English Language English 394 Previous cases: Auditory agnosia

~ ~~ ~ Auditory agnosia with relative sparing of Self-monitoring behaviour in a case of speech perception severe auditory agnosia with aphasia

J. Lambert, F. Eustache, B. Lechevalier, R. C. Marshall, B. 2. Rappaport and Y. Kossa and F. Viader L. Garcia-Bunuel Abstract Abstract The iiutliors report a casc of auditory agnosia in which the initial clinical This case report describes an unusual combination of speecli and language picturc hegan with generalired auditory agnosia for verbal and non-verbal deficits secondary to bilateral infarctions in a 62-year-old woman. The paticnt ~otiiid~.hut riipiilly changed to a selective auditory agnosia confined to the was administered an extensive series of speech. language. and :iudiologic te\t\ perception ol nori-vcrhal \ounds. CT scanning and MRI did not demonstrate and was found to exhibit a fluent aphasia in which reading arid writing werc cortical or subcortical damage. except for bilateral ventricular enlargement. extremely well preserved in comparison to auditory coinprehmsion and oriil The patient was suhniittcd to audiological investigations including physical expression. and a severe auditory agnosia. In spite of her auditory processing :uid p\ychoacou\tic studies. Delicits were revealed during the decay and deficits. the patient exhibited unexpected sell-monitoring ahility and the Iotidness discrimination test. hut 1111 temporal auditory acuity deficit was capacity to form acoustic images on visual tasks. The iiiaiiiic'r in which \he ohscrvcd. The re\ulrs ot these studics are discussed in relation to the clinical corrected and attempted to correct her phonemic errors. while ignoring sciiiaiitic pictui-e. Alw. the dissociation hetween verbal perception and non-verbal errors, suggests that different mechanisms may underlie tht. monitoring (11' perception 15 di\cussed. these errors. Journal Journal Cortc~I?)X'J: 2.5: 7 ILX2 Brain and Language 1985; 24: 297-313 Neurocase Reference Number: Neurocase Reference Number: I'YJO P.59 I Primary diagnosis of interest Primary diagnosis of interest Auditory ;ignosia secondary to head injury Auditory agnosia Author's designation of case Author's designation of case ID Not mentioned Key theoretical issue Key theoretical issue 0 Patient with generalized auditory agnosia developing to selective deficit Patient with generalized auditory agnosia and fluent aphasia who, despite tor noii-verbal sounds (although sonie evidence of difficulty with phonemic the auditory agnosia. showed awareness of her specch production error9 identilicationI Key \vort/.s: auditory agnosia: Huent aphasia A';c,\ 11 OU/.\: auditory agnosia: speech Scan, EEG and related measures Scan, EEG and related measures EEG. CT CT. MKI, EEG Standardized assessment Standardized assessment Word Intelligibility Picture Identification Test, Peabody Picture Vocabular) Key Figure copy. French Aphasia Battery Test. Lusher's Test Test, Token Test, Auditory Comprehension Test for Sentences. Porch Index for Communication Ability, Boston Diagnostic Aphasia Examination. Other assessment Minnesota Test for Differential Diagnosis of Aphasia, WAIS. Raven's Coloured Audion~ctricscrecning. sound matching, music reception. sound discrimination Progressive Matrices, Rey-Osterrieth Battery tests, voice. rhythm and language perception, click counting, verbal coiiiprchcnsion wt. written word matching test Other assessment Audiometric testing. reading comprehension battery for aphasii. multilingual Lesion location aphasia test

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 0 (.T hilnteral ccrehral ventricles Lesion location Lesion type 0 CT bilateral middle and superior temporal gyri. extending on left tci inferior Ii;lelllllrrllilgc parietal lobe and insula Language Lesion type Ellgll\ll Hypodensity Language English Previous cases: Auditory agnosia 395

Cortical auditory disorders: clinical and Word deafness mistaken for Alzheimer's psychoacoustic features disease: differential characteristics

M. F. Mendez and G. R. Geehan M. F. Mendez and S. Rosenberg Abstract Abstract The symptoms of two patients with bilateral cortical auditory lesions evolved The inability to understand spoken words may result from speech from cortical deafness to other auditory syndromes: generalized auditory comprehension disorders such as Wernicke's aphasia, hearing loss. and pure agnosia, amusia and/or pure word deafness, and a residual impairment of word deafness (PWD). However, clinicians may still mistake PWD, a temporal sequencing. On investigation, both had dysacusis. absent middle rare cortical disorder with an isolated inability to understand speech, with latency evoked responses, acoustic errors in sound recognition and matching, Alzheimer's disease (AD). The correct differentiation of PWD from AD has inconsistent auditory behaviours. and similarly disturbed psychoacoustic implications for the prognosis and treatment of these patients. This report discrimination tasks. These findings indicate that the different clinical evaluated a patient with word deafness who was misdiagnosed as having AD. syndromes caused by cortical auditory lesions form a spectrum of related The misdiagnosis caused her much distress as well as a delay in initiating auditory processing disorders. Differences between syndromes may depend proper management. Additionally, this report characterizes the clinical features on the degree of involvement of a primary cortical processing system. the of PWD and compares them to the language changes of AD and of other more diffuse accessory system, and possibly the efferent auditory system. disturbances with which PWD is often confused. Journal Journal Journal of Neurology. Neurosurgery and Psychiatry 1988; 5 I : 1-9 Journal of the American Geriatrics Society 1991; 39: 209-11 Neurocase Reference Number: Neurocase Reference Number: P592 P.593 Primary diagnosis of interest Primary diagnosis of interest Generalized auditory agnosia Pure word deafness Author's designation of case Author's designation of case Case 1 and Case 2 Not mentioned Key theoretical issue Key theoretical issue 0 Psychoacoustic features of cortical auditory disorders Differential diagnosis of pure word deafness from Alzheimer's disease

