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PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU

Year 2015, Volume: 5 Issue: 1, 0 - 0, 27.02.2015

Abstract

İşitsel nöropati, işitsel beyin cevabı alınamamasına karşın dış tüylü hücre fonksiyonlarının korunduğu işitme bozukluğudur. Günümüzdeki mevcut yöntemlerle patolojinin tam olarak hangi seviyede olduğunu saptamak mümkün değildir. İşitsel nöropati hem çocuk hem de erişkin yaş grubunda, çeşitli işitme seviyeleri ile görülebilmektedir. Bu nedenle Kulak Burun Boğaz Hastalıkları ve Çocuk Hastalıkları hekimlerinin bu hastalığın klinik özelliklerini akılda tutması ve gözden kaçırmaması önemlidir. Çünkü doğru tanı konulup doğru tedavi yöntemi uygulanmadığında işitmenin daha da kötüleşip hastanın psikososyal gelişiminin de kötü yönde etkilenmesine neden olabilir. Bu çalışmada, 17 yaşında işitsel nöropati tanısı koyduğumuz bir hastayı ve bu hastaya klinik yaklaşımımızı sunduk. Hastaya işitme cihazı uygulamadık, konuşma ve dil gelişimi için rehabilitasyon eğitimine yönlendirdik. Bu olgu ile işitsel nöropatinin klinik ve odyolojik tetkik özelliklerini, fizyopatolojisini ve tedavi yaklaşımını gözden geçirmeyi amaçladık.

References

  • Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy.Brain. 1996 Jun;119 ( Pt 3):741-53.
  • Tang TP, McPherson B, Yuen KC, Wong LL, Lee JS. Auditory neuropathy/auditory dys-synchrony in school children with hearing loss: frequency of occurrence. IntJ Pediatr Otorhinolaryngol. 2004 Feb;68(2):175-83.
  • Baylan M.Y, Gün R, Yorgancılar E, Topçu İ. Primer İşitsel Nöropati: Olgu Sunumu. KBB ve BBC Dergisi 19 (2):84-8, 2011.
  • Rodríguez-Ballesteros M, del Castillo FJ, Martín Y, Moreno-Pelayo MA, Morera C, Prieto F, Marco J, Morant A, Gallo-Terán J, Morales-Angulo C, Navas C, Trinidad G, Tapia MC, Moreno F, del Castillo I. Auditory neuropathy in patients carrying mutations in the otoferlin gene (OTOF). Hum Mutat. 2003 Dec;22(6):451-6.
  • Korver AM, vanZanten GA, Meuwese-Jongejeugd A, vanStraaten HL, Oudesluys- urphy AM. Auditory neuropathy in a low-risk population: a review of the literature. Int J Pediatr Otorhinolaryngol. 2012 Dec;76(12):1708-11.
  • Raveh E, Buller N, Badrana O, Attias J. Auditory neuropathy: clinical characteristics and therapeutic approach. Am J Otolaryngol. 2007 Sep-Oct;28(5):302-8.
  • Rance G, McKay C, Grayden D. Perceptual characterization of children with auditory neuropathy. EarHear. 2004 Feb;25(1):34-46.
  • Shapiro SM. Bilirubin toxicity in the developing nervous system. Pediatr Neurol. 2003 Nov;29(5):410-21.
  • Yilmaz Y, Değirmenci S, Akdaş F, Külekçi S, Ciprut A, Yüksel S, Yildiz F, Karadeniz L, Say A. Prognostic value of auditory brainstem response for neurologic outcome in patients with neonatal indirect hyperbilirubinemia. J Child Neurol. 2001 Oct;16(10):772-5.
  • Madden C, Rutter M, Hilbert L, Greinwald JH Jr, Choo DI. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg. 2002 Sep; 128(9): 1026-30.
  • Ceranić B, Luxon LM. Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy. J Neurol Neurosurg Psychiatry. 2004Apr;75(4):626-30.
  • Berlin CI, Hood L, Morlet T, Rose K, Brashears S. Auditory neuropathy/dys-synchrony: diagnosis and management. Ment Retard Dev Disabil Res Rev. 2003;9(4):225-31.
  • Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michalewski HJ. Transient deafness due to temperature-sensitive auditory neuropathy. EarHear. 1998Jun;19(3):169-79.
  • Kumar UA, Jayaram MM. Prevalence and audiological characteristics in individuals with auditory neuropathy/auditorydys-synchrony. Int J Audiol. 2006 Jun;45(6):360-6.
  • Sawada S, Mori N, Mount RJ, Harrison RV. Differential vulnerability of inner and outer hair cell systems to chronic mild hypoxia and glutamate ototoxicity: insights into the cause of auditory neuropathy. J Otolaryngol. 2001 Apr;30(2):106-14.
  • Lin CY, Chen YJ, Wu JL. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005Feb;262(2):139-41.

PRIMARY AUDITORY NEUROPATHY: CASE REPORT

Year 2015, Volume: 5 Issue: 1, 0 - 0, 27.02.2015

Abstract

Auditory neuropathy is a hearing disorder characterized by the preservation of outer hair cell function despite the absence of auditory brainstem evoked responses.  The exact site of the pathological lesion in auditory neuropathy hasn’t been demonstrated, yet. Auditory neuropathy in both adults and children may be seen with various levels of hearing loss. Therefore, ENT doctors should keep in mind the clinical features of this disease in order not to overlook the disease. Because under diagnosis and/or inappropriate treatment of the disease may worsen hearing loss and also may negatively effect the psychosocial development of the patient. In this case, we presented a 17 years old patient with auditory neuropathy. We didn’t implement ear aid but we only suggested auditory rehabilitation. We aimed to review the clinical features, audiometric findings, psychopathologic and treatment approaches to patients with auditory neuropathy.

