Auditory neuropathy

Handbook of Clinical Neurology

Volumn 129, Issue , 2015, Pages 495-508

  Starr, Arnold ^a   Rance, Gary ^b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF MELBOURNE   (Australia)

Author keywords

Auditory neuropathy; Axonal; Dendritic; Inner air cells disorder; Postsynaptic; Presynaptic

Indexed keywords

AUDITORY STIMULATION; HEARING; HEARING LOSS, CENTRAL; HUMAN; PATHOPHYSIOLOGY;

ACOUSTIC STIMULATION; AUDITORY PERCEPTION; HEARING LOSS, CENTRAL; HUMANS;

EID: 84923326587     PISSN: 00729752     EISSN: 22124152     Source Type: Book Series    
DOI: 10.1016/B978-0-444-62630-1.00028-7     Document Type: Chapter

References (83)

1. Acar M., Aycan Z., Acar B., et al. Audiologic evaluation in pediatric patients with type 1 diabetes mellitus. J Pediatr Endocrinol Metab 2012, 25(5-6):503-508.
2. Amatuzzi M., Liberman M.C., Northrop C. Selective inner hair cell loss in prematurity: a temporal bone study of infants from a neonatal intensive care unit. J Assoc Res Otolaryngol 2011, 12(5):595-604.
3. Attias J., Raveh E., Aizer-Dannon A., et al. Auditory system dysfunction due to infantile thiamine deficiency: long-term auditory sequelae. Audiol Neurootol 2012, 17(5):309-320.
4. Bahmad F., Merchant S.N., Nadol J.B., et al. Otopathology in Mohr-Tranebjaerg syndrome. Laryngoscope 2007, 117(7):1202-1208.
5. Berlin C.I., Bordelon J., St John P., et al. Reversing click polarity may uncover auditory neuropathy in infants. Ear Hear 1998, 19(1):37-47.
6. Berlin C.I., Hood L.J., Morlet T., et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol 2010, 49(1):30-43.
7. Buchman C.A., Roush P.A., Teagle H.F.B., et al. Auditory neuropathy characteristics in children with cochlear nerve deficiency. Ear Hear 2006, 27:399-408.
8. Butinar D., Starr A., Zidar J., et al. Auditory nerve is affected in one of two different point mutations of the neurofilament light gene. Clin Neurophysiol 2008, 119(2):367-375.
9. Cacace A.T., Pinheiro J.M. The mitochondrial connection in auditory neuropathy. Audiol Neurootol 2011, 16(6):398-413.
10. Cacace A.T., Satya Murti S., Grimes C.T. Frequency selectivity and temporal processing in Friedreich's ataxia. Ann Otol Rhinol Laryngol 1983, 92:276-280.
11. Carlson M.L., Breen J.T., Gifford R.H., et al. Cochlear implantation in the octogenarian and nonagenarian. Otol Neurotol 2010, 31(8):1343-1349.
12. Celis-Aguilar E., Macias-Valle L., Coutinho-De Toledo H. Auditory neuropathy secondary to cryptococcal central nervous system infection in 2 immunocompromised patients. Otolaryngol Head Neck Surg 2012, 147(3):597-598.
13. Ching T., Day J., Dillon H., et al. Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at 3 years of age. Int J Audiol 2013, 52(Suppl 2):S55-S64.
14. Eggermont J.J., Odenthal D. Methods in eletrtocochleograohy. Acta Otolaryngol Suppl 1974, 316:17-24.
15. Fujikawa S., Starr A. Vestibular neuropathy accompanying auditory and peripheral neuropathies. Arch Otolaryngol Head Neck Surg 2000, 126(12):1453-1456.
16. Furman A.C., Kujawa S.G., Liberman M.C. Noise-induced cochlear neuropathy is selective for fibers with low spontaneous rates. J Neurophysiol 2013, 110(3):577-586.
17. Galambos R., Despland P.A. The auditory brainstem response (ABR) evaluates risk factors for hearing loss in the newborn. Pediatr Res 1980, 14(2):159-163.
18. Hallpike C.S., Harriman D.G., Wells C.E. A case of afferent neuropathy and deafness. J Laryngol Otol 1980, 94(8):945-964.
