Speech Perceived through a Damaged Temporal Window: Lessons from Word Deafness and Aphasia

 

 Buy Article Permissions and Reprints

ABSTRACT

Word deafness is an intriguing neurological syndrome characterized by severe difficulties in the ability to understand or reproduce spoken language with otherwise intact speech production and nonauditory language comprehension. The disorder is of significant theoretical importance because it putatively supports the modularity of speech recognition from more central language computational networks. However, the specificity and functional locus of the processing disturbances resulting in word deafness remains unclear. This article discusses the nature and potential treatment of word deafness, with particular reference to a detailed case study (neuropsychological, neuroimaging, and event-related potential) of an adult case of Landau-Kleffner syndrome. The findings in this case suggested temporal lobe pathophysiology involving cortical mechanisms concerned with temporal processing of rapid frequency changes in sound. We propose that these deficits may impede the analysis of linguistically important transitional frequency cues in speech and account for the profound difficulties in understanding and producing speech. The implications of these findings for approaches to the treatment of word deafness are discussed.

REFERENCES

• 1 Goldstein K. Language and Language Disturbance. New York, NY; Grune & Stratton 1948
  Google Scholar
• 2 Fung V S, Sue C M, Somerville E R. Paroxysmal word deafness secondary to focal epilepsy.  Neurology. 2000;  54 533-534
  PubMedGoogle Scholar
• 3 Stefanatos G, Kinsbourne M, Wasserstein J. Acquired epileptiform aphasia: a dimensional view of Landau-Kleffner syndrome and the relation to regressive autistic spectrum disorders.  Child Neuropsychol. 2002;  8 195-228
  CrossrefPubMedGoogle Scholar
• 4 Seliger G M, Lefever F, Lukas R et al.. Word deafness in head injury: implications for coma assessment and rehabilitation.  Brain Inj. 1991;  5 53-56
  CrossrefPubMedGoogle Scholar
• 5 Wirkowski E, Echausse N, Overby C, Ortiz O, Radler L. I can hear you yet cannot comprehend: a case of pure word deafness.  J Emerg Med. 2006;  30 53-55
  CrossrefPubMedGoogle Scholar
• 6 Hattiangadi N, Pillion J P, Slomine B, Christensen J, Trovato M K, Speedie L J. Characteristics of auditory agnosia in a child with severe traumatic brain injury: a case report.  Brain Lang. 2005;  92 12-25
  CrossrefPubMedGoogle Scholar
• 7 Otsuki M, Soma Y, Sato M, Homma A, Tsuji S. Slowly progressive pure word deafness.  Eur Neurol. 1998;  39 135-140
  CrossrefPubMedGoogle Scholar
• 8 Croisile B, Laurent B, Michel D, Le Bars D, Cinotti L, Mauguiere F. Different clinical types of degenerative aphasia [Review] [in French].  Rev Neurol (Paris). 1991;  147 192-199
  PubMedGoogle Scholar
• 9 Mesulam M M. Slowly progressive aphasia without generalized dementia.  Ann Neurol. 1982;  11 592-598
  CrossrefPubMedGoogle Scholar
• 10 Goldstein M N, Brown M, Hollander J. Auditory agnosia and cortical deafness: analysis of a case with three-year followup.  Brain Lang. 1975;  2 324-332
  CrossrefPubMedGoogle Scholar
• 11 Donaldson J O, Hale M S, Klau M. A case of reversible pure-word deafness during lithium toxicity.  Am J Psychiatry. 1981;  138 242-243
  PubMedGoogle Scholar
• 12 Kussmaul A. Disturbances of speech. In: von Ziemssien H Cyclopedia of the Practice of Medicine. New York, NY; William Wood 1877: 581-875
