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Original Articles

Prevalence, risk factors, and audiological characteristics of auditory neuropathy

ORCID Icon, , &
Pages 1018-1026 | Received 27 May 2021, Accepted 30 Nov 2021, Published online: 20 Dec 2021

References

  • Al-Abduljawad, K. A., and S. M. Zakzouk. 2003. “The Prevalence of Sensorineural Hearing Loss among Saudi Children.” International Congress Series 1240: 199–204. doi:10.1016/S0531-5131(03)00913-0.
  • Alkahtani, R., D. Rowan, N. Kattan, and N. A. Alwan. 2019. “Age of Identification of Sensorineural Hearing Loss and Characteristics of affected children: Findings from two cross-sectional studies in Saudi Arabia .” International Journal of Pediatric Otorhinolaryngology 122: 27–34. doi:10.1016/j.ijporl.2019.03.019.
  • Almazroua, A. M., L. Alsughayer, R. Ababtain, Y. Al-Shawi, and A. A. Hagr. 2020. “The Association between Consanguineous Marriage and Offspring with Congenital Hearing Loss.” Annals of Saudi Medicine 40 (6): 456–461. doi:10.5144/0256-4947.2020.456.
  • Al-Sayed, A. A., and A. AlSanosi. 2017. “Cochlear Implants in Children: A Cross-Sectional Investigation on the Influence of Geographic Location in Saudi Arabia.” Journal of Family & Community Medicine 24 (2): 118–121. doi:10.4103/jfcm.JFCM_142_15.
  • Alshawi, Y. A., N. Al-Gazlan, F. Alrawaf, and F. Almuhawas. 2019. “Value of Newborn Hearing Screening on Early Intervention in the Saudi Population and Review of International Records.” Cureus 11 (10): e5990.
  • Alyami, H., M. Soer, A. Swanepoel, and L. Pottas. 2016. “Deaf or Hard of Hearing Children in Saudi Arabia: Status of Early Intervention Services.” International Journal of Pediatric Otorhinolaryngology 86: 142–149. doi:10.1016/j.ijporl.2016.04.010.
  • Alzhrani, F., M. Yousef, F. Almuhawas, and H. Almutawa. 2019. “Auditory and Speech Performance in Cochlear Implanted ANSD Children.” Acta Oto-Laryngologica 139 (3): 279–283.
  • Barreira-Nielsen, C., E. M. Fitzpatrick, and J. Whittingham. 2016. “Characteristics and Outcomes of Children with Auditory Neuropathy Spectrum Disorder.” Canadian Journal of Speech-Language Pathology and Audiology 40: 67–79.
  • Berg, A. L., J. B. Spitzer, H. M. Towers, C. Bartosiewicz, and B. E. Diamond. 2005. “Newborn Hearing Screening in the NICU: profile of Failed Auditory Brainstem Response/Passed Otoacoustic Emission.” Pediatrics 116 (4): 933–938. doi:10.1542/peds.2004-2806.
  • Berlin, C. I., J. Bordelon, P. St John, D. Wilensky, A. Hurley, E. Kluka, and L. J. Hood. 1998. “Reversing Click Polarity May Uncover Auditory Neuropathy in Infants.” Ear Hear 19 (1): 37–47.
  • Berlin, Charles I., Linda J. Hood, Thierry Morlet, Diane Wilensky, Li Li, Kelly Rose Mattingly, Jennifer Taylor-Jeanfreau, et al. 2010. “Multi-Site Diagnosis and Management of 260 Patients with Auditory Neuropathy/Dys-Synchrony (Auditory Neuropathy Spectrum Disorder).” International Journal of Audiology 49 (1): 30–43. doi:10.3109/14992020903160892.
  • Beutner, D., A. Foerst, R. Lang-Roth, H. von Wedel, and M. Walger. 2007. “Risk Factors for Auditory Neuropathy/Auditory Synaptopathy.” Journal for Oto-Rhino-Laryngology 69 (4): 239–244.
  • Breneman, A. I., R. H. Gifford, and M. D. Dejong. 2012. “Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder: Long-Term Outcomes.” Journal of the American Academy of Audiology 23 (1): 5–17.
  • Bruijnzeel, Hanneke, Aren Bezdjian, Anke Lesinski-Schiedat, Angelika Illg, Konstance Tzifa, Luisa Monteiro, Antonio Della Volpe, Wilko Grolman, and Vedat Topsakal. 2017. “Evaluation of Pediatric Cochlear Implant Care throughout Europe: Is European Pediatric Cochlear Implant Care Performed according to Guidelines?” Cochlear Implants International 18 (6): 287–296. doi:10.1080/14670100.2017.1375238.