19. Hecox K., Galambos R. Brain stem auditory evoked responses in human infants and adults. Arch Otolaryngol 1974, 99(1):30-33.
20. Hildesheimer M., Muchnik C., Rubinstein M. Problems in interpretation of brainstem-evoked response audiometry results. Audiology 1985, 24:374-379.
21. Hood L.J. A review of objective methods of evaluating neural pathways. Laryngoscope 1999, 109:1745-1748.
22. Huang T., Santarelli R., Starr A. Mutation of OPA1 gene causes deafness by affecting function of auditory nerve terminals. Brain Res 2009, 1300:97-104.
23. Jing Z., Rutherford M.A., Takago H., et al. Disruption of the presynaptic cytomatrix protein bassoon degrades ribbon anchorage, multiquantal release, and sound encoding at the hair cell afferent synapse. J Neurosci 2013, 33(10):4456-4467.
24. Johnstone K.N., John A.B., Hall J.W., et al. Multiple benefits of personal FM system use by children with auditory processing disorder (APD). Int J Audiol 2009, 48:371-383.
25. Kaga K., Tanaka Y. Auditory brainstem response and behavioral audiometry. Developmental correlates. Arch Otolaryngol 1980, 106(9):564-566.
26. Kraus N., Ozdamar O., Stein L., et al. Absent auditory brain stem response: peripheral hearing loss or brain stem dysfunction. Laryngoscope 1984, 94(3):400-406.
27. Kraus N., Bradlow A.R., Cheatham J., et al. Consequences of neural asynchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol 2000, 1(1):33-45.
28. Licklider J.C.R. The influence of interaural phase relation upon the masking of speech by white noise. J Acoust Soc Am 1948, 20:150-159.
29. Madden C., Rutter M., Hilbert L., et al. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg 2002, 128:1026-1030.
30. Marlin S., Feldmann D., Nguyen Y., et al. Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: Deafening fever!. Biochem Biophys Res Commun 2010, 394(3):737-742.
31. Martin W.H., Pratt H., Schwegler J.W. The origin of the human auditory brain-stem response wave II. Electroencephalogr Clin Neurophysiol 1995, 96(4):357-370.
32. Mason J.C., De Michele A., Stevens C., et al. Cochlear implantation in patients with auditory neuropathy of varied etiologies. Laryngoscope 2003, 113(1):45-49.
33. Masuda T., Kaga K. Influence of aging over 10 years on auditory and vestibular functions in three patients with auditory neuropathy. Acta Otolaryngol 2011, 131(5):562-568.
34. Matsunaga T., Kanzaki J. Morphological evidence that impaired intraneural microcirculation is a possible mechanism of eighth nerve conduction block in acoustic neuromas. Eur Arch Otorhinolaryngol 2000, 257(8):412-417.
35. Merchant S.N., McKenna M.J., Nadol J.B., et al. Temporal bone histopathologic and genetic studies in Mohr-Tranebjaerg syndrome (DFN-1). Otol Neurotol 2001, 22(4):506-511.
36. Michalewski H.W., Starr A., Nguyen T.T., et al. Auditory temporal processes in normal hearing individuals and in patients with auditory neuropathy. Clin Neurophysiol 2005, 116(3):669-680.
37. Michalewski H.J., Starr A., Zeng F.G., et al. N100 cortical potentials accompanying disrupted auditory nerve activity in auditory neuropathy (AN): effects of signal intensity and continuous noise. Clin Neurophysiol 2009, 120(7):1352-1363.
38. Micheyl C., Carlyon R.P., Gutshalk A., et al. The role of auditory cortex in the formation of auditory streams. Hear Res 2007, 229:116-131.