  Google Scholar
• 13 Polster M R, Rose S B. Disorders of auditory processing: evidence for modularity in audition.  Cortex. 1998;  34 47-65
  CrossrefPubMedGoogle Scholar
• 14 Poeppel D. Pure word deafness and the bilateral processing of the speech code.  Cogn Sci. 2001;  25 679-693
  CrossrefPubMedGoogle Scholar
• 15 Kale U, el-Naggar M, Hawthorne M. Verbal auditory agnosia with focal EEG abnormality: an unusual case of a child presenting to an ENT surgeon with “deafness”.  J Laryngol Otol. 1995;  109 431-432
  PubMedGoogle Scholar
• 16 Rapin I. Verbal auditory agnosia in children.  Dev Med Child Neurol. 1988;  30 685
  PubMedGoogle Scholar
• 17 Blum A, Tremont G, Donahue J et al.. Landau-Kleffner syndrome with lateral temporal focal cortical dysplasia and mesial temporal sclerosis: a 30-year follow-up.  Epilepsy Behav. 2007;  10 495-503
  CrossrefPubMedGoogle Scholar
• 18 Cole A J, Andermann F, Taylor L et al.. The Landau-Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis.  Neurology. 1988;  38 31-38
  PubMedGoogle Scholar
• 19 Praline J, Hommet C, Barthez M A et al.. Outcome at adulthood of the continuous spike-waves during slow sleep and Landau-Kleffner syndromes.  Epilepsia. 2003;  44 1434-1440
  CrossrefPubMedGoogle Scholar
• 20 Rossi P G, Parmeggiani A, Posar A, Scaduto M C, Chiodo S, Vatti G. Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES).  Brain Dev. 1999;  21 90-98
  CrossrefPubMedGoogle Scholar
• 21 Wernicke C. The aphasic symptom complex: a psychological study on a neurological basis. Reprinted in Cohen RS, Wartofsky MW Boston Studies in the Philosophy of Science. Vol. 4. Boston, MA; Reidel 1874
  Google Scholar
• 22 Lichtheim L. On aphasia.  Brain. 1885;  7 433-484
  PubMedGoogle Scholar
• 23 Bastian H C. Some problems in connexion with aphasia and other speech defects.  Lancet. 1897;  1 1131-1137
  PubMedGoogle Scholar
• 24 Liepmann H. Ein Fall von reiner Sprachtaubheit. In: Wernicke C Psychiatrische Abhandlungen. Germany; Schlette 1898
  Google Scholar
• 25 Gazzaniga M S, Glass A V, Sarno M T, Posner J B. Pure word deafness and hemispheric dynamics: a case history.  Cortex. 1973;  9 136-143
  PubMedGoogle Scholar
• 26 Goldstein M. Auditory agnosia for speech (pure word-deafness): a historical review with current implications.  Brain Lang. 1974;  1 195-204
  CrossrefPubMedGoogle Scholar
• 27 Buchman A S, Garron D C, Trost-Cardamone J E, Wichter M D, Schwartz M. Word deafness: one hundred years later.  J Neurol Neurosurg Psychiatry. 1986;  49 489-499
  CrossrefPubMedGoogle Scholar
• 28 Stefanatos G A, Gershkoff A, Madigan S. On pure word deafness, temporal processing, and the left hemisphere.  J Int Neuropsychol Soc. 2005;  11 456-470 discussion 455
  PubMedGoogle Scholar
• 29 Bauer R M, Zawacki T. Auditory agnosia and amusia. In: Farah MJ, Feinberg TE Patient-Based Approaches to Cognitive Neuroscience: Issues in Clinical and Cognitive Neuropsychology. Cambridge, MA; MIT Press 2000: 97-106
  Google Scholar
• 30 Metz-Lutz M-N, Dahl E. Analysis of word comprehension in a case of pure word deafness.  Brain Lang. 1984;  23 13-25
  CrossrefPubMedGoogle Scholar
• 31 Wang E, Peach R K, Xu Y, Schneck M, Manry II C. Perception of dynamic acoustic patterns by an individual with unilateral verbal auditory agnosia.  Brain Lang. 2000;  73 442-455
  CrossrefPubMedGoogle Scholar
• 32 Takahashi N, Kawamura M, Shinotou H, Hirayama K, Kaga K, Shindo M. Pure word deafness due to left hemisphere damage.  Cortex. 1992;  28 295-303
