J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.49.5.489 on 1 May 1986. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry 1986;49:489-499 Word deafness: one hundred years later ARON S BUCHMAN,* DAVID C GARRON,t JUDITH E TROST-CARDAMONE,$ MELVIN D WICHTER,§ MICHAEL SCHWARTZ§ From the Department of Neurological Sciences* and Department of Psychology and Social Sciences,t Rush-Presbyterian-St. Lukes Medical Center, Chicago, Illinois, the Department of Communicative Disorders,t California State University, Northridge, Northridge, California, the Department of Neurology,§ Christ Hospital, Oaklawn, Illinois, USA SUMMARY Since its original description the diagnosis of word deafness has been greatly expanded. Confusion has arisen with regard to the usage of the related terms pure word deafness, auditory agnosia, and cortical deafness. Three new cases of word deafness are presented including one case with CT and necropsy correlation. These cases are compared with 34 previously reported cases of various cortical auditory disorders. Our revieW establishes that patients with word deafness who have had formal testing of linguistic and non-linguistic sound comprehension and musical abilities always demonstrated a more pervasive auditory agnosia. Despite the spectrum of auditory deficits and associated language abnormalities, patients with word deafness share common features includ- ing aetiology, pathology, clinical presentation and course. These common features justify inclusion Protected by copyright. of heterogeneous cortical auditory disorders under the rubric of word deafness. Despite some limitations the term "word deafness" should be retained for this syndrome, since inability to comprehend spoken words is the most distinctive clinical deficit. Word deafness is most frequently caused by cerebrovascular accidents of presumed cardiac embolisation, with bitemporal cortico- subcortical lesions. The sequence of cerebral injury is not predictive of resulting auditory deficits. Impairment of musical abilities parallels the severity of the auditory disorder. In 1885, Lichtheim described the syndrome of word other linguistic functions on the other hand, dis- deafness as a rare disorder characterised by defective tinguishes them from aphasic patients. In the latter comprehension, repetition, and writing to dictation.' group, the linguistic impairment typically cuts across Defective repetition differentiates this disorder from all or most modalities. In contrast non-aphasic a transcortical sensory aphasia, and preservation patients with cortical auditory disorders demonstrate of reading, writing and spontaneous speech overall preservation of non-auditory language func- differentiates it from Wernicke's aphasia.2 3 Patients tions. Table 1 summarises this discrepancy between http://jnnp.bmj.com/ are not deaf and may have normal or near normal auditory and non-auditory linguistic behaviour for pure tone audiometric findings. the three common varieties ofcortical auditory disor- Since its original description, the diagnosis of word ders and presents a conceptual framework for viewing deafness has been greatly expanded. Confusion has their clinical features. Despite the differing labels, arisen with regard to the usage of the related terms these cortical auditory disorders present similar be- pure word deafness, auditory agnosia, and cortical havioural clusters. The most divergent feature is im- deafness. Clinically there is indeed a relatively rare but paired hearing sensitivity in patients with cortical behaviourally distinct subgroup of patients whose deafness. on September 23, 2021 by guest. marked disparity between performance in speech A review of the literature of cortical auditory dis- comprehension and repetition on the one hand, and orders establishes that no patients previously reported with word deafness who have had formal testing of Address for reprint requests: Aron S Buchman, MD, Rush- linguistic and non-linguistic sound comprehension Presbyterian-St. Lukes Medical Center, Professional Building Room and musical abilities have had "pure" word deafness. 