39. Miyamoto R.T., Iler Kirk K., Renshaw J., et al. Cochlear implantation in auditory neuropathy. Laryngoscope 1999, 109:181-185.
40. Moser T., Predoehl F., Starr A. Review of hair cell synapse defects in sensorineural hearing impairment. Otol Neurotol 2013, 34(6):995-1004.
41. Nadol J.B., Adams J.C., O'Malley J.T. Temporal bone histopathology in a case of sensorineural hearing loss caused by superficial siderosis of the central nervous system and treated by cochlear implantation. Otol Neurotol 2011, 32(5):748-755.
42. Narne V.K., Vanaja C.S. Speech identification and cortical potentials in individuals with auditory neuropathy. Behav Brain Funct 2008, 4:15.
43. Norton S.J., Gorga M.P., Widen J.E., et al. Identification of neonatal hearing impairment: summary and recommendations. Ear Hear 2000, 21(5):529-535.
44. Onishi S., Davis H. Effects of duration and rise time of tone bursts on evoked V potentials. J Acoust Soc Am 1968, 44(2):582-591.
45. Orabi A.A., Mawman D., Al-Zoubi F., et al. Cochlear implant outcomes and quality of life in the elderly: Manchester experience over 13 years. Clin Otolaryngol 2006, 31(2):116-122.
46. Poissant S.F., Beaudoin F., Huang J., et al. Impact of cochlear implantation on speech understanding, depression, and loneliness in the elderly. J Otolaryngol Head Neck Surg 2008, 37(4):488-494.
47. Rance G. Auditory neuropathy/dys-synchrony and its perceptual consequences. Trends Amplif 2005, 9(1):1-433.
48. Rance G., Beer D.E., Cone-Wesson B., et al. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 1999, 20(3):238-252.
49. Rance G., Cone-Wesson B., Wunderlich J., et al. Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear 2002, 23:239-253.
50. Rance G., McKay C., Grayden D. Perceptual characterisation of children with auditory neuropathy. Ear Hear 2004, 25:34-46.
51. Rance G., Fava R., Baldock H., et al. Speech perception ability in individuals with Friedreich ataxia. Brain 2008, 131(Pt 8):2002-2012.
52. Rance G., Corben L.A., DuBourg E., et al. Successful treatment of auditory perceptual disorder in individuals with Friedreich ataxia. Neuroscience 2010, 171:552-555.
53. Rance G., Ryan M.M., Bayliss K., et al. Auditory function in children with Charcot-Marie-Tooth disease. Brain 2012, 135:1412-1422.
54. Rance G., O'Hare F., O'Leary S., et al. Auditory processing deficits in individuals with primary open-angle glaucoma. Int J Audiol 2012, 51(1):10-15.
55. Riedel H., Kollmeier B. Comparison of binaural auditory brainstem responses and the binaural difference potential evoked by chirps and clicks. Hear Res 2002, 169(1-2):85-96.
56. Rodríguez-Ballesteros M., Reynoso R., Olarte M., et al. A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum Mutat 2008, 29(6):823-831.
57. Roush P., Frymark T., Venedtiktov R., et al. Audiologic management of auditory neuropathy spectrum disorder in children: a systematic review of the literature. Am J Audiol 2011, 20:159-170.
58. Santarelli R. Information from cochlear potentials and genetic mutations helps localize the lesion site in auditory neuropathy. Genome Med 2010, 2(12):91.
59. Santarelli R., Arslan E. Electrocochleography in auditory neuropathy. Hear Res 2002, 170(1-2):32-47.
60. Santarelli R., Starr A., Michalewski H.J., et al. Neural and receptor cochlear potentials obtained by transtympanic electrocochleography in auditory neuropathy. Clin Neurophysiol 2008, 119(5):1028-1041.