  PubMedGoogle Scholar
• 33 Saffran E M, Marin O S, Yeni-Komshian G H. An analysis of speech perception in word deafness.  Brain Lang. 1976;  3 209-228
  CrossrefPubMedGoogle Scholar
• 34 Franklin S. Disssociations in auditory word comprehension: evidence from fluent aphasic patients.  Aphasiology. 1989;  3 189-207
  CrossrefPubMedGoogle Scholar
• 35 Praamstra P, Hagoort P, Maassen B, Crul T. Word deafness and auditory cortical function. A case history and hypothesis.  Brain. 1991;  114(Pt 3) 1197-1225
  CrossrefPubMedGoogle Scholar
• 36 Albert M L, Bear D. Time to understand. A case study of word deafness with reference to the role of time in auditory comprehension.  Brain. 1974;  97 373-384
  CrossrefPubMedGoogle Scholar
• 37 Landau W M, Kleffner F R. Syndrome of acquired aphasia with convulsive disorder in children.  Neurology. 1957;  7 523-530
  CrossrefPubMedGoogle Scholar
• 38 Adam C, Rouleau I, Saint-Hilaire J M. Postictal aphasia and paresis: a clinical and intracerebral EEG study.  Can J Neurol Sci. 2000;  27 49-54
  PubMedGoogle Scholar
• 39 Goldberg-Stern H, Gadoth N, Cahill W, Privutera M. Language dysfunction after frontal lobe partial seizures.  Neurology. 2004;  62(9) 1637-1638
  PubMedGoogle Scholar
• 40 Worster-Drought C. An usual form of acquired aphasia in children.  Dev Med Child Neurol. 1971;  13 563-571
  PubMedGoogle Scholar
• 41 Bishop D V. Comprehension of spoken, written and signed sentences in childhood language disorders.  J Child Psychol Psychiatry. 1982;  23 1-20
  CrossrefPubMedGoogle Scholar
• 42 Rapin I, Mattis S, Rowan A J, Golden G G. Verbal auditory agnosia in children.  Dev Med Child Neurol. 1977;  19 197-207
  PubMedGoogle Scholar
• 43 Gordon N. Acquired aphasia in childhood: the Landau-Kleffner syndrome.  Dev Med Child Neurol. 1990;  32 270-274
  PubMedGoogle Scholar
• 44 Korkman M, Granstrom M L, Appelqvist K, Liukonen E. Neuropsychological characteristics of five children with the Landau-Kleffner syndrome: dissociation of auditory and phonological discrimination.  J Int Neuropsychological Society. 1998;  4 566-575
  PubMedGoogle Scholar
• 45 Perez E R, Davidoff V. Sign language in childhood epileptic aphasia (Landau-Kleffner syndrome).  Dev Med Child Neurol. 2001;  43 739-744
  CrossrefPubMedGoogle Scholar
• 46 Woll B, Sieratzki J S. Sign language for children with acquired aphasia.  J Child Neurol. 1996;  11 347-349
  PubMedGoogle Scholar
• 47 van de Sandt-Koenderman W M, Smit I A, van Dongen H R, van Hest J B. A case of acquired aphasia and convulsive disorder: some linguistic aspects of recovery and breakdown.  Brain Lang. 1984;  21 174-183
  CrossrefPubMedGoogle Scholar
• 48 Deonna T, Peter C, Ziegler A L. Adult follow-up of the acquired aphasia-epilepsy syndrome in childhood. Report of 7 cases.  Neuropediatrics. 1989;  20 132-138
  Article in Thieme ConnectPubMedGoogle Scholar
• 49 Penn C, Friedlander R I, Saling M M. Acquired childhood aphasia with convulsive disorder (Landau-Kleffner syndrome). A case report.  S Afr Med J. 1990;  77 158-161
  PubMedGoogle Scholar
• 50 Paquier P F, Van Dongen H R, Loonen C B. The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder. Long-term follow-up of six children and a review of the recent literature.  Arch Neurol. 1992;  49 354-359
  CrossrefPubMedGoogle Scholar
• 51 Gascon G, Victor D, Lombroso C T. Language disorders, convulsive disorder, and electroencephalographic abnormalities. Acquired syndrome in children.  Arch Neurol. 1973;  28 156-162
