The Japanese journal of neuropsychology

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ArticleTitle	A case of neurodegenerative disease presenting with primary progressive hearing loss and logoclonia
Language	J
AuthorList	Yeong-ae Eiai Lee¹⁾, Sumio Ishiai²⁾, Sadakiyo Watabiki³⁾, Mafuyu Takahashi³⁾
Affiliation	1) Department of Speech Therapy, Musashino Red Cross Hospital 2) Department of Rehabilitation, Tokyo Metropolitan Institute for Neuroscience 3) Department of Neurology, Musashino Red Cross Hospital
Publication	Japanese Journal of Neuropsychology: 17 (1), 54-61, 2001
Received	Aug 28, 2000
Accepted	Sep 19, 2000
Abstract	Logoclonia sometimes occurs as a language disorder in the late-stage of Alzheimar's disease. We report a 69-year-old right-handed woman who showed slowly progressive hearing loss and logoclonia. She initially developed mild difficulty in hearing voice over the phone. About one year later, her family noticed that her speech gradually became abnormal. The hearing level of the patient worsened in four years to 81 dB in the right ear and 105 dB in the left ear. Auditory brain stem response was evoked normally on either side. Her speech was characterized by rapid repetitions of syllables which were often difficult to describe because of distortion, assimilation, and addition of irrelevant syllables. The speech disturbance was considered to be classified as logoclonia, although repetitions were not restricted to the last syllable of words or sentences. Unlike stuttering, prolongation and blocks were not observed, and she was indifferent to her speech disturbance. After about five years of progressive hearing loss and logoclonia, the patient developed a tendency to be mute and apparent aphasic symptoms, while her visuospatial ability was well preserved. Besides, she became very obstinate and adopted a fixed daily routine. The presence of mild dementia was suspected in the daily activities. The patient showed no extrapyramidal sign or neuroradiological abnormality on MRI and SPECT examinations. We, however, consider that degenerative changes may have progressed in the subcortical auditory pathways and the basal ganglionic structures that regulate speech output. The appearance of aphasia, mutism, and personality change without visuospatial impairment in the late stage suggests that the patient suffered a cerebral disease related to front-temporal dementia. The present case demonstrated that hearing loss and logoclonia may be the initial symptoms of degenerative diseases.
Keywords	cortical deafness, speech disorder, logoclonia, reiterative speech, neurodegenerative disease