References

  • Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy.Brain. 1996 Jun;119 ( Pt 3):741-53.
  • Tang TP, McPherson B, Yuen KC, Wong LL, Lee JS. Auditory neuropathy/auditory dys-synchrony in school children with hearing loss: frequency of occurrence. IntJ Pediatr Otorhinolaryngol. 2004 Feb;68(2):175-83.
  • Baylan M.Y, Gün R, Yorgancılar E, Topçu İ. Primer İşitsel Nöropati: Olgu Sunumu. KBB ve BBC Dergisi 19 (2):84-8, 2011.
  • Rodríguez-Ballesteros M, del Castillo FJ, Martín Y, Moreno-Pelayo MA, Morera C, Prieto F, Marco J, Morant A, Gallo-Terán J, Morales-Angulo C, Navas C, Trinidad G, Tapia MC, Moreno F, del Castillo I. Auditory neuropathy in patients carrying mutations in the otoferlin gene (OTOF). Hum Mutat. 2003 Dec;22(6):451-6.
  • Korver AM, vanZanten GA, Meuwese-Jongejeugd A, vanStraaten HL, Oudesluys- urphy AM. Auditory neuropathy in a low-risk population: a review of the literature. Int J Pediatr Otorhinolaryngol. 2012 Dec;76(12):1708-11.
  • Raveh E, Buller N, Badrana O, Attias J. Auditory neuropathy: clinical characteristics and therapeutic approach. Am J Otolaryngol. 2007 Sep-Oct;28(5):302-8.
  • Rance G, McKay C, Grayden D. Perceptual characterization of children with auditory neuropathy. EarHear. 2004 Feb;25(1):34-46.
  • Shapiro SM. Bilirubin toxicity in the developing nervous system. Pediatr Neurol. 2003 Nov;29(5):410-21.
  • Yilmaz Y, Değirmenci S, Akdaş F, Külekçi S, Ciprut A, Yüksel S, Yildiz F, Karadeniz L, Say A. Prognostic value of auditory brainstem response for neurologic outcome in patients with neonatal indirect hyperbilirubinemia. J Child Neurol. 2001 Oct;16(10):772-5.
  • Madden C, Rutter M, Hilbert L, Greinwald JH Jr, Choo DI. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg. 2002 Sep; 128(9): 1026-30.
  • Ceranić B, Luxon LM. Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy. J Neurol Neurosurg Psychiatry. 2004Apr;75(4):626-30.
  • Berlin CI, Hood L, Morlet T, Rose K, Brashears S. Auditory neuropathy/dys-synchrony: diagnosis and management. Ment Retard Dev Disabil Res Rev. 2003;9(4):225-31.
  • Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michalewski HJ. Transient deafness due to temperature-sensitive auditory neuropathy. EarHear. 1998Jun;19(3):169-79.
  • Kumar UA, Jayaram MM. Prevalence and audiological characteristics in individuals with auditory neuropathy/auditorydys-synchrony. Int J Audiol. 2006 Jun;45(6):360-6.
  • Sawada S, Mori N, Mount RJ, Harrison RV. Differential vulnerability of inner and outer hair cell systems to chronic mild hypoxia and glutamate ototoxicity: insights into the cause of auditory neuropathy. J Otolaryngol. 2001 Apr;30(2):106-14.
  • Lin CY, Chen YJ, Wu JL. Cochlear implantation in a Mandarin Chinese-speaking child with auditory neuropathy. Eur Arch Otorhinolaryngol. 2005Feb;262(2):139-41.
There are 16 citations in total.

Details

Journal Section Case Report
Authors

Murat Şereflican

Tuğçe Şimşek This is me

Veysel Yurtttaş This is me

Fatih Rüzgar This is me

Mustafa Dilek This is me

Sevil Bilir Göksügür This is me

Publication Date February 27, 2015
Published in Issue Year 2015 Volume: 5 Issue: 1

Cite

APA Şereflican, M., Şimşek, T., Yurtttaş, V., Rüzgar, F., et al. (2015). PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU. Bozok Tıp Dergisi, 5(1).
AMA Şereflican M, Şimşek T, Yurtttaş V, Rüzgar F, Dilek M, Göksügür SB. PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU. Bozok Tıp Dergisi. February 2015;5(1).
Chicago Şereflican, Murat, Tuğçe Şimşek, Veysel Yurtttaş, Fatih Rüzgar, Mustafa Dilek, and Sevil Bilir Göksügür. “PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU”. Bozok Tıp Dergisi 5, no. 1 (February 2015).
EndNote Şereflican M, Şimşek T, Yurtttaş V, Rüzgar F, Dilek M, Göksügür SB (February 1, 2015) PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU. Bozok Tıp Dergisi 5 1
IEEE M. Şereflican, T. Şimşek, V. Yurtttaş, F. Rüzgar, M. Dilek, and S. B. Göksügür, “PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU”, Bozok Tıp Dergisi, vol. 5, no. 1, 2015.
ISNAD Şereflican, Murat et al. “PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU”. Bozok Tıp Dergisi 5/1 (February 2015).
JAMA Şereflican M, Şimşek T, Yurtttaş V, Rüzgar F, Dilek M, Göksügür SB. PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU. Bozok Tıp Dergisi. 2015;5.
MLA Şereflican, Murat et al. “PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU”. Bozok Tıp Dergisi, vol. 5, no. 1, 2015.
Vancouver Şereflican M, Şimşek T, Yurtttaş V, Rüzgar F, Dilek M, Göksügür SB. PRİMER İŞİTSEL NÖROPATİ: VAKA SUNUMU. Bozok Tıp Dergisi. 2015;5(1).
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