Key words: auditory agnosia; fluent aphasia Kev words: pure word deafness; Alzheimer's disease Scan, EEG and related measures Scan, EEG and related measures CT, echocardiography, brainstem auditory evoked response CT Other assessment Standardized assessment Pure tone audiometry Not mentioned Lesion location Other assessment 0 Case I : bilateral haemorrhagic temporal infarcts bilaterally involving the Audiometric screening, aphasia screening superior temporal gyri and surrounding hypodensity from temporal tips to parietal lobes Lesion location 0 Case 2: right frontoparietotemporal infarct and left parietotemporal lesion 0 Hypodensity deep in the medial aspect of the left temporal lobe Lesion type Lesion type Embolism CVA assumed Language Language Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 English English 396 Previous cases: Auditory agnosia

Analysis of word comprehension in a Deep dysphasia: an analogue of deep case of pure word deafness dyslexia in the auditory modality

M. N. Metz-Lutz and E. Dahl F. Michel and E. Andreewsky Abstract Abstract A case of pure word deafness due to a left temporal infarct is reported. The A right-handed patient, with two left hemisphere lesions, a .;mall one in the results of dichotic tests suggest that auditory verbal material may be processed prefrontal lobe and a larger one in the temporal. presents an unusual syndrome: in the right hemisphere. The inability to repeat nonsense words, the frequent a massive deficit for oral language (expression and comprehenion) contrasting semantic paraphasias in real-word repetition tasks, and the capacity to give a with a fairly good preservation of written language (expression iind partial account of the meaning of a word that the patient cannot repeat show comprehension). The processing of isolated words and sentences ha\ hew that despite the impairment of the phonological analysis, lexical semantic extensively tested with repetition and dictation tasks. The patient perlorin\ processing is possible. An attempt is made to demonstrate that the patient rather well with nouns. verbs. and adjectives. poorly with adverbs and I’unciion resorts to this semantic processing and that this reflects the linguistic words, and completely fails with nonsense words. A remarkable feature ol competence of the right hemisphere. his repetition is the frequency of semantic paraphasias. Thus. this paticill exhibits a behaviour rather similar to deep dyslexia, hence the possihle label Journal ‘deep dysphasia’. The paper presents a ‘prc-understanding’ hypothehi\ 10 Brain and Language 1084; 23: 13-25 account for such behaviours. Neurocase Reference Number: Journal PSY-1 Brain and Language 1983; 18: 212-23 Primary diagnosis of interest Neurocase Reference Number: Pure word deafness and deep dysphasia P595 Author’s designation of case Primary diagnosis of interest GI< Deep dysphasia Key theoretical issue Author’s designation of case Involvement of right hemisphere in repetition Not stated

Ke! icvwrls: pure word deafness; deep dysphasia Key theoretical issue Description of deep dysphasia as a syndrome associated with scinaniic Scan, EEG and related measures errors during repetition of spoken words and writing to dictation CT. EEC, brain stem evoked responses Key words: deep dysphasia; temporal lobe; word deafne Standardized assessment BDAE Scan, EEG and related measures CT Other assessment Audiometric testing, non-verhal and verbal auditory, repetition of words and Standardized assessment non-words. dichotic listening studies, auditory comprehension None specified Lesion location Other assessment 0 Left temporal hypodensity spreading from cortex to the deep structures lQ, memory, oral comprehension. repetition. dichotic liaiening. writicn comprehension, tachistoscopic reading Lesion type CVA Lesion location Left anterior frontal lobe, left temporal lobe Language Lesion type

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 English Possible infarct Language English Previous cases: Auditory agnosia 397

Auditory agnosia. Analysis of a case with Clinical and anatomic findings in a case bilateral subcortical lesions of auditory agnosia

