Abstract
Introduction: People with auditory neuropathy spectrum disorder have problems processing sound and understanding language; by having one or more structures of the auditory pathway auditory pathway affected. The cochlear implant has been proposed as a rehabilitation option. Electrocochleography applied in patients with auditory neuropathy spectrum disorder, allows for a topo-diagnosis of the lesion, to obtain more reliable expectations of the auditory performance of a patient with this pathology who is a candidate for cochlear implant. Objective: To demonstrate the usefulness of electrocochleography in the evaluation protocol of patients with auditory neuropathy spectrum disorder for cochlear implantation. Methods: Qualitative literature review study based on a literature search between 2020 and 2023 in the EBSCO, Scielo, PubMed and Medline databases, using as descriptors the words electrocochleography AND auditory neuropathy spectrum disorder OR auditory neuropathy, electrocochleography AND auditory neuropathy spectrum disorder OR auditory neurpathy, electrocochleography AND cochlear implant, electrocochleography AND cochlear implant, cochlear implant AND auditory neuropathy spectrum disorder OR auditory neuropathy, cochlear implant AND auditory neuropathy spectrum disorder OR auditory neuropathy, articles published from 2012 to 2024 were selected, in Spanish, English and Portuguese languages, for a total of 21 bibliographical references. Results: Auditory rehabilitation in people with auditory neuropathy spectrum disorder has been a topic under discussion, while some support the idea of cochlear implant, others do not. Electrocochleography gives us the opportunity to study the site of the lesion and provides us with a more realistic prognosis in terms of approach, performance, and expectations of cochlear implant treatment. Conclusions: The application of electrocochleography in cochlear implant candidate patients diagnosed with auditory neuropathy spectrum disorder has a great contribution in terms of topo-diagnosis, for the proper management of expectations, regarding the results of the implant.
References
Organización Mundial de la Salud [Internet]. Suiza:la Organización; 2024. Sordera y pérdida de la audición. [aprox. 7 pantallas]. Disponible en: https://www.who.int/es/news-room/fact-sheets/detail/deafness-and-hearing-loss#:~:text=P%C3%A9rdida%20de%20audici%C3%B3n%20y%20sordera,%2C%20moderada%2C%20grave%20o%20profunda.
American Speech Languaje Hearing Association. Tipo, grado y configuración de la pérdida de audición [Internet]. Estados Unidos: ASHA;2023. Disponible en: https://www.asha.org/siteassets/ais/ais-type-degree-and-configuration-of-hearing-loss-spanish.pdf?srsltid=AfmBOoou_g9hRBEmKyFNYzyyBFlVz5jKpEyoOlCBppiEkQ3QCgD05GMF
Núñez F, Jáudenes C, Sequí JM, Vivanco A, Zubicaray J, Olleta I. Actualización de los programas de detección precoz de la sordera infantil: recomendaciones CODEPEH2019 (Niveles 2,3 y 4: diagnóstico, tratamiento y seguimiento). Rev Esp Discapac[Internet]. 2020;8(1):219-246. Disponible en: https://www.cedid.es/redis/index.php/redis/article/view/664
Chaverri J. El Implante Coclear: sus indicaciones. Acta Med Costarric [Internet]. 201860(3):132-135. doi: 10.51481/amc.v60i3.1008