  • Budenz, C. L., S. A. Telian, C. Arnedt, K. Starr, H. A. Arts, H. K. El-Kashlan, and T. A. Zwolan. 2013. “Outcomes of Cochlear Implantation in Children with Isolated Auditory Neuropathy versus Cochlear Hearing Loss.” Otology & Neurotology 34 (3): 477–483. doi:10.1097/MAO.0b013e3182877741.
  • Buss, E., R. F. Labadie, C. J. Brown, A. J. Gross, J. H. Grose, and H. C. Pillsbury. 2002. “Outcome of Cochlear Implantation in Pediatric Auditory Neuropathy.” Otology & Neurotology 23 (3): 328–332.
  • Ching, Teresa Y. C., Julia Day, Harvey Dillon, Kirsty Gardner-Berry, Sanna Hou, Mark Seeto, Angela Wong, and Vicky Zhang. 2013. “Impact of the Presence of Auditory Neuropathy Spectrum Disorder (ANSD) on Outcomes of Children at Three Years of Age.” International Journal of Audiology 52 (Suppl 2): S55–S64. doi:10.3109/14992027.2013.796532.
  • Chisin, R., M. Perlman, and H. Sohmer. 1979. “Cochlear and Brain Stem Responses in Hearing Loss following Neonatal Hyperbilirubinemia.” The Annals of Otology, Rhinology, and Laryngology 88 (3 Pt 1): 352–357.
  • Corley, V. M., and L. S. Crabbe. 1999. “Auditory Neuropathy and a Mitochondrial Disorder in a Child: case Study.” J Am Acad Audiol 10 (9): 484–488.
  • Deltenre, P., A. L. Mansbach, C. Bozet, F. Christiaens, P. Barthelemy, D. Paulissen, and T. Renglet. 1999. “Auditory Neuropathy with Preserved Cochlear Microphonics and Secondary Loss of Otoacoustic Emissions.” Audiology 38 (4): 187–195.
  • Dowley, A. C., W. P. Whitehouse, S. M. Mason, Y. Cope, J. Grant, and K. P. Gibbin. 2009. “Auditory Neuropathy: Unexpectedly Common in a Screened Newborn Population.” Developmental Medicine & Child Neurology 51 (8): 642–646. doi:10.1111/j.1469-8749.2009.03298.x.
  • El Mouzan, M. I., A. A. Al Salloum, A. S. Al Herbish, M. M. Qurachi, and A. A. Al Omar. 2008. “Consanguinity and Major Genetic Disorders in Saudi Children: A Community-Based Cross-Sectional Study.” Annals of Saudi Medicine 28 (3): 169–173.
  • Fernandes, N. F., M. Morettin, E. H. Yamaguti, O. A. Costa, and M. C. Bevilacqua. 2015. “Performance of Hearing Skills in Children with Auditory Neuropathy Spectrum Disorder Using Cochlear Implant: A Systematic Review.” Brazilian Journal of Otorhinolaryngology 81 (1): 85–96.
  • Fitzpatrick, E. M., J. Ham, and J. Whittingham. 2015. “Pediatric Cochlear Implantation: Why Do Children Receive Implants Late?” Ear and Hearing 36 (6): 688–694.
  • Foerst, A., D. Beutner, R. Lang-Roth, K. B. Huttenbrink, H. von Wedel, and M. Walger. 2006. “Prevalence of Auditory Neuropathy/Synaptopathy in a Population of Children with Profound Hearing Loss.” International Journal of Pediatric Otorhinolaryngology 70 (8): 1415–1422. doi:10.1016/j.ijporl.2006.02.010.
  • Harrison, R. V., K. A. Gordon, B. C. Papsin, J. Negandhi, and A. L. James. 2015. “Auditory Neuropathy Spectrum Disorder (ANSD) and Cochlear Implantation.” International Journal of Pediatric Otorhinolaryngology 79 (12): 1980–1987. doi:10.1016/j.ijporl.2015.10.006.
  • Joint Committee on Infant Hearing. 2019. “Year 2019 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs.” Journal of Early Hearing Detection and Intervention 4 (2): 1–44.