N. Motomura, A. Yamadori, E. Mori and D. R. Oppenheimer and F. Newcombe F. Tamaru Abstract A case ih reported of severe agnosia for verbal and non-verbal sounds in a Abstract setting of well-preserved intelligence and transient dysphasic symptoms. The A case of typical auditory agnosia is reported. It is unique in that the clinical lesions responsible for this disorder were bilateral cerebral infarcts, one of picture began with generalized auditory agnosia for verbal and non-verbal which had virtually destroyed the areas of Wernicke and Broca. The relation sounds and changed. over a period of two months, to a restricted auditory between lesions and disabilities is discussed and comparisons made with other agnosia, contined to the recognition of non-verbal sounds. The lesions, published cases. confirmed by CT scanning and MRI, were subcortical bilaterally, without evidence of cortical damage. and have not previously been described. The Journal results of audiological studies on temporal discrimination were similar to Archives of Neurology 1978; 35: 712-9 those described for bilateral temporal lobe lesions. The relationships between click intervals and the threshold to detect sound, and between numbers of Neurocase Reference Number: clicks and the threshold, were also examined. The authors observed a tendency PS97 for the threshold to decline as the number of clicks was increased or the interval between two clicks was shortened. These phenomena are similar lo Primary diagnosis of interest temporal summation in cellular neurophysiology and the authors speculate Auditory agnosia that this phenomenon is an important factor in the auditory disturbances shown by the present case. Author’s designation of case Journal Not mentioned Brain 1986; 109: 379-91 Published erratum appears in Brain 1986; 109: 1322 Key theoretical issue Neurocase Reference Number: 0 Discussion of pathological findings in a patient with auditory agnosia PSY6 Kq wordy: auditory agnosia; word deafness Primary diagnosis of interest Scan, EEG and related measures Auditory agnosia None mentioned Author’s designation of case Standardized assessment Not mentioned None mentioned Key theoretical issue Other assessment 0 Apparently earliest report of auditory agnosia changing from a generalized Audiometric screening, block design, cube counting. non-verbal span, word to a selective non-verbal deficit. associated with subconical lesions repetition. sound tests Key work auditory agnosia; subcortical lesion Lesion location Left inferior frontal region and inferior parietal lobule. right superior Scan, EEG and related measures temporal gyrus CT, MRI. EEG Lesion type Standardized assessment Infarct WAIS, Token Test Language Other assessment English Audiometric testing. environmental sounds, rhythm and melody recognition tests, vowel identification, phonemic discrimination, temporal discrimination. Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 music testing. dichotic listening test, click fusion test, loudness discrimination, click detection threshold Lesion location CT left posterior thalamus, right posterior region Lesion type Left: hyperdensity Right: hypodensity Language English 398 Previous cases: Auditory agnosia

Slowly progressive pure word deafness Right unilateral auditory agnosia following left lenticular haemorrhage M. Otsuki, Y. Soma, M. Sato, A. Homma and S. Tsuji F. Pasquier, D. Leys, M. Steinling, J. I). Guieu, Abstract H. Petit and J. Cambier Among the reports of primary progressive aphasia, there are few about patients who exhibited progressive pure word deafness with detailed auditory and Abstract radiological examination as well as neuropsychological assessment. The A 33-year-old patient who had had left lenticular haeniorrhage prcsentcd with authors describe a 67-year-old right-handed man who exhibited slowly an inability to understand oral language. with the right ear. and. in a le\\ progressive pure word deafness over a period of 9 years without exhibiting dramatic way, non-verbal sounds. This unilateral auditory agnosia was tirst any other cognitive or mental deterioration. Magnetic resonance imaging of associated with a right motor under-utilization and right motor. sensitive. his brain revealed generalized cortical atrophy, particularly in the left superior visual and auditive extinctions. Speech discrimination scores were IOO%' with temporal region. Auditory examination revealed severe disability in the left ear and IS% with the right ear, even less in dichotic conditiona. Tonal discriminating each syllable or mora of Japanese words, despite adequate audiogram. as well as early and late components of the iiuditory evohcd auditory acuity. He also showed impairment in temporal auditory discrimination potentials were normal. Cerebral regional perfusion and irietaboliani wcrc impaired over the left parietotemporal area. There was severc hypoactivatioii as\esscd hy the click fusion test and the click counting test. His ability to di\criminate meaningful environmental sounds was mildly impaired. This of the left hemisphere with right monaural verbal stimulation!;. Rehabilitation paper discusses the pathophysiology of slowly progressive pure word deafness consisting of non-specific attention tasks and repetitions of words reaching only the right ear was undertaken months after the stroke. The oriil over ii period of many years which was not complicated by other language IS or cognitive dysfunctions. language comprehension improved, as did the left hemisphert the extinction phenomena disappeared. except for the auditory one. The Journal unilaterality of the auditory agnosia could be due, in parl. to a pecul~ar European Neurology 1998; 39: 13.540 physiological processing in this patient. such as poor performnce of his right ipsilateral auditory pathway which could he improved with practice. A striaral Neurocase Reference Number: lesion could induce a spatial hemi-inattention as reflected by the niultiniodal PS98 extinction in this case. Besides, a lack of selective activation for verhal stimulation of the left hemisphere is suggested. Primary diagnosis of interest Progressivc pure word deafness Journal Revue Neurologique (Paris) 1991; 147: 129-37 Author's designation of case Not mentioned Neurocase Reference Number: P599 Key theoretical issue Primary diagnosis of interest 0 Progressive form of piire word deafness Unilateral auditory agnosia KC,\WO,Z/.\: auditory agnosia; primary progressive aphasia; pure word deafness Author's designation of case Scan, EEG and related measures 85068 I MRI. I-IMP SPECT. brain stem evoked potentials Key theoretical issue Standardized assessment 0 Auditory agnosia for right ear only Western Aphasia Battery (Japanese version), Token Test, WAIS-R, Raven Coloured Progressive Matrices. Benton Visual Memory Scale K~vwords: unilateral auditory agnosia Other assessment Scan, EEG and related measures Click fuaion and click counring test. environmental sounds, pure tone MRI. CT. SPECT, auditory evoked potentials audiomet ry Other assessment Lesion location Audiometric screening, speech discrimination Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Generali/ed atrophy inost striking in the left superior temporal gyrus Lesion location Lesion type Left posterior putamen and posterior and inferior white matter Progressive atrophy Lesion type Language Haemorrhage English Language French Previous cases: Auditory agnosia 399

