Tohoku J. Exp. Med., 2012 Dec, 228(4)

Surgical Improvement of Speech Disorder Caused by Amyotrophic Lateral Sclerosis

HIDETO SAIGUSA,1 SATOSHI YAMAGUCHI,1 TSUYOSHI NAKAMURA,1 TARO KOMACHI,1 OSAMU KADOSONO,1 HIROYUKI ITO,1 MAKOTO SAIGUSA2 and SEIJI NIIMI3

1Department of Otolarynology, Nippon Medical School, Tokyo, Japan
2Private Practice, Tyler Oral & Facial Surgery, Tyler, Texas, USA
3Department of Speech-Language Pathology and Audiology, International University of Health and Welfare, Otawara, Tochigi, Japan

Amyotrophic lateral sclerosis (ALS) is a progressive debilitating neurological disease. ALS disturbs the quality of life by affecting speech, swallowing and free mobility of the arms without affecting intellectual function. It is therefore of significance to improve intelligibility and quality of speech sounds, especially for ALS patients with slowly progressive courses. Currently, however, there is no effective or established approach to improve speech disorder caused by ALS. We investigated a surgical procedure to improve speech disorder for some patients with neuromuscular diseases with velopharyngeal closure incompetence. In this study, we performed the surgical procedure for two patients suffering from severe speech disorder caused by slowly progressing ALS. The patients suffered from speech disorder with hypernasality and imprecise and weak articulation during a 6-year course (patient 1) and a 3-year course (patient 2) of slowly progressing ALS. We narrowed bilateral lateral palatopharyngeal wall at velopharyngeal port, and performed this surgery under general anesthesia without muscle relaxant for the two patients. Postoperatively, intelligibility and quality of their speech sounds were greatly improved within one month without any speech therapy. The patients were also able to generate longer speech phrases after the surgery. Importantly, there was no serious complication during or after the surgery. In summary, we performed bilateral narrowing of lateral palatopharyngeal wall as a speech surgery for two patients suffering from severe speech disorder associated with ALS. With this technique, improved intelligibility and quality of speech can be maintained for longer duration for the patients with slowly progressing ALS.

keywords —— amyotrophic lateral sclerosis (ALS); narrowing of lateral palatopharyngeal wall surgery; speech disorder; speech surgery; velopharyngeal closure incompetence

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Tohoku J. Exp. Med., 2012, 228, 371-376

Correspondence: Hideto Saigusa, Department of Otolaryngology, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.

e-mail: s-hideto@nms.ac.jp