  • Kontorinis, Georgios, Simon K. W. Lloyd, Lise Henderson, Deanne Jayewardene-Aston, Kerri Milward, Iain A. Bruce, Martin O'Driscoll, Kevin Green, and Simon R. M. Freeman. 2014. “Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorders.” Cochlear Implants International 15 (Suppl 1): S51–S54. doi:10.1179/1467010014Z.000000000157.
  • Kraus, N., A. R. Bradlow, M. A. Cheatham, J. Cunningham, C. D. King, D. B. Koch, T. G. Nicol, T. J. Mcgee, L. K. Stein, and B. A. Wright. 2000. “Consequences of Neural Asynchrony: A Case of Auditory Neuropathy.” Journal of the Association for Research in Otolaryngology 1 (1): 33–45. doi:10.1007/s101620010004.
  • Madden, C., M. Rutter, L. Hilbert, J. H. J. Greinwald, and D. I. Choo. 2002. “Clinical and Audiological Features in Auditory Neuropathy.” Archives of Otolaryngology-Head & Neck Surgery 128 (9): 1026–1030.
  • Manchaiah, V. K., F. Zhao, A. A. Danesh, and R. Duprey. 2011. “The Genetic Basis of Auditory Neuropathy Spectrum Disorder (ANSD).” International Journal of Pediatric Otorhinolaryngology 75 (2): 151–158.
  • Maris, M.,. C. Venstermans, and A. N. Boudewyns. 2011. “Auditory Neuropathy/Dyssynchrony as a Cause of Failed Neonatal Hearing Screening.” International Journal of Pediatric Otorhinolaryngology 75 (7): 973–975.
  • Marlin, Sandrine, Delphine Feldmann, Yann Nguyen, Isabelle Rouillon, Natalie Loundon, Laurence Jonard, Crystel Bonnet, et al. 2010. “Temperature-Sensitive Auditory Neuropathy Associated with an Otoferlin Mutation: Deafening Fever!” Biochemical and Biophysical Research Communications 394 (3): 737–742. doi:10.1016/j.bbrc.2010.03.062.
  • Meleca, J. B., G. Stillitano, M. Y. Lee, W. Lyle, Y. C. Carol Liu, and S. Anne. 2020. “Outcomes of Audiometric Testing in Children with Auditory Neuropathy Spectrum Disorder.” International Journal of Pediatric Otorhinolaryngology 129: 109757. doi:10.1016/j.ijporl.2019.109757.
  • Mulwafu, W., H. Kuper, and R. J. Ensink. 2016. “Prevalence and Causes of Hearing Impairment in Africa.” Tropical Medicine & International Health 21 (2): 158–165.
  • Ohl, C., L. Dornier, C. Czajka, J. C. Chobaut, and L. Tavernier. 2009. “Newborn Hearing Screening on Infants at Risk.” International Journal of Pediatric Otorhinolaryngology 73 (12): 1691–1695. doi:10.1016/j.ijporl.2009.08.027.
  • Olds, C., and J. S. Oghalai. 2015. “Audiologic Impairment Associated with Bilirubin-Induced Neurologic Damage.” Seminars in Fetal & Neonatal Medicine 20 (1): 42–46.
  • Olusanya, B. O. 2012. “Neonatal Hearing Screening and Intervention in Resource-Limited Settings: An Overview.” Archives of Disease in Childhood 97 (7): 654–659. doi:10.1136/archdischild-2012-301786.
  • Pearce, W., and R. L. Martin. 2009. “On Auditory Neuropathy, Aka Auditory Neuropathy Spectrum.” Hear J 6 (2): 38–39.
  • Psarommatis, I., C. Voudouris, I. Kapetanakis, F. Athanasiadi, and K. Douros. 2017. “Recovery of Abnormal ABR in Neonates and Infants at Risk of Hearing Loss.” International Journal of Otolaryngology 2017: 7912127. doi:10.1155/2017/7912127.
  • Quar, T. K., C. Umat, and Y. Y. Chew. 2019. “The Effects of Manufacturer's Prefit and Real-Ear Fitting on the Predicted Speech Perception of Children with Severe to Profound Hearing Loss.” Journal of the American Academy of Audiology 30 (5): 346–356.
  • Rance, G. 2005. “Auditory Neuropathy/Dys-Synchrony and Its Perceptual Consequences.” Trends in Amplification 9 (1): 1–43. doi:10.1177/108471380500900102.
  • Rance, G., and E. J. Barker. 2009. “Speech and Language Outcomes in Children with Auditory Neuropathy/Dys-Synchrony Managed with Either Cochlear Implants or Hearing Aids.” International Journal of Audiology 48 (6): 313–320. doi:10.1080/14992020802665959.