Correlation between EEG and auditory Functional dissociations following perceptual measures in auditory agnosia bilateral lesions of auditory cortex

P. S. Pearce and H. Darwish I. Peretz, R. Kolinsky, M. Tramo, Abstract R. Labrecque, C. Hublet, G. Demeurisse and The authors describe a child who acquired a gradual sustained speech deficit S. Belleville for which no specific aetiology was found, and who had an associated epileptogenic abnormality on EEG. Assessment of auditory perceptual skills Abstract and receptive language confirmed that gradual parallel improvement occurred Two cases of patients with bilateral lesions of the superior temporal cortex with the EEG after therapeutic anticonvulsant blood levels were obtained. are described showing a number of dissociations in the auditory domain. In Prospective trials of anticonvulsant drugs in conjunction with serial measures both patients, perception of speech and environmental sounds was normal of central auditory abilities are necessary to establish their value in the while perception of tunes, prosody and voice was affected. Also, both patients management of the linguistic deficit in children with auditory agnosia. showed impaired melodic, but not rhythmic processing. It is suggested that melody processing is mediated by specialized cortical subsystems instead of Journal a more general purpose auditory architecture. Brain and Language 1984; 22: 41-8 Journal Neurocase Reference Number: Brain 1994; 117: 1283-301 PhOO Neurocase Reference Number: Primary diagnosis of interest P60 I Auditory agnosia Primary diagnosis of interest Author’s designation of case Amusia, auditory agnosia JA Author’s designation of case Key theoretical issue Not mentioned Auditory agnosia associated with epileptogenic abnormality and recovery following anticonvulsant medication Key theoretical issue Melody processing could be mediated by specialized cortical systems Kev words: auditory agnosia; EEG abnormality; anticonvulsant medication instead of a more general-purpose architecture