Valverde M. Manual de Protocolo para la Colocación de un Implante Coclear. [Tesis de Maestría en Otorrinolaringología y Cirugía de Cabeza y Cuello]. Costa Rica:Universidad de Costa Rica;2015. Disponible en: https://repositorio.sibdi.ucr.ac.cr/items/be058260-4ded-4acd-aa6a-58fd6437e579
Sanz R, Martín E. Exploración Otoneurológica.Interpretación de las pruebas vestibulares. Abordaje práctico del paciente con vértigo y alteraciones del equilibrio. [Internet]. 1a ed. España:Amplifon Ibérica;2016.. Disponible en: https://www.researchgate.net/profile/EduardoMartinSanz/publication/319091678_Exploracion_Otoneurologica_Interpretaciondelaspruebasvestibulares/links/599016df458515b87b4a6664/ExploracionOtoneurologicaInterpretaciondelaspruebasvestibulares.pdf
Moser T, Starr A. Auditory neuropathy-neural and synaptic mechanisms. Nat Rev Neurol [Internet]. 2016;12:135-149. doi: 10.1038/nrneurol.2016.10
Rubio-Partida G, Celis-Aguilar E, Verdiales-Lugo S, Castro-Urquizo A, De la Mora-Fernández A, Coutinho-de Toledo H. Neuropatía auditiva en México: la importancia de realizar potenciales auditivos de tallo. An Orl Mex [Internet]. 2020;65(3):137-146. Disponible en: https://www.medigraphic.com/pdfs/anaotomex/aom-2020/aom203d.pdf
Shearer A, Hansen M. Auditory synaptopathy, auditory neuropathy, and cochlear implantation. Laryngoscope Investig Otolaryngol[Internet]. 2019;4(4): 429-440. doi: 10.1002/lio2.288
Rodríguez M, Algarra J. Audiología [Internet]. España: SEORL Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial;2014. Disponible en: https://es.slideshare.net/ssuser5c95731/2014-audiologiapdf
Asociación Colombiana de Otorrinolaringología, Cirugía de Cabeza y Cuello Maxilofacial y Estética Facial. Esbozo preliminar de una guía para el diagnóstico y manejo de la neuropatía auditiva [Internet]. encolombia.com. 2014 [citado 13 enero 2025]. Disponible en: https://encolombia.com/medicina/revistas-medicas/aoccc/vsuple-302/otorrino30202supl-esbozo/
Fontenot T, Giardina CK, Teagle HF, Park LR, Adunka OF, Buchman CA, et al. Clinical role of electrocochleography in children with auditory neuropathy spectrum disorder. Int J Pediatr Otorhinolaryngol [Internet]. 2017;99:120-127. doi: 10.1016/j.ijporl.2017.05.026
Rico-Romero B, Gutiérrez-Farfán I, Alonso L. Reporte de un caso con trastorno en el espectro de la neuropatía auditiva (DENA). Rev Mex Comun Audiol otoneurol Foniatr [Internet]. 2015;4(1):32-39. Disponible en: https://www.medigraphic.com/pdfs/audiologia/fon-2015/fon151e.pdf
Rance G, Starr A. Pathophysiological mechanisms and functional hearing consequences of auditory neuropathy. Brain [Internet]. 2015;138(11):3141-3158. doi:10.1093/brain/awv270
Saidia A, Ruel J, Bahloul A, Chaix B, Venail F, Wang J. Current Advances in Gene Therapies of Genetic Auditory Neuropathy Spectrum Disorder. J Clin Med [Internet]. 2023;12(3):1-19. doi: 10.3390/jcm12030738
Luryi A, Schutt C. Electrocochleography. En: Babu S, Schutt C, Bojrab D, editores. Diagnosis and Treatment of Vestibular Disorders [Internet]. Alemania: Springer;2019.113-125. Disponible en: https://doi.org/10.1007/978-3-319-97858-1_9
Lirola-Delgado A. Neuropatías Auditivas. Rev Soc Otorrinolaringol Castilla Leon Cantab La Rioja [Internet]. 2014;5(25):207-211. Disponible en: https://gredos.usal.es/bitstream/handle/10366/124557/revistaorl201425_neuropatiaauditiva?sequence=1
Do Amaral I, De Lemos P, Tenorio A, Lira KC, Gomes O. Study of cochlear microphonic potentials in auditory neuropathy. Braz J Otorhinolaryngol [Internet]. 2016;82(6):722-736. doi: 10.1016/j.bjorl.2015.11.022