  PubMedGoogle Scholar
• 52 Deuel R K, Lenn N J. Treatment of acquired epileptic aphasia.  J Pediatr. 1977;  90 959-961
  PubMedGoogle Scholar
• 53 Cooper J A, Ferry P C. Acquired auditory verbal agnosia and seizures in childhood.  J Speech Hear Disord. 1978;  43 176-184
  PubMedGoogle Scholar
• 54 Sato S, Dreifuss F E. Electroencephalographic findings in a patient with developmental expressive aphasia.  Neurology. 1973;  23 181-185
  PubMedGoogle Scholar
• 55 Marien P, Saerens J, Verslegers W, Borggreve F, De Deyn P P. Some controversies about type and nature of aphasic symptomatology in Landau-Kleffner's syndrome: a case study.  Acta Neurol Belg. 1993;  93 183-203
  PubMedGoogle Scholar
• 56 Holmes G L, McKeever M, Saunders Z. Epileptiform activity in aphasia of childhood: an epiphenomenon?.  Epilepsia. 1981;  22 631-639
  PubMedGoogle Scholar
• 57 Nakano S, Okuno T, Mikawa H. Landau-Kleffner syndrome. EEG topographic studies.  Brain Dev. 1989;  11 43-50
  PubMedGoogle Scholar
• 58 Nevsimalova S, Tauberova A, Doutlik S, Kucera V, Dlouha O. A role of autoimmunity in the etiopathogenesis of Landau-Kleffner syndrome?.  Brain Dev. 1992;  14 342-345
  PubMedGoogle Scholar
• 59 Perniola T, Margari L, Buttiglione M, Andreula C, Simone I L, Santostasi R. A case of Landau-Kleffner syndrome secondary to inflammatory demyelinating disease.  Epilepsia. 1993;  34 551-556
  CrossrefPubMedGoogle Scholar
• 60 Connolly A M, Chez M G, Pestronk A, Arnold S T, Mehta S, Deuel R K. Serum autoantibodies to brain in Landau-Kleffner variant, autism, and other neurologic disorders.  J Pediatr. 1999;  134 607-613
  PubMedGoogle Scholar
• 61 Zardini G, Molteni B, Nardocci N, Sarti D, Avanzini G, Granata T. Linguistic development in a patient with Landau-Kleffner syndrome: a nine-year follow-up.  Neuropediatrics. 1995;  26 19-25
  Article in Thieme ConnectPubMedGoogle Scholar
• 62 Tharpe A M, Johnson G D, Glasscock III M E. Diagnostic and management considerations of acquired epileptic aphasia or Landau-Kleffner syndrome.  Am J Otol. 1991;  12 210-214
  PubMedGoogle Scholar
• 63 Denes G, Balliello S, Volterra V, Pellegrini A. Oral and written language in a case of childhood phonemic deafness.  Brain Lang. 1986;  29 252-267
  CrossrefPubMedGoogle Scholar
• 64 Isnard J, Fischer C, Bastuji H, Badinand N, Villard R. Auditory early (BAEP) and middle-latency (MLAEP) evoked potentials in patients with CSWS and Landau-Kleffner syndrome. In: Beaumanior A, Bureau M, Deonna T, Mira L, Tassinari CA Continuous Spikes and Waves during Slow Sleep. Eastleigh, United Kingdom; John Libbey 1995: 99-103
  Google Scholar
• 65 Paetau R, Kajola M, Korkman M, Hamalainen M, Granstrom M L, Hari R. Landau-Kleffner syndrome: epileptic activity in the auditory cortex.  Neuroreport. 1991;  2 201-204
  PubMedGoogle Scholar
• 66 Boyd S G, Rivera-Gaxiola M, Towell A D, Harkness W, Neville B G. Discrimination of speech sounds in a boy with Landau-Kleffner syndrome: an intraoperative event-related potential study.  Neuropediatrics. 1996;  27 211-215
  Article in Thieme ConnectPubMedGoogle Scholar
• 67 Paetau R, Kajola M, Korkman M, Hamalainen M, Granstrom M L, Hari R. Landau-Kleffner syndrome: epileptic activity in the auditory cortex.  Neuroreport. 1991;  2 201-204
  PubMedGoogle Scholar
• 68 Paetau R. Sounds triggers spikes in the Landau-Kleffner syndrome.  J Clin Neurophysiol. 1994;  11 231-241
  PubMedGoogle Scholar