Scan, EEG and related measures Key words: auditory agnosia: amusia; auditory cortex EEG, CT Scan, EEG and related measures Standardized assessment CT scans Peabody Picture Vocabulary Test, Reynell Verbal Comprehension Scale, pre- school language scale, Leiter International Performance Scale, Wechsler Standardized assessment Intelligence Scale for Children-Revised Boston Diagnostic Aphasia Examination, Wechsler adult intelligence scale, Wechsler memory scale, Token test, Warrington recognition memory test Other assessment Discrimination of speech sounds, discrimination of environmental sounds, Other assessment speech discrimination Language examination, recall and recognition of familiar melodies, lyric recognition, discrimination and recognition of unfamiliar tunes, recognition Lesion location of environmental sounds and musical instruments, prosody recognition, voice CT - normal recognition and discrimination, singing and related expressive behaviour Lesion type Lesion location CT - normal 0 Rostra1 auditory association cortex (both cases) Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Language Lesion type English Case 1 : ischaemic infarction Case 2: aneurysmal subarachnoid haemorrhage Language English 400 Previous cases: Auditory agnosia

~~~~~ ~ A case of auditory agnosia Pure word deafness and unilateral right temporo-parietal lesion: a case report M. Reinhold Abstract M. Roberts, P. Sandercock and E. Ghadiali A patient is described who suffered from a defective understanding of sounds including speech and music, although her hearing was not impaired. She had Abstract also Io)rt the understanding of rhythm. Her auditory imagery was very The authors discuss a patient with an agnosia predominantly fiir spoken words defective. She was unable to think in terms of abstractions. Many symbols, who had il unilateral right hemisphere temporo-parietal lesion demonstrated such as those of arithmetic and grammar, were without meaning for her. She by computed tomography. showed therefore a degree of acalculia. Her spelling was moderately defective. 21s was her grammar in written (but not in spoken) language. She was unable Journal to perlorm two tasks simultaneously. She had, for a time. at the onset of her Journal of Neurology, Neurosurgery and Psychiatry 1987; SO. 1708-9 illness. suffered from auditory hallucinations. Expressive speech was very little disturbed. Reading was not impaired as regards the motor aspect, Neurocase Reference Number: although comprehension of content, when dealing with abstract ideas. was P603 defective. Writing was only slightly impaired - again not in the least as regards the motor aspect. hut rather as regards punctuation, grammar and Primary diagnosis of interest spelling. The patient showed no apraxia, no astereognosis. and no disorientation Pure word deafness in space or time. She had little or no insight into her disabilities. She unconsciously used tactics of distraction to hide them. She preserved, in spite Author’s designation of case Not mentioned of them. ii lively interest in current events. music. clothes. pen friends, embroidery. photography. reading and charitable works. Key theoretical issue Journal 0 Pure word deafness in a right-handed patient associated with ;I riphl Brain 1950; 7.3: 203-23 temporo-parietal lesion Neurocase Reference Number: Kq words: pure word deafness; lesion localization; croshed anhaski PhOZ Scan, EEG and related measures Primary diagnosis of interest CT Auditory agnosia Standardized assessment Author’s designation of case WAIS, Handedness Inventory Not mentioned Lesion location Key theoretical issue Right parietal lesion extending inferiorly into the posterior temporal lobe 0 Early description of generalized auditory agnosia without aphasia Lesion type Infarct Kr? itvwd\: auditory agnosia; aphasia; amusia Scan, EEG and related measures Language None mentioned English Standardized assessment None mentioned Other assessment Sound interpretation. auditory memory, insight, spontaneous speech, naming of objects and colours. reading, writing, calculation. visual memory, orientation in space and time, general knowledge, memory, praxis, conceptual thinking,

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 auditory imagery and hallucinations Lesion location 0 Not stated Lesion type Not statcd Language English Previous cases: Auditory agnosia 401

Clinical and audiological findings in a An analysis of speech perception in word case of auditory agnosia deafness

G. Rosati, P. De Bastiani, E. Paolino, S. Prosser, E. M. Saffran, 0. S. Marin and E. Arslan and M. Artioli G. H. Yeni-Komshian Abstract Abstract A case is reported of severe agnosia for verbal and non-verbal sounds without A patient with a rather pure word deafness showed extreme suppression of associated aphasic disorder. A CT scan revealed bilateral, temporal lobe right ear signals under dichotic conditions, suggesting that speech signals lesions from two ischaemic accidents that had occurred 9 months apart. The were being processed in the right hemisphere. Systematic errors in the search for subtle deficits in the patient showed normal sensitivity to changes identification and discrimination of natural and synthetic stop consonants in the intensity and frequency of simple sounds; in contrast, his ability to further indicated that speech sounds were not being processed in the normal discriminate sound duration and musical note sequences was severely impaired. manner. Auditory comprehension improved considerably however, when the The simultaneous recording of the whole auditory-evoked ksponse pattern range of speech stimuli was limited by contextual constraints. Possible revealed no abnormality in the early components. which reflect the activation implications for the mechanism of word deafness are discussed. of the auditory nuclei and pathways of the brain stem. However. the middle and late components were delayed and slowed. These results and others in Journal the literature suggest that the neocortex in man, as in other mammals, plays Brain and Language 1976; 3: 209-28 an essential role in the temporal aspects of