  • Rance, G., and A. Starr. 2015. “Pathophysiological Mechanisms and Functional Hearing Consequences of Auditory Neuropathy.” Brain: a Journal of Neurology 138 (Pt 11): 3141–3158.
  • Rance, G., E. Barker, M. Mok, R. Dowell, A. Rincon, and R. Garratt. 2007. “Speech Perception in Noise for Children with Auditory Neuropathy/Dys-Synchrony Type Hearing Loss.” Ear and Hearing 28 (3): 351–360.
  • Rance, G., D. E. Beer, B. Cone-Wesson, R. K. Shepherd, R. C. Dowell, A. M. King, F. W. Rickards, and G. M. Clark. 1999. “Clinical Findings for a Group of Infants and Young Children with Auditory Neuropathy.” Ear and Hearing 20 (3): 238–252.
  • Rodríguez-Ballesteros, Montserrat, Francisco J. del Castillo, Yolanda Martín, Miguel A. Moreno-Pelayo, Constantino Morera, Félix Prieto, Jaime Marco, et al. 2003. “Auditory Neuropathy in Patients Carrying Mutations in the Otoferlin Gene (OTOF).” Human Mutation 22 (6): 451–456. doi:10.1002/humu.10274.
  • Roush, P., T. Frymark, R. Venediktov, and B. Wang. 2011. “Audiologic Management of Auditory Neuropathy Spectrum Disorder in Children: A Systematic Review of the Literature.” American Journal of Audiology 20 (2): 159–170.
  • Saluja, S.,. A. Agarwal, N. Kler, and S. Amin. 2010. “Auditory Neuropathy Spectrum Disorder in Late Preterm and Term Infants with Severe Jaundice.” International Journal of Pediatric Otorhinolaryngology 74 (11): 1292–1297.
  • Santarelli, R. 2010. “Information from Cochlear Potentials and Genetic Mutations Helps Localize the Lesion Site in Auditory Neuropathy.” Genome Medicine 2 (12): 91. doi:10.1186/gm212.
  • Sarankumar, T., S. V. Arumugam, S. Goyal, N. Chauhan, A. Kumari, and M. Kameswaran. 2018. “Outcomes of Cochlear Implantation in Auditory Neuropathy Spectrum Disorder and the Role of Cortical Auditory Evoked Potentials in Benefit Evaluation.” Turkish Archives of Otorhinolaryngology 56 (1): 15–20.
  • Shallop, J. K., A. Peterson, G. W. Facer, L. B. Fabry, and C. L. Driscoll. 2001. “Cochlear Implants in Five Cases of Auditory Neuropathy: postoperative Findings and Progress.” The Laryngoscope 111 (4 Pt 1): 555–562.
  • Shapiro, S. M., and H. Nakamura. 2001. “Bilirubin and the Auditory System.” Journal of Perinatology, 21 (Suppl 1): S52–S55. discussion S59–S62.
  • Sininger, Y. S. 2002. “Identification of Auditory Neuropathy in Infants and Children.” Seminars in Hearing 23 (3): 193–200. doi:10.1055/s-2002-34456.
  • Starr, A., and G. Rance. 2015. Auditory Neuropathy Handbook of Clinical Neurology. Vol. 129, 495–508. UK: Elsevier.
  • Starr, A., T. W. Picton, Y. Sininger, L. J. Hood, and C. I. Berlin. 1996. “Auditory Neuropathy.” Brain 119 (3): 741–753. doi:10.1093/brain/119.3.741.
  • Starr, A., Y. S. Sininger, and H. Pratt. 2000. “The Varieties of Auditory Neuropathy.” Journal of Basic and Clinical Physiology and Pharmacology 11 (3): 215–230.
  • Starr, A., Y. Sininger, M. Winter, M. J. Derebery, S. Oba, and H. J. Michalewski. 1998. “Transient Deafness Due to Temperature-Sensitive Auditory Neuropathy.” Ear Hear 19 (3): 169–179.
  • Swanepoel, D. W., L. Johl, and D. Pienaar. 2013. “Childhood Hearing Loss and Risk Profile in a South African Population.” International Journal of Pediatric Otorhinolaryngology 77 (3): 394–398. doi:10.1016/j.ijporl.2012.11.034.
