Cochlear implantation in patient with Charcot-Marie-Tooth disease |
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| Authors: | Masumi Kobayashi Tadao Yoshida Satofumi Sugimoto Masaaki Teranishi Daisuke Hara Yukari Kimata Michihiko Sone |
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| Affiliation: | 1. Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, Nagoya, Japan;2. Department of Rehabilitaion, Nagoya University Graduate School of Medicine, Nagoya, Japan;1. Department of Otolaryngology, National Hospital Organization Tokyo Medical Center, 2-5-1 Higashigaoka, Meguro, Tokyo 152-8902, Japan;2. Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Japan;3. Medical Genetics Center, National Hospital Organization Tokyo Medical Center, Japan;1. Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, 65, Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan;2. Department of Otolaryngology-Head and Neck Surgery, Stanford University School of Medicine, Stanford, CA, United States;3. Department of Urology, Nagoya University Graduate School of Medicine, Nagoya, Japan;1. Department of Otorhinolaryngology, Head and Neck Surgery, Tokyo Medical University, Tokyo, Japan;2. Department of Otolaryngology-Head and Neck Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan;3. Department of Anatomic Pathology, Tokyo Medical University School of Medicine, Tokyo, Japan;1. New York University Grossman School of Medicine, Department of Otolaryngology-Head and Neck Surgery, 462 1st Avenue, NBV 5E5, New York 10016, NY, USA;2. House Ear Institute, Los Angeles, CA, USA |
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| Abstract: | Two patients with auditory neuropathy spectrum disorder (ANSD) considered to be associated with Charcot-Marie-Tooth (CMT) are reported. In case 1, a 23-year-old man presented with progressive bilateral sensorineural hearing loss (SNHL) from 10 years of age and was diagnosed with ANSD. He was later diagnosed with CMT by neurological testing. In case 2, a 16-year-old girl, the younger sister of the patient in case 1, presented with progressive SNHL with similar auditory findings since 6 years of age as those of her brother. Both cases underwent bilateral cochlear implantation. In case 1, the maximum discrimination score improved to 45% 24 months after the first side of cochlear implantation from 5% before the surgery. In case 2, the score was 5% 10 months after cochlear implant (CI) surgery from 0% before the surgery. CI treatment for CMT patients has been considered difficult because of both failure in synchronization of nerve conduction due to demyelination and axonal failure of the auditory nerve. Though slower progress compared to the average subset of patients receiving CI was seen, significant improvement was gradually observed in the present patients after bilateral cochlear implantations. CI is thus a viable option for rehabilitation of SNHL in CMT patients. |
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