hearing. Also, the two main ingredients commonly recognized in auditory agnosia, i.e. word deafness and Neurocase Reference Number: the inability to interpret non-verbal sounds. are caused by the disruption of P605 elementary, bilaterally represented cortical functions which start the processing of every kind of auditory information. Primary diagnosis of interest Pure word deafness Journal Joumal of Neurology 1982; 227: 21-7 Author’s designation of case Not mentioned Neurocase Reference Number: P604 Key theoretical issue 0 Formal analysis of speech perception in pure word deafness Primary diagnosis of interest Auditory agnosia Kev words: auditory agnosia: pure word deafness; speech perception Author’s designation of case Scan, EEG and related measures MC EEG. carotid angiogram Key theoretical issue Standardized assessment 0 Bilateral cortical mechanisms may be involved early in the perception of Seashore Measure of Musical Talents, Dichotic Names Test verbal and non-verbal auditory stimuli Other assessment Kev brordy: auditory agnosia; word deafness; auditory-evoked potentials; Audiometric screening, speech sound discrimination and comprehension. bitemporal lesions repetition tasks Scan, EEG and related measures Lesion location CT. EEG 0 Not mentioned Standardized assessment Lesion type Token Test. WAIS. WMS Not mentioned Other assessment Language Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Audiometric screening. language test, test of non-verbal sounds English Lesion location 0 Bilateral temporal lobe, left more than right Lesion type Ischaemia Language English 402 Previous cases: Auditory agnosia

Speech discrimination and lip reading in Auditory agnosia without aphasia patients with word deafness or auditory agnosia 0. Spreen, A. L. Benton and R. W. Fincham Abstract A 65-year-old patient. after a cerebrovascular accident. showed an inability M. Shindo, K. Kaga and Y. Tanaka to understand the meaning of common sounds. but retained the full Abstract understanding of language. No other agnostic defects were found. Repeated The purpose of this study was to assess the ability of four patients with word examinations over a period of eighteen months appear to confinn the finding deafness or auditory agnosia to discriminate speech by reading lips. The of an isolated sound agnosia. Autopsy study showed a large defect involving patients were studied using nonsense monosyllables to tests for speech the right frontal, temporal and parietal areas as well as the insula. No damage discrimination. a lip reading test, the Token Test for auditory comprehension. was demonstrated in the left hemisphere or the corpus callowin. and the Aphasia test. Our results show that patients with word deafness or auditory agnosia without aphasia can improve speech comprehension by Journal reading lips in combination with listening, as compared with lip reading or Archives of Neurology 1965; 13: 84-92 listening alone. In conclusion. lip reading was shown to be useful for speech comprehension among these patients. Neurocase Reference Number: P607 Journal Primary diagnosis of interest Brain and Language 1991: 40: 153-61 Auditory agnosia without aphasia Neurocase Reference Number: Author's designation of case PhOh Not mentioned Primary diagnosis of interest Pure word deafness Key theoretical issue Auditory agnosia without aphasia may result from damage to right Author's designation of case hemisphere structures only Case I, Case 2. Case 3. Case 4 Kev words: auditory agnosia: aphasia; right hemisphere Key theoretical issue Scan, EEG and related measures 0 Influence of lip reading on speech comprehension in pure word deafness EEG. ECG Kr! ttwrls: pure word deafness; auditory agnosia; lip reading Standardized assessment Scan, EEG and related measures WAIS CT. auditory brain stem responses Other assessment Standardized assessment Audiometric screening. right-left orientation. finger IocaliLation, visuol attention tests, short-term memory, speech tests, word Huency, arithmetic tests. Token Test reading and writing tasks, sound discrimination. pitch discrimination tash Other assessment Lesion location Audiometric screening, speech discrimination test for auditory perception, lip 0 Right frontal, temporal. inferior parietal regions. insula reading test. speech discrimination, lip reading ability Lesion location Lesion type Haematoma 0 Case I : hilateral auditory radiation 0 Case 2: hilateral temporal Language 0 Case 3: hilateral temporal English 0 Case 4: left temporal

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Lesion type Case I : Moyamoya disease Case 2: suharachnoidal haeniorrhage Case 3: cerebral infarction Case 4: cerebral bleeding Language English Previous cases: Auditory agnosia 403

~______~______Childhood auditory agnosia Pure word deafness due to left hemisphere damage L. K. Stein and F. K. Curry Abstract N. Takahashi, M. Kawamura, H. Shinotou, The authors present the case of a 22-year-old female unable to understand K. Hirayama, K. Kaga and M. Shindo speech. but with an apparently normal sensitivity for pure tones. At about the age of 10 hearing was much improved, and was demonstrated to he normal Abstract at the age of 20. Speech audiometry was not successful. Speech output The authors report the case of a 55-year-old right-handed man who presented resembled that of a severely deaf person. The authors diagnose an isolated with a long-lasting pure word deafness following left thalamic bleeding. There form of ‘auditory agnosia’. . The auditory deficit was specific for language, while recognition of music and environmental sounds was normal. CT, MRI and Journal PET examinations showed that the lesion was anatomically and functionally Journal of Speech and Hearing Disorders 1968; 33: 361-70 confined to the left cerebral hemisphere. mainly the white matter of the temporal and parietal lobes. Wernicke’s area was largely preserved. It is Neurocase Reference Number: proposed that pure word deafness was consequent to the isolation of Wernicke’s P608 area from incoming auditory inforniation due to the interruption both of the association libres from the right