FONKSİYONEL İŞİTME KAYBI MI İŞİTSEL NÖROPATİ SPEKTRUM BOZUKLUĞU MU? POSTLİNGUAL OLGU SUNUMU

Year 2024, Volume: 38 Issue: 1, 53 - 60, 24.05.2024

Emine Ayça Ödemişlioğlu Aydın Hande Evin Eskicioğlu Serpil Mungan Durankaya Günay Kırkım

https://doi.org/10.18614/deutip.1337930

Abstract

İşitsel nöropati spektrum bozukluğu anormal işitsel beyin sapı cevabı ve korunmuş otoakustik emisyon cevapları ile karakterize işitme siniri boyunca bozulmuş sinyal işleme ya da sinyalin iç tüylü hücrelerden işitme sinirine yetersiz iletilmesidir. İşitsel nöropati spektrum bozukluğu doğumsal olduğu gibi postlingual olarak da gelişebilir. İşitsel nöropatide karşılaşılan işitme eşiklerindeki değişimler fonksiyonel işitme kaybıyla da karışabilmektedir. Bu çalışmada işitme azlığı ve konuşmayı anlama problemi yaşayan ilk aşamada fonksiyonel işitme kaybı düşünülen ancak işitsel nöropati spektrum bozukluğu tanısı alan 64 yaşındaki olgunun ayırıcı tanıda kullanılan odyolojik değerlendirmeleri ve test sonuçları irdelenmiştir.

Keywords

İşitsel Nöropati Spektrum Bozukluğu, Postlingual İşitme Kaybı, Fonksiyonel İşitme Kaybı.

FUNCTIONAL HEARING LOSS OR AUDITORY NEUROPATHY SPECTRUM DISORDER? POSTLINGUAL CASE REPORT

Abstract

Auditory neuropathy spectrum disorder is impaired signal processing along the auditory nerve or inadequate transmission of the signal from the inner hair cells to the auditory nerve, characterized by abnormal auditory brainstem responses and preserved otoacoustic emission responses. Auditory neuropathy spectrum disorder can develop postlingually as well as congenitally. Changes in hearing thresholds encountered in auditory neuropathy can also be confused with functional hearing loss. In this study, the audiological evaluations and test results used in the differential diagnosis of a 64-year-old patient with hearing impairment and speech comprehension problems, who was thought to have functional hearing loss at the first stage but was later diagnosed with auditory neuropathy spectrum disorder, were examined.

Keywords

Auditory Neuropathy Spectrum Disorder, Postlingual Hearing Loss, Functional Hearing Loss.