915, 1753 West Harrison Street, Chicago, Illinois 60612, USA. All patients always demonstrated a more pervasive Received 30 May 1985 and revised form I October 1985. auditory agnosia ranging from complete deafness to Accepted 16 October 1985 more subtle auditory processing disturbances. It is our 489 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.49.5.489 on 1 May 1986. Downloaded from 490 Buchman, Garron, Trost-Cardamone, Wichter, Schwartz Table 1 Classification of cortical auditory disorders* Table 3 Results from selected subsets of the Boston Diagnostic Aphasia Examination for Patient 2 Pure word Auditory Cortical deafness agnosia deafness Subtest Score Maximum possible 1. Speech score comprehension + + + Fluency 2. Speech repetition + + + Articulation rating 5 5 Phrase length 5 7 3. Non-linguistic Verbal agility 6 7 auditory comp. - + + Auditory comprehension Word discrimination 56 72 4. Hearing sensitivity - - + Commands 5 15 Complex material 0 12 5. Spontaneous speech - - - Naming Responsive naming DNT - 6. Reading Confrontation 85 105 comprehension - - Oral reading Word 30 30 7. Written language - - Sentence 10 10 Repetition CNT - *Based on Benson FD, 19722 and Oppenheimer DR and Newcombe Automatised speech F, 1978.3 Automatised sequences 8 8 Key: + = impaired;-= normal. Reading comprehension Word-picture matching 10 10 Reading sentences, paragraphs 7 10 Writing Mechanics 3 3 Table 2 Results from selected subsets of the Western Narrative writing 3 4 Aphasia Battery for Patient I Key: CNT-Could Not Test. DNT-Did Not Test. Subtest Score Maximum Protected by copyright. possible score thesis that despite the spectrum of auditory deficits and associated language abnormalities, patients with Spontaneous speech word deafness share common features including Functional content 8 10 pathology, clinical presentation and course. Fluency 10 10 aetiology, Auditory verbal comprehension These common features justify inclusion of hetero- Yes/No Questions 0, 60* 60 geneous cortical auditory disturbances under the Auditory word recognition 51 60 Sequential commands 0, 66* 80 rubric of word deafness. Repetition (Words, Phrases, Sentences) 0 100 In the present report, three patients are presented, Naming Object naming 60t 60 exemplifying the spectrum of auditory deficits and Word fluency CNT - variable language dysfunction in word deafness. Sentence completion 0, 10* 10 These patients are compared with 34 previously re- Responsive speech 0, 10* Reading ported cases of cortical auditory disorders with Reading comprehension of specific reference to auditory comprehension deficits, sentences 32 40 Reading commands 17 20 language dysfunction and abnormalities of musical 1 4- 37 Written word stimulus/Object abilities (tables 4-6). http://jnnp.bmj.com/ matching 6 6 Written word stimulus/Picture matching 6 6 Case reports Picture stimulus/Written word matching 6 6 Patient I Spoken word/Written word A 75-year-old right-handed female was admitted in Decem- matching 4 4 Letter discrimination 6 6 ber 1983, for depression, suicidal and paranoid ideation, and Spelled word recognition (oral) 0 6 aggressive behaviour during the previous six to eight months. Spelled (orally presented words) 0 6 On the day of admission she had tried to climb out of the Writing Writing on request (name, address) 6 6 window of a moving car. She was acutely psychotic and on September 23, 2021 by guest. Written output (picture description) 24 34 threatened suicide. No visual or auditory hallucinations were Writing to dictation CNT - described. Her past medical history included hypertension, Writing visually presented words 10 10 congestive heart failure, and intermittent atrial fibrillation Alphabet 12-5 12 5 Numbers 0 to 20 10 10 controlled with digoxin and diuretics. Dictated letters and numbers CNT - The family described an episode lasting a few hours in Copying of words of a sentence 10 10 1981 when she was confused, combative and reported Key: *Adjusted score based on reading the stimulus items. difficulty in hearing. In August 1982 she was hospitalised for tlncluded responses containing phonemic (literal) paraphasias. a cerebrovascular accident, described as the sudden onset of CNT-Could Not Test. tPoor patient cooperation. hearing difficulties, confusion and an inability to recognise J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.49.5.489 on 1 May 1986. Downloaded from Word deafness: one hundred years later 491 Table 4 Summary of results for current cases of word deafness I CVA R->L Fluent + + - + + + + + L, C 2 CVA R - >L Subfluent + + + ? + ? + - L, C 3 Encephalitis 2ey CVAetalceseizures imr-veLBil. FluentSubflueadnt ±mrvd+ +-mard;-oml+ ++-nufiin? in+for+ationC+ ? Key:C-contextualcuesimproved;L-lipreadinge + her daughter. She was unable to understand spoken words deficit. Speech discrimination could not be tested. There was but had no difficulty understanding written information. no history of previous psychiatric illness. The family Neurological examination in 1982 was reported to be nor- described the gradual onset of depression following her mal, except for paraphasic errors in otherwise fluent speech. stroke. An isotope scan was normal, but a CT scan of the head On examination in December 1983, the patient was awake, showed the right ventricle larger than the left and a non- alert and fully oriented. Neurological examination including enhancing area of