auditory area travelling across the corpus Primary diagnosis of interest callosum and of the left auditory radiations. Childhood auditory agnosia Journal Author’s designation of case Cortex 1992; 28: 29.5-303 Not mentioned Neurocase Reference Number: Key theoretical issue P609 Auditory agnosia in childhood Primary diagnosis of interest Kev words: auditory agnosia Pure word deafness Scan, EEG and related measures Author’s designation of case EEG Not mentioned Standardized assessment Key theoretical issue Randall’s Island Test, WAIS. Raven Progressive Matrices. Rorschach, Wepman Neural basis of pure word deafness Auditory Discrimination Test. IPTA Other assessment Kq W~S:pure word deafness: auditory agnosia Audiometric screening, right-left discrimination. finger identification. number Scan, EEG and related measures recognition, auditory and perceptual discrimination tests CT. MRI. PET Lesion location Standardized assessment 0 Not known WAIS. Western Aphasia Battery Lesion type Other assessment Not known Audiometric testing, speech, music and environmental sound recognition tests Language Lesion location English 0 Left thalamus. posterior internal capsule and white matter of the left temporal lobe Lesion type

Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Haematoma Language English 304 Previous cases: Auditory agnosia

~ Pure word deafness following bilateral Imageability and category-specificity lesions. A psychophysical analysis L. K. Tyler and H. E. Moss Y. Tanaka, A. Yamadori and E. Mori Abstract In this paper, the authors report the case of DrO. a patient who has heel1 Abstract described as having a selective problem understanding the meoning of abstract A 26-year-old woman suffered from pure word deafness associated with words in the auditory modality. This claim is tested by means of an on-line receptive and expressive ainusia after cerebral infarcts bilaterally in the semantic priming task. comparing the automatic activation of scniantic teniporoparietal lobes. She did not comprehend spoken words yet production information in both the auditory and visual modalities. Although Dr0 showed (11 speech. reading and writing appeared to be unaffected. A disturbance of priming for both abstract and concrete words in the visual modality, there tenipor;il resolution was found in the auditory, visual and somatosensory was only priming for concrete words in the auditory modality. Ho\cever. niod;ilitic\. The clinical defect was limited only to the auditory modality. The DrO’s reaction times and errors in the auditory priming study suggested that ;iuthors suggest that the auditory modality is more dependent upon a temporal he might have a generalized auditory processing impairment. The authurs resolution th;in other sensory modalities. tested and confirmed this hypothesis in a series of further studies. They propose an account of why a general auditory processing impirment would Journal affect abstract words more than concrete words by appealing to an auditory Brain 1087: 110: 3x1403 analogue of Plaut and Shallice’s (Cognitive Neuropsychology 1993: 10: 377- Neurocase Reference Number: 500) computational model of deep dyslexia. Ph I0 Journal Primary diagnosis of interest Cognitive Neuropsychology 1997: 14: 293-3 18 Pure word deal’neas. ainusia. auditory agnosia Neurocase Reference Number: Author’s designation of case P61 I Not mentioned Primary diagnosis of interest Key theoretical issue Word meaning deafness 0 Auditory inodality is more dependent upon a temporal resolution than other Author’s designation of case wisory modalities DrO musia: auditory agnosia: pure word deafness Key theoretical issue Does a generalized auditory processing impairnlent produce word Scan, EEG and related measures meaning deafness’? CT. MKI. EEC Standardized assessment Key rvordy: word meaning deafness: imageability: auditory inipairment Token Test. Standard Language Test of Aphasia. WAlS Scan, EEG and related measures Other assessment CT Test for memory and calculations. audiological examination, linguistic studies. Standardized assessment iests of musical capacity. neurophysiological investigation. neuroacoustic Not mentioned \tudies. discrimination tests in various sensory modalities Other assessment Lesion location Pure tone audiogram, online priming of lexical decision (visual and auditory ), Left: middle and posterior portion of the superior gyrus involving most of phoneme discrimination. repetition Hcschl’\ gyms and the geniculotemporal auditory radiation; supramarginal and angular gyri Lesion location 0 Kight: middle ;uid posterior portion of the superior temporal gyrus and in 0 Left middle cerebral infarct the parietal lohe involving the supramarginal and angular gyri; Heschl’s gyrtis and the iiuditory radiation Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Lesion type Lesion type CVA (‘crchral infarct. gencrdired convulsions Language Language English lingli\h Previous cases: Auditory agnosia 405

The syndrome of auditory agnosia. Clinical picture and morbid anatomy in A case report and an attempt at a case of ‘pure word deafness’ neuropsychological qualification G. Wohlfart, A. Lindgren and B. Jernelius G. Ulrich Abstract Pure word deafness is rare, but of great theoretical interest. The authors Abstract observed a patient in whom there was almost complete acoustic agnosia Proceeding from the neuropsychological examination of a patient with an (verbal, mubical and general) but no word blindness. paraphaaia or agraphia. exceptionally selective impairment of auditory gnosis of vascular origin, we The internal language was intact. It was possible to examine the brain make an attempt to analyse structurally the syndrome of auditory agnosia, a following the patient’s death 1; years later. This paper reports in detail the study of which has been neglected in comparison with analyses of visual clinical observations in this case and discuss these in the light of the cerebral agnosia. In our analysis we refer to 28 case reports, documented in the lesions. Similar cases have been reported earlier as ‘pure word deafness’ literature. Decisive syndromal