Curet C. Implante coclear y neuropatía auditiva. Desincronización auditiva. Rev FASO [Internet]. 2012;19(1):58-62. Disponible en: http://faso.org.ar/revistas/2012/1/11.pdf
Vona B, Rad A, Reisinger E. The Many Faces of DFNB9: Relating OTOF Variants to Hearing Impairment. Genes [Internet]. 2020;11(12):1411. doi: 10.3390/genes11121411
Santarelli R, Rossi R, Scimemi P, Cama E, Valentino M, La Morgia Ch, et al. OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation. Brain [Internet]. 2015;138(3):563-576. doi: 10.1093/brain/awu378
Santos S, Pradillo E, Duat A, González N, Cervera J, Bartolomé M. Neuropatía Auditiva en síndrome de capos pediátrico: Evolución favorable con implante coclear. Rev Port Otorrinolaringol Cir Cabeça Pescoço [Internet]. 2019 ;57(3):121-125. doi: 10.34631/sporl.737
Anzalone L, Nuhanovic S, Olund A, Carlson M. Cochlear Implantation in Charcot-Marie-Tooth Disease: Case Report and Review of the Literature. Hindawi Case Rep Med [Internet]. 2018; 2018(1):1-3. doi: 10.1155/2018/1760978
Martínez Del Río, C. Implante coclear: Posible tratamiento para la neuropatía auditiva [Tesis de Grado en Medicina]. España: Universidad de Valladolid; 2016. Disponible en: http://uvadoc.uva.es/handle/10324/18808
Xin L, Rajam P, Hui T, Faizatul R, Tan LJ. Analysis of paediatric cochlear implant candidacy: Single centre, retrospective observational study. Med J Malaysia [Internet]. 2022;77(2):143-149. Disponible en: https://www.e-mjm.org/2022/v77n2/paediatric-cochlear-implant.pdf
Tekin A, Baelen H, Heuninck E, Bayazit Y, Mertens G,Van Rompaey V, et al. Evaluation of a Less Invasive Cochlear Implant Surgery in OPA1 Mutations Provoking Deafblindness. Genes [Internet]. 2023;14(3):627. doi: 10.3390/genes14030627
Vandana V, Sankaran P, Nagappa M, Sinha S, Taly A. Audiological findings in Infantile Refsum disease. Int J Pediatr Otorhinolaryngol [Internet]. 2015;79(8):1366-1369. doi: 10.1016/j.ijporl.2015.05.023
Manrique M, Zubicaray J, Ruiz de Erenchun I, Huarte A, Manrique-Huarte R. Guía Clínica para la indicación de implantes cocleares en la Comunidad Floral de Navarra. An Sist Sanit Navar [Internet]. 2015;38(2):289-296. Disponible en: https://scielo.isciii.es/pdf/asisna/v38n2/revision2.pdf
Zheng D, Liu X. Cochlear Implantation Outcomes in Patients with OTOF Mutations. Front Neurosci [Internet]. 2020;14(447):1-7. doi: 10.3389/fnins.2020.00447
Goswami J, Bruce I, Green K, O’Driscoll M. Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot−Marie−Tooth disease. Cochlear Implants Int [Internet]. 2013;13(3):184-187. doi: 10.1179/1754762811Y.0000000021
Stärh K, Kuechler A, Gencik M, Arnolds J, Dendy M, Lang S, et al. Cochlear Implantation in Siblings with Refsum’s Disease. An Otol Rhinol Laryngol [Internet]. 2017;126(8):611-614. doi: 10.1177/0003489417717269
Nogueira C, Meehan T, O’Donoghue G. Refsum’s Disease and Cochlear Implantation. An Otol Rhinol Laryngol [Internet]. 2014;123(6):425-427. doi:10.1177/0003489414526846
Kontorinis G, Lloyd S, Henderson L, Jayewardene-Aston D, Milward K, Bruce I, et al. Cochlear implantation in children with auditory neuropathy spectrum disorders. Cochlear Implants Int [Internet]. 2014;15(sup1):51-54. doi: 10.1179/1467010014Z.000000000157
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