61. Satya-Murti S., Wolpaw J.R., Cacace A.T., et al. Late auditory evoked potentials can occur without brain stem potentials. Electroencephalogr Clin Neurophysiol 1983, 56(4):304-308.
62. Shallop J.K. Auditory neuropathy spectrum disorder in adults and children. Seminars in Hearing 2002, 23(3):215-223.
63. Shapiro S.M. Bilirubin toxicity in the developing nervous system. Pediatr Neurol 2003, 29(5):410-421.
64. Sininger Y.S., Oba S. Patients with auditory neuropathy: Who are they and what can they hear?. Auditory Neuropathy 2001, 15-36. Singular Publishing, San Diego. Y.S. Sininger, A. Starr (Eds.).
65. Spoendlin H. Optic cochleovestibular degenerations in hereditary ataxias. II. Temporal bone pathology in two cases of Friedreich's ataxia with vestibulo-cochlear disorders. Brain 1974, 97(1):41-48.
66. Starr A., Achor J. Auditory brain stem responses in neurological disease. Arch Neurol 1975, 32(11):761-768.
67. Starr A., McPherson D., Patterson J., et al. Absence of timing cues. Brain 1991, 114(Pt 3):1157-1180.
68. Starr A., Picton T.W., Sininger Y.S., et al. Auditory neuropathy. Brain 1996, 119(3):741-753.
69. Starr A., Sininger Y., Winter M., et al. Transient deafness due to temperature-sensitive auditory neuropathy. Ear Hear 1998, 19(3):169-179.
70. Starr A., Sininger Y.S., Pratt H. The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol 2000, 11(3):215-230.
71. Starr A., Sininger Y., Nguyen T., et al. Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy. Ear Hear 2001, 22(2):91-99.
72. Starr A., Michalewski H.J., Zeng F.G., et al. Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145->Ser). Brain 2003, 126(Pt 7):1604-1619. Erratum in: Brain 2003;126(Pt 7):1718.
73. Teagle H., Roush P., Woodward J.S., et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Am J Audiol 2010, 20:159-170.
74. Trautwein P., Shallop J., Fabry L., et al. Cochlear implantation of patients with auditory neuropathy. Auditory Neuropathy 2001, 203-232. Singular Publishing, San Diego, CA. Y.S. Sininger, A. Starr (Eds.).
75. Ueda N., Kuroiwa Y. Sensorineural deafness in Guillain-Barré syndrome. Brain Nerve 2008, 60(10):1181-1186.
76. Varga R., Avenarius M.R., Kelley P.M., et al. OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele. J Med Genet 2006, 43(7):576-581.
77. Vinay, Moore B.C.J. Ten(HL)-test results and psychophysical tuning curves for subjects with auditory neuropathy. Int J Audiol 2007, 46(1):39-46.
78. Worthington D.W., Peters J.F. Quantifiable hearing and no ABR: paradox or error?. Ear Hear 1980, 1(5):281-285.
79. Wynne D.P., Zeng F.G., Bhatt S., et al. Loudness adaptation accompanying ribbon synapse and auditory nerve disorders. Brain 2013, 136(Pt 5):1626-1638.
80. Yellin M.W., Jerger J., Fifer R.C. Norms for disproportionate loss in speech intelligibility. Ear Hear 1989, 10(4):231-234.
81. Zeng F.-G., Liu S. Speech perception in individuals with auditory neuropathy. J Speech Lang Hear Res 2006, 49:36.
82. Zeng F.-G., Oba S., Starr A. Supra threshold processing deficits due to desynchronous neural activities in auditory neuropathy. Physiological and Psychophysical bases of Auditory Function 2001, 365-372. Shaker Publishing, Maastricht, Netherlands. D.J. Breebaart, A.J.M. Houstma, A. Kohlrausch (Eds.).
83. Zeng F.G., Kong Y.Y., Michalewski H.J., et al. Perceptual consequences of disrupted auditory nerve activity. J Neurophysiol 2005, 93(6):3050-3063.