points of view are: (a) there is an absence of although ‘isolated acoustic agnosia’ would perhaps be more descriptive significant audiological deficiencies; (b) errors of the acoustic-discriminative and adequate. type prevail in auditory-visual matching tasks; (c) the gnostic deficit is modality-specific; (d) the same items are variably reproduced on repeated Journal presentation; (e) there is marked fluctuation of performance; (0 there is Journal of Nervous and Mental Disease 1952; 116: 818-27 exceptional irreversibility of the impairment; (g) amusia is a more or less obligatory accompanying phenomenon; (h) in cases of vascular origin there Neurocase Reference Number: is always a history of repeated temporal lobe damage. this damage being P613 predominantly in the form of bilateral lesions. This analysis indicates a close structural analogy to the syndrome of perceptive visual agnosia. The authors Primary diagnosis of interest place great importance in the relationship between approaches and models of Pure word deafness cognitive psychology [Neisser (Cognitive Psychology. New York: Appleton. 1967)] which they see as important for further theoretical development in Author’s designation of case neuropsychology. Not mentioned Journal Key theoretical issue Archiv fur Psychiatrie und Nervenkrankheiten 1977; 224: 22 1-33 Clinical and post-mortem findings in a case of pure word deafness

Neurocase Reference Number: Key brords: auditory agnosia; amusia P612 Scan, EEG and related measures Primary diagnosis of interest Pneumoencephalograni. EEG. post-mortem examination Auditory agnosia Other assessment Author’s designation of case Audiometric screening. speech. music and environmental sound discrimination. Not mentioned voluntary speech. foreign language comprehension, writing and calculation Key theoretical issue tasks Metanalysis of 28 case reports of auditory agnosia Lesion location 0 Large lesion in the region of the right Sylvian fissure. smaller lesions in Key words: pure word deafness; categorical processing; acoustic-discriminative the base of the right cerebellar hemisphere and left occipital lobe errors; repeated temporal lobe impairment Lesion type Scan, EEG and related measures Embolism Not mentioned Language Standardized assessment English Not mentioned Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Other assessment Not mentioned Lesion location 0 Not mentioned Lesion type Not mentioned Language German 406 Previous cases: Auditory agnosia

Pure word deafness (acquired verbal Word deafness and Wernicke’s aphasia: auditory agnosia) in an Arabic speaking report of cases and discussion of the patient syndrome

B. A. Yaqub, G. G. Gascon, M. Al-Nosha and D. K. Ziegler H. Whitaker Abstract A case of complete auditory agnosia (‘pure word deafne3.s’) and one 01‘ Abstract Wemicke’s aphasia are reported. The difference between the two syndromes A 3X-year-old. right-handed Arabic-speaking male developed pure word is discussed. Total deafness and auditory hallucinations occurred at different dealnrs 3 days after myocardial infarction. He could recognize Arabic music times in the course of the illness of the patients with auditory agnosia. Thctr and instruments hut not words of songs: a radio broadcast from the Koran, significance is commented on. Severe hehavioural disturbance occurred in hut not the individual words; a male as opposed to female voice; Arabic and both patients. The difficulty of its differentiation from purdy psychogenic non-Arabic languages: and whether sentences were questions. exclamations. disease is indicated. and its relation to temporal lobe dysfunction is suggested. or imperatives. He discerned whether the speaker was emotionally neutral, happy. angry or sad. Contcxtual cues and reducing the rate of speaking aided Journal verbal comprehension. Pure tone threshold audiometry revealed mild bilateral Archives of Neurology and Psychiatry. Chicago 195 I ; 67: 323-3 I wnwrineural loss up to 2000 Hz and a moderate high frequency loss. Br,’.iinsteni . auditory evoked potentials were normal, cortical auditory evoked Neurocase Reference Number: potentids ahnormal. CT scan revealed bilateral infarcts subcortically just P615 pohterior to the left superior temporal gyrus and the right posterior superior and niidtcmporal regions. Nrurolinguistic tests indicated that the deficit is Primary diagnosis of interest prcphoneniic and not due to impairment of linguistic discrimination. Pure word deafness and Wernicke’s aphasia Journal Author’s designation of case Brain IOXX: I I I: 45746 Case I: CN Neurocase Reference Number: Case 2: BI Phl4 Key theoretical issue Primary diagnosis of interest Dissociation of pure word deafness from generalized aphasia Pure word deafness Kev words: pure word deafness; auditory agnosia: Wemicke’s aphasia Author’s designation of case Scan, EEG and related measures Not mentinned EEG. pneumoencephalograni Key theoretical issue Standardized assessment Clinical tindings in a case of pure word deafness Goldstein Stick Test Key wordv: pure word deafness: auditory agnosia Other assessment Scan, EEG and related measures Case 1 : spontaneous speech, reading. writing. naming. calculation. comprehension of auditory stimuli. comprehension of environmental soundh. CT. EEC. hrain stein evoked potentials, cortical auditory evoked potentials music interpretation Other assessment Case 2: spontaneous speech and behaviour. repetition. recognition of written and spoken words, writing, calculation, abstraction tests. naming Audiometric screening. verbal and non-verbal sound assessment, speech di..’scriminntion tasks. reading aloud and for comprehension, praxis. right-left Lesion location discrimination, tinger identification. naming of body parts, geometrical shapes Not known and colours. simple addition. copy figure, immediate and remote memory, click fusion and click counting. vowel identification. phonemic discrimination/ Lesion type identification Downloaded by [The University of Manchester Library] at 13:18 22 June 2012 Not known Lesion location Language Bilateral tempornl regions English Lesion type I nfarct Language Enpli\h