J Clin Neurol.  2011 Dec;7(4):227-230. 10.3988/jcn.2011.7.4.227.

Pure Word Deafness in a Patient with Early-Onset Alzheimer's Disease: An Unusual Presentation

• Kim SH
• Suh MK
• Seo SW
• Chin J
• Han SH
• Na DL

• KMID: 2179009
• DOI: http://doi.org/10.3988/jcn.2011.7.4.227

Abstract

BACKGROUND AND PURPOSE
The occurrence of PWD in neurodegenerative disease is very rare, and this is the first report of it being related to early-onset AD. We describe a patient with early-onset Alzheimer's disease (AD) who presented with pure word deafness (PWD).
CASE REPORT
The patient had experienced PWD for 2 years, followed by other cognitive deficits suggestive of parietotemporal dysfunction. Brain imaging including 18FDG-PET and [11C] PIB-PET supported the diagnosis of AD.
CONCLUSIONS
Our case highlights the clinical variability that characterizes early-onset AD.

Keyword

pure word deafness; Alzheimer's disease; primary progressive aphasia

MeSH Terms

Alzheimer Disease
Aphasia, Primary Progressive
Deafness
Humans
Neurodegenerative Diseases
Neuroimaging

Figure

• 

  Fig. 1 Brain MRI of the patient 2 years after the onset of pure word deafness. (A) Mild left temporal atrophy was suspected on axial and coronal scans. (B) 18FDG-PET demonstrated bilateral temporoparietal hypometabolism with greater involvement on the left side, and (C) [11C] PIB-PET showed increased PIB uptake in diffuse cortical areas. (D) A cortical thickness analysis showed cortical thinning in the bilateral Heschl's gyri, planum temporale, and superior temporal sulcus.

Reference

1. Coslett HB, Brashear HR, Heilman KM. Pure word deafness after bilateral primary auditory cortex infarcts. Neurology. 1984. 34:347–352.

2. Denes G, Semenza C. Auditory modality-specific anomia: evidence from a case of pure word deafness. Cortex. 1975. 11:401–411.

3. Iizuka O, Suzuki K, Endo K, Fujii T, Mori E. Pure word deafness and pure anarthria in a patient with frontotemporal dementia. Eur J Neurol. 2007. 14:473–475.

4. Otsuki M, Soma Y, Sato M, Homma A, Tsuji S. Slowly progressive pure word deafness. Eur Neurol. 1998. 39:135–140.

5. Seo SW, Im K, Lee JM, Kim YH, Kim ST, Kim SY, et al. Cortical thickness in single- versus multiple-domain amnestic mild cognitive impairment. Neuroimage. 2007. 36:289–297.

6. Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol. 1982. 11:592–598.

7. Kaga K, Shindo M, Tanaka Y. Central auditory information processing in patients with bilateral auditory cortex lesions. Acta Otolaryngol Suppl. 1997. 532:77–82.

8. Kaga K, Shindo M, Tanaka Y, Haebara H. Neuropathology of auditory agnosia following bilateral temporal lobe lesions: a case study. Acta Otolaryngol. 2000. 120:259–262.

9. 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.

10. Hayashi K, Hayashi R. Pure word deafness due to left subcortical lesion: neurophysiological studies of two patients. Clin Neurophysiol. 2007. 118:863–868.

11. Vitte E, Tankéré F, Bernat I, Zouaoui A, Lamas G, Soudant J. Midbrain deafness with normal brainstem auditory evoked potentials. Neurology. 2002. 58:970–973.

12. Jäncke L, Wüstenberg T, Scheich H, Heinze HJ. Phonetic perception and the temporal cortex. Neuroimage. 2002. 15:733–746.