  • Talaat, H. S., A. H. Kabel, H. Samy, and M. Elbadry. 2009. “Prevalence of Auditory Neuropathy (an) among Infants and Young Children with Severe to Profound Hearing Loss.” International Journal of Pediatric Otorhinolaryngology 73 (7): 937–939. doi:10.1016/j.ijporl.2009.03.009.
  • Teagle, H. F., P. A. Roush, J. S. Woodard, D. R. Hatch, C. J. Zdanski, E. Buss, and C. A. Buchman. 2010. “Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder.” Ear and Hearing 31 (3): 325–335.
  • Trautwein, P., Y. S. Sininger, and R. Nelson. 2000. “Cochlear Implantation of Auditory Neuropathy.” Journal of the American Academy of Audiology 11 (6): 309–315.
  • Varga, R., M. R. Avenarius, P. M. Kelley, B. J. Keats, C. I. Berlin, L. J. Hood, T. G. Morlet, et al. 2006. “OTOF Mutations Revealed by Genetic Analysis of Hearing Loss Families Including a Potential Temperature Sensitive Auditory Neuropathy allele.” J Med Genet 43 (7): 576–581. doi:10.1136/jmg.2005.038612.
  • Varga, R., P. M. Kelley, B. J. Keats, A. Starr, S. M. Leal, E. Cohn, and W. J. Kimberling. 2003. “Non-Syndromic Recessive Auditory Neuropathy is the Result of Mutations in the Otoferlin (OTOF) Gene.” Journal of Medical Genetics 40 (1): 45–50.
  • Vlastarakos, Petros V., Thomas P. Nikolopoulos, Evangelia Tavoulari, George Papacharalambous, and Stavros Korres. 2008. “Auditory Neuropathy: Endocochlear Lesion or Temporal Processing Impairment? Implications for Diagnosis and Management.” International Journal of Pediatric Otorhinolaryngology 72 (8): 1135–1150. doi:10.1016/j.ijporl.2008.04.004.
  • Walker, E., R. McCreery, M. Spratford, and P. Roush. 2016. “Children with Auditory Neuropathy Spectrum Disorder Fitted with Hearing Aids Applying the American Academy of Audiology Pediatric Amplification Guideline: Current Practice and Outcomes.” Journal of the American Academy of Audiology 27 (03): 204–218. doi:10.3766/jaaa.15050.
  • Wang, Da-Yong, Yi-Chen Wang, Dominique Weil, Ya-Li Zhao, Shao-Qi Rao, Liang Zong, Yu-Bin Ji, et al. 2010. “Screening Mutations of OTOF Gene in Chinese Patients with Auditory Neuropathy, Including a Familial Case of Temperature-Sensitive Auditory Neuropathy.” BMC Medical Genetics 11: 79. doi:10.1186/1471-2350-11-79.
  • Wynne, D. P., F. G. Zeng, S. Bhatt, H. J. Michalewski, A. Dimitrijevic, and A. Starr. 2013. “Loudness Adaptation Accompanying Ribbon Synapse and Auditory Nerve Disorders.” Brain 136 (Pt 5): 1626–1638.
  • Yasunaga, S., M. Grati, M. Cohen-Salmon, A. El-Amraoui, M. Mustapha, N. Salem, E. El-Zir, J. Loiselet, and C. Petit. 1999. “A Mutation in OTOF, Encoding Otoferlin, a FER-1-like Protein, Causes DFNB9, a Nonsyndromic Form of Deafness.” Nature Genetics 21 (4): 363–369. doi:10.1038/7693.
  • Young, N. M., B. K. Reilly, and L. Burke. 2011. “Limitations of Universal Newborn Hearing Screening in Early Identification of Pediatric Cochlear Implant Candidates.” Archives of Otolaryngology-Head & Neck Surgery 137 (3): 230–234.
  • Zakzouk, S. M., K. A. Fadle, and F. H. Al Anazy. 1995. “Familial Hereditary Progressive Sensorineural Hearing Loss among Saudi Population.” International Journal of Pediatric Otorhinolaryngology 32 (3): 247–255.
  • Zeng, F. G., S. Oba, S. Garde, Y. Sininger, and A. Starr. 1999. “Temporal and Speech Processing Deficits in Auditory Neuropathy.” Neuroreport 10 (16): 3429–3435.
  • Zhang, Qiujing, Lan Lan, Wei Shi, Lan Yu, Lin-Yi Xie, Fen Xiong, Cui Zhao, et al. 2016. “Temperature Sensitive Auditory Neuropathy.” Hearing Research 335: 53–63. doi:10.1016/j.heares.2016.01.008.

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