In Vivo Electrocochleography in Hybrid Cochlear Implant Users Implicates TMPRSS3 in Spiral Ganglion Function

Scientific Reports
A Eliot ShearerRichard J H Smith

Abstract

Cochlear implantation, a surgical method to bypass cochlear hair cells and directly stimulate the spiral ganglion, is the standard treatment for severe-to-profound hearing loss. Changes in cochlear implant electrode array design and surgical approach now allow for preservation of acoustic hearing in the implanted ear. Electrocochleography (ECochG) was performed in eight hearing preservation subjects to assess hair cell and neural function and elucidate underlying genetic hearing loss. Three subjects had pathogenic variants in TMPRSS3 and five had pathogenic variants in genes known to affect the cochlear sensory partition. The mechanism by which variants in TMPRSS3 cause genetic hearing loss is unknown. We used a 500-Hz tone burst to record ECochG responses from an intracochlear electrode. Responses consist of a cochlear microphonic (hair cell) and an auditory nerve neurophonic. Cochlear microphonics did not differ between groups. Auditory nerve neurophonics were smaller, on average, in subjects with TMPRSS3 deafness. Results of this proof-of-concept study provide evidence that pathogenic variants in TMPRSS3 may impact function of the spiral ganglion. While ECochG as a clinical and research tool has been around for decades, this...Continue Reading

References

Oct 1, 1989·Hearing Research·R B PatuzziB M Johnstone
Nov 1, 1983·The Annals of Otology, Rhinology, and Laryngology·M PortmannY Cazals
May 23, 2000·The American Journal of Otology·D E BrackmannJ N Fayad
Sep 29, 2001·International Journal of Pediatric Otorhinolaryngology·J B Nadol, S N Merchant
Feb 1, 1962·The Journal of Speech and Hearing Disorders·G E PETERSON, I LEHISTE
Oct 2, 2003·The Laryngoscope·Bruce J Gantz, Christopher W Turner
Aug 4, 2006·The Laryngoscope·Jose N Fayad, Fred H Linthicum
Oct 14, 2008·Nature·Elisabeth VerpyChristine Petit
Jan 22, 2010·Ear and Hearing·Holly F B TeagleCraig A Buchman
Jul 17, 2010·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Jae-Ryong KimLee-Suk Kim
Jul 26, 2011·Journal of the Association for Research in Otolaryngology : JARO·Nicole J D WeegerinkHenricus P M Kunst
Dec 21, 2012·Human Molecular Genetics·Laurence MolinaBenjamin Delprat
Jun 28, 2013·Journal of Medical Genetics·A Eliot ShearerRichard J H Smith
Sep 3, 2013·International Journal of Audiology·Thomas LenarzAlain Uziel
Oct 18, 2013·Journal of Neurophysiology·Mathieu ForguesDouglas C Fitzpatrick
Dec 10, 2013·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Douglas C FitzpatrickOliver F Adunka
Feb 12, 2014·Journal of the Association for Research in Otolaryngology : JARO·J T LichtenhanA N Salt
Feb 15, 2014·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Juyong ChungByung Yoon Choi
Aug 15, 2014·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Mohammad Seyyedi, Joseph B Nadol
Jul 15, 2015·The Laryngoscope·J Thomas RolandUNKNOWN Multicenter Clinical Trial Group
Jan 13, 2016·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Aanand N AcharyaGunesh P Rajan
Jan 13, 2016·The Laryngoscope·Bruce J GantzChristopher Turner
Mar 13, 2016·Human Genetics·Christina M Sloan-HeggenRichard J H Smith
Aug 16, 2016·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Kathleen CampbellColleen Le Prell

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Citations

Aug 28, 2019·Laryngoscope Investigative Otolaryngology·Aiden Eliot Shearer, Marlan R Hansen
Oct 28, 2019·Cochlear Implants International·Leslie P Molina-RamirezGraeme C M Black
Feb 26, 2020·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Christopher R KaufmannMarlan R Hansen
May 19, 2020·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Eleonora M C TreccaOliver F Adunka
Feb 9, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Jourdan T HolderRené H Gifford
Apr 23, 2021·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Eric NisenbaumXue Zhong Liu

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Methods Mentioned

BETA
cochlear implant
cochlear implants
three

Software Mentioned

ECochG
OtoSCOPE
Custom Sound EP

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