• 69 Klein S K, Kurtzberg D, Brattson A et al.. Electrophysiologic manifestations of impaired temporal lobe auditory processing in verbal auditory agnosia.  Brain Lang. 1995;  51 383-405
  CrossrefPubMedGoogle Scholar
• 70 Paty J, Morault P. Electrocortical study of acquired language disorders in children.  Rev Laryngol Otol Rhinol (Bord). 1984;  105 333-338
  PubMedGoogle Scholar
• 71 Stefanatos G A. Frequency modulation analysis in children with Landau-Kleffner syndrome.  Ann N Y Acad Sci. 1993;  682 412-414
  CrossrefPubMedGoogle Scholar
• 72 Auerbach S H, Allard T, Naeser M, Alexander M P, Albert M L. Pure word deafness. Analysis of a case with bilateral lesions and a defect at the prephonemic level.  Brain. 1982;  105 271-300
  CrossrefPubMedGoogle Scholar
• 73 Miller G, Taylor J. Perception of repeated bursts of noise.  J Acoust Soc Am. 1948;  20 171-182
  CrossrefPubMedGoogle Scholar
• 74 Kay R H, Matthews D R. On the existence in human auditory pathways of channels selectively tuned to the modulation present in frequency-modulated tones.  J Physiol. 1972;  225 657-677
  PubMedGoogle Scholar
• 75 Regan D, Tansley B W. Selective adaptation to frequency-modulated tones: evidence for an information-processing channel selectively sensitive to frequency changes.  J Acoust Soc Am. 1979;  65 1249-1257
  CrossrefPubMedGoogle Scholar
• 76 Stefanatos G A, Foley C, Grover W, Doherty B. Steady-state auditory evoked responses to pulsed frequency modulations in children.  Electroencephalogr Clin Neurophysiol. 1997;  104 31-42
  PubMedGoogle Scholar
• 77 Kaga M. Language disorders in Landau-Kleffner syndrome.  J Child Neurol. 1999;  14 118-122
  CrossrefPubMedGoogle Scholar
• 78 Baynes K, Kegl J A, Brentari D, Kussmaul C, Poizner H. Chronic auditory agnosia following Landau-Kleffner syndrome: a 23 year outcome study.  Brain Lang. 1998;  63 381-425
  CrossrefPubMedGoogle Scholar
• 79 Sieratzki J S, Calvert G A, Brammer M, David A, Woll B. Accessibility of spoken, written, and sign language in Landau-Kleffner syndrome: a linguistic and functional MRI study.  Epileptic Disord. 2001;  3 79-89
  PubMedGoogle Scholar
• 80 Sawhney I M, Suresh N, Dhand U K, Chopra J S. Acquired aphasia with epilepsy–Landau-Kleffner syndrome.  Epilepsia. 1988;  29 283-287
  CrossrefPubMedGoogle Scholar
• 81 Johnsrude I S, Zatorre R J, Milner B A, Evans A C. Left-hemisphere specialization for the processing of acoustic transients.  Neuroreport. 1997;  8 1761-1765
  PubMedGoogle Scholar
• 82 Binder J R, Frost J A, Hammeke T A et al.. Human temporal lobe activation by speech and nonspeech sounds.  Cereb Cortex. 2000;  10 512-528
  CrossrefPubMedGoogle Scholar
• 83 Zatorre R J, Belin P, Penhune V B. Structure and function of auditory cortex: music and speech.  Trends Cogn Sci. 2002;  6 37-46
  CrossrefPubMedGoogle Scholar
• 84 Patterson J, Green D. Discrimination of transient signals having identical energy spectra.  J Acoust Soc Am. 1970;  48 894-905
  CrossrefPubMedGoogle Scholar
• 85 Hirsch I. Temporal aspects of hearing. In: Tower DB The Nervous System. 3rd volume: Human Communication Disorders. New York, NY; Raven Press 1975: 157-162
  Google Scholar
• 86 Godefroy O, Leys D, Furby A et al.. Psychoacoustical deficits related to bilateral subcortical hemorrhages. A case with apperceptive auditory agnosia.  Cortex. 1995;  31 149-159
  CrossrefPubMedGoogle Scholar
• 87 Motomura N, Yamadori A, Mori E, Tamaru F. Auditory agnosia. Analysis of a case with bilateral subcortical lesions.  Brain. 1986;  109 379-391