References

• 1. De Siati RD, Rosenzweig F, Gersdorff G, Gregoire A, Rombaux P, Deggouj N. Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports. Journal of Clinical Medicine. 2020;9(4):1074.
• 2. Lin P-H, Hsu C-J, Lin Y-H, Lin Y-H, Yang S-Y, Yang T-H, et al. An integrative approach for pediatric auditory neuropathy spectrum disorders: revisiting etiologies and exploring the prognostic utility of auditory steady-state response. Scientific Reports. 2020;10(1).
• 3. Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain : a journal of neurology. 1996;119(3):741-53.
• 4. Berlin CI, Hood LJ, Morlet T, Wilensky D, St John P, Montgomery E, et al. Absent or elevated middle ear muscle reflexes in the presence of normal otoacoustic emissions: a universal finding in 136 cases of auditory neuropathy/dys-synchrony. Journal of the American Academy of Audiology. 2005;16(8):546-53.
• 5. Meleca JB, Stillitano G, Lee MY, Lyle W, Carol Liu YC, Anne S. Outcomes of audiometric testing in children with auditory neuropathy spectrum disorder. Int J Pediatr Otorhinolaryngol. 2020;129:109757.
• 6. Fontenot TE, 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. 2017;99:120-7.
• 7. Chandan HS, Prabhu P. Audiological changes over time in adolescents and young adults with auditory neuropathy spectrum disorder. European Archives of Oto-Rhino-Laryngology. 2015;272(7):1801-7.
• 8. Pflug C, Kiehn S, Koseki J-C, Pinnschmidt H, Müller F, Nienstedt JC, et al. Prognostic factors in non-organic hearing loss in children. International journal of audiology. 2022;61(5):400-7.
• 9. Morita S, Suzuki M, Iizuka K. Non-organic hearing loss in childhood. International Journal of Pediatric Otorhinolaryngology. 2010;74(5):441-6.
• 10. Durankaya SM. Fonksiyonel İşitme Kayıplarında Odyolojik Tanı. Kulak Burun Boğaz Baş Boyun Cerrahisi Otoloji Cilt 1. 2021:68-70.
• 11. Lin J, Staecker H. Nonorganic hearing loss. Seminars in neurology. 2006;26(3):321-30.
• 12. Kim Y, Han JJ, Oh J, Han JH, Kim MY, Jung J, et al. Audiogram Configuration, Molecular Etiology, and Outcome of Cochlear Implantation in Postlingual Auditory Neuropathy Spectrum Disorder. Otology & Neurotology. 2023:10.1097/MAO.0000000000003915.
• 13. Sarve AR, Venu HP, Shaju M. Nonorganic Hearing Loss: A Case Profile. Journal of Health and Allied Sciences NU. 2019;09(01):35-7.
• 14. Weisleder DH, Weisleder P. Functional Hearing Disorder in Children. Seminars in Pediatric Neurology. 2022;41:100956.
• 15. Starr A, Rance G. Auditory neuropathy. Handbook of clinical neurology. 2015;129:495-508.
• 16. Hayes D, Sininger YS, editors. Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder. 2008.
• 17. Xie D, Luo J, Chao X, Li J, Liu X, Fan Z, et al. Relationship Between the Ability to Detect Frequency Changes or Temporal Gaps and Speech Perception Performance in Post-lingual Cochlear Implant Users. Frontiers in Neuroscience. 2022;16:904724.
• 18. Kirkim G, Serbetcioglu B, Erdag TK, Ceryan K. The frequency of auditory neuropathy detected by universal newborn hearing screening program. International Journal of Pediatric Otorhinolaryngology. 2008;72(10):1461-9.
• 19. Vignesh SS, Jaya V, Muraleedharan A. Prevalence and Audiological Characteristics of Auditory Neuropathy Spectrum Disorder in Pediatric Population: A Retrospective Study. Indian Journal of Otolaryngology and Head & Neck Surgery. 2016;68(2):196-201.
• 20. Kumar UA, Jayaram MM. Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony. International journal of audiology. 2006;45(6):360-6.
• 21. Prabhu P, Avilala VKY, Manjula PP. Predisposing factors in individuals with late-onset auditory dys-synchrony. Asia Pacific Journal of Speech, Language and Hearing. 2012;15(1):41-50.
• 22. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR, et al. Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder. International journal of audiology. 2010;49(1):30-43.
• 23. Gardner-Berry K, Chang H, Ching TY, Hou S. Detection Rates of Cortical Auditory Evoked Potentials at Different Sensation Levels in Infants with Sensory/Neural Hearing Loss and Auditory Neuropathy Spectrum Disorder. Seminars in hearing. 2016;37(1):53-61.
• 24. Sharma A, Cardon G, Henion K, Roland P. Cortical maturation and behavioral outcomes in children with auditory neuropathy spectrum disorder. International journal of audiology. 2011;50(2):98-106.
• 25. Alvarenga KF, Amorim RB, Agostinho-Pesse RS, Costa OA, Nascimento LT, Bevilacqua MC. Speech perception and cortical auditory evoked potentials in cochlear implant users with auditory neuropathy spectrum disorders. Int J Pediatr Otorhinolaryngol. 2012;76(9):1332-8.
• 26. Rance G, McKay C, Grayden D. Perceptual characterization of children with auditory neuropathy. Ear and hearing. 2004;25(1):34-46.
• 27. Keintzel T, Raffelsberger T, Niederwanger L, Gundacker G, Rasse T. Systematic Literature Review and Early Benefit of Cochlear Implantation in Two Pediatric Auditory Neuropathy Cases. Journal of Personalized Medicine. 2023;13(5):848.
• 28. Kaga K. Auditory nerve disease and auditory neuropathy spectrum disorders. Auris Nasus Larynx. 2016;43(1):10-20.
• 29. Jeong SW, Kim LS. Auditory neuropathy spectrum disorder: predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification. Acta Otolaryngol. 2013;133(7):714-721.
• 30. Wei X, Li Y, Chen B, et al. Predicting Auditory Outcomes from Radiological Imaging in Cochlear Implant Patients with Cochlear Nerve Deficiency. Otol Neurotol. 2017;38(5):685-693.
• 31. Teagle, Holly F.B.; Roush, Patricia A.; Woodard, Jennifer S.; Hatch, Debora R.; Zdanski, Carlton J.; Buss, Emily; Buchman, Craig A. Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder. Ear and Hearing. 2010; 31(3): p 325-335.
• 32. Gardner-Berry K, Gibson WP, Sanli H. Pre-operative testing of patients with neuropathy or dys-synchrony. Hear J. 2005; 58:24–31.
• 33. Aryal, S., Sharma, Y. & Prabhu, P. A systematic review of the efficacy of cochlear implantation in adults with auditory neuropathy spectrum disorders. Egypt J Otolaryngol. 2023; 39, 177.
• 34. Walton J, Gibson WP, Sanli H, Prelog K. Predicting cochlear implant outcomes in children with auditory neuropathy. Otol Neurotol. 2008; 29:302–9.
• 35. Manchaiah VKC, Zhao F, Danesh AA, Duprey R. The genetic basis of auditory neuropathy spectrum disorder (ANSD). Int J Pediatr Otorhinolaryngol. 2011 ;75 :151-8.
• 36. Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL. Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. Int J Pediatr Otorhinolaryngol. 2015 Dec;79(12):1980-7.
• 37. Hood LJ. Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management. Otolaryngol Clin North Am. 2015 ;48(6):1027-40.
• 38. De Siati RD, Rosenzweig F, Gersdorff G, Gregoire A, Rombaux P, Deggouj N. Auditory Neuropathy Spectrum Disorders: From Diagnosis to Treatment: Literature Review and Case Reports. Journal of Clinical Medicine. 2020; 9(4):1074.