Abstract
Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT. A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%. A Med-el Flex(SOFT) cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively. This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT...Continue Reading
References
Jan 1, 1987·Acta Oto-laryngologica·E RaglanP Rudge
Jun 1, 1996·Brain : a Journal of Neurology·A StarrC I Berlin
Dec 22, 1999·Neuroreport·F G ZengA Starr
Jun 27, 2000·Brain : a Journal of Neurology·K M KrajewskiM E Shy
May 19, 2001·The Laryngoscope·J K ShallopC L Driscoll
Mar 29, 2002·American Journal of Medical Genetics·M J KovachV E Kimonis
May 23, 2002·The Annals of Otology, Rhinology & Laryngology. Supplement·Yvonne S Sininger, Patricia Trautwein
Jan 7, 2003·The Laryngoscope·John C MasonGeorge T Hashisaki
Feb 13, 2003·Neurology·Nyamkhishig SambuughinKumaraswamy Sivakumar
Jun 7, 2003·International Journal of Pediatric Otorhinolaryngology·Isabelle Rapin, Judith Gravel
Apr 22, 2004·Neuromuscular Disorders : NMD·In Soo JooJong-Won Kim
Nov 3, 2004·The Neurologist·Tulio BertoriniHani Rashed
Jun 15, 2006·The Journal of Laryngology and Otology·J T F Postelmans, R J Stokroos
Nov 21, 2007·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Christina L Runge-SamuelsonP Ashley Wackym
Feb 5, 2008·Cochlear Implants International·William P R Gibson, John M Graham
Mar 21, 2008·Seminars in Neurology·Agnes Jani-AcsadiMichael E Shy
Mar 12, 2009·European Journal of Human Genetics : EJHG·Kinga Szigeti, James R Lupski
Citations
Oct 16, 2015·Brain : a Journal of Neurology·Gary Rance, Arnold Starr
Mar 15, 2013·Cochlear Implants International·Becky FrewinNeil Donnelly
Dec 25, 2016·Cochlear Implants International·Sarah E Mowry, Sarah King
Aug 9, 2019·Molecular Genetics & Genomic Medicine·Justine LeratAnne-Sophie Lia
Aug 28, 2019·Laryngoscope Investigative Otolaryngology·Aiden Eliot Shearer, Marlan R Hansen
May 22, 2018·Case Reports in Medicine·C Lane AnzaloneMatthew L Carlson
Apr 16, 2020·Journal of Clinical Medicine·Romolo Daniele De SiatiNaïma Deggouj
Feb 25, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Shinsaku Matsuda, Kimitaka Kaga