  CrossrefPubMedGoogle Scholar
• 88 Tanaka Y, Yamadori A, Mori E. Pure word deafness following bilateral lesions. A psychophysical analysis.  Brain. 1987;  110(Pt 2) 381-403
  CrossrefPubMedGoogle Scholar
• 89 Billard C, Santini J J, Dulac O, Autret A, de Giovanni E. Acquired verbal deafness in the child. 5 cases (aphasia-epilepsy syndrome) [author's translation].  Rev Otoneuroophtalmol. 1981;  53 331-336
  PubMedGoogle Scholar
• 90 Moatti L, Dulac O, Arthuis M, Cotin G. Apparent deafness as a manifestation of an aphasia-epilepsy syndrome.  Ann Otolaryngol Chir Cervicofac. 1983;  100 281-285
  PubMedGoogle Scholar
• 91 Appleton R E. The Landau-Kleffner syndrome.  Arch Dis Child. 1995;  72 386-387
  CrossrefPubMedGoogle Scholar
• 92 Gerard C L, Dugas M, Sagar D. Speech therapy in Landau and Kleffner syndrome. In: Martins ACIP, Dogen HRV Acquired Aphasia in Children: Acquisition and Breakdown of Language in the Developing Brain. Dordrecht, The Netherlands; Kluwer Academic Publishers 1991: 279-290
  Google Scholar
• 93 Vance M S. Educational and therapeutic approaches used with a child presenting with acquired aphasia with convulsive disorder (Landau-Kleffner syndrome).  Child Lang Teach Ther. 1991;  7 41-60
  CrossrefPubMedGoogle Scholar
• 94 Pedro V M, Leisman G. Hemispheric integrative therapy in Landau-Kleffner syndrome: applications for rehabilitation sciences.  Int J Neurosci. 2005;  115 1227-1238
  CrossrefPubMedGoogle Scholar
• 95 Gielewski R. Acoustic analysis and auditory retraining in the remediation of sensory aphasia. In: Code C, Muller DJ Aphasia Therapy. London, United Kingdom; Whurr; 1989: 38-145
  Google Scholar
• 96 Tessier C, Weill-Chounlamountry A, Michelot N, Pradat-Diehl P. Rehabilitation of word deafness due to auditory analysis disorder.  Brain Inj. 2007;  21 1165-1174
  CrossrefPubMedGoogle Scholar
• 97 Morris J, Franklin S, Ellis A W, Turner J E, Bailey P J. Remediation a speech perception deficit in an aphasic patient.  Aphasiology. 1996;  10 137-158
  CrossrefPubMedGoogle Scholar
• 98 Maneta A, Marshall J, Lindsay J. Direct and indirect therapy for word sound deafness.  Int J Lang Commun Disord. 2001;  36 91-106
  PubMedGoogle Scholar
• 99 Morris J. Remediating auditory processing deficits in adults with aphasia. In: Chiat S, Law J, Marshall J Language Disorders in Children and Adults. London, United Kingdom; Whurr 1997
  Google Scholar
• 100 Shindo M, Kaga K, Tanaka Y. Speech discrimination and lip reading in patients with word deafness or auditory agnosia.  Brain Lang. 1991;  40 153-161
  CrossrefPubMedGoogle Scholar
• 101 Tallal P, Miller S L, Bedi G et al.. Language comprehension in language-learning impaired children improved with acoustical modified speech.  Science. 1996;  271(5245) 81-84
  CrossrefPubMedGoogle Scholar
• 102 Stefanatos G A, Gershkoff A, Madigan S. Computer-mediated tools for the investigation and rehabilitation of auditory and phonological processing in aphasia.  Aphasiology. 2005;  19 955-964
  CrossrefPubMedGoogle Scholar
• 103 Wee J, Menard M R. “Pure word deafness”: implications for assessment and management in communication disorder—a report of two cases.  Arch Phys Med Rehabil. 1999;  80 1106-1109
  CrossrefPubMedGoogle Scholar

Gerry A StefanatosD.Phil. 

Department of Communication Sciences and Disorders, Temple University

1201 13th Street, Suite 111, Philadelphia, PA 19122

Email: stefang@temple.edu