Abstract
Discrepancy of normal hearing and auditory brainstem response (ABR) abnormality has been a focus of attention since the early stages of the introduction of ABR to clinical medicine. The concept of auditory neuropathy (AN) seems to be in chaos because AN should be a syndrome with various etiologies and clinical features with mutual discrepancy in neurophysiological findings of ABR and otoacoustic emission (OAE). In this situation, we have found three patients with almost no ABR and normal OAE in our child neurology clinic. Among these three patients, the pure type of AN was diagnosed only in one patient, and for the other two patients the diagnoses were “fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability” and “alternating hemiplegia of childhood (AHC). ” Thus, we would like to recommend that patients who have pure auditory nerve symptoms and are suspected of localized pathology in the auditory nerve should be named as having “auditory nerve disease (AND) ” and patients with other complicated physical symptoms should be diagnosed as “auditory neuropathy (AN) ” as the special symptom of the underlying diseases.
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References
Worthington DW, Peters JF (1980) Quantifiable hearing and no ABR: paradox or error? Ear Hear 1:281–285
Kraus N, Ozdamar O, Stein L, et al (1984) Absent auditory brain stem response: peripheral hearing loss or brain stem dysfunction? Laryngoscope 94:400–406
Starr A, Picton TW, Sininger Y, et al (1996) Auditory neuropathy. Brain 119(pt 3):741–753
Deltenre P, Mansbach AL, Bozet C, et al (1997) Auditory neuropathy: a report on three cases with early onsets and major neonatal illnesses. Electroencephalogr Clin Neurophysiol 104:17–22
Kaga K, Nakamura M, Shinogami M, et al (1996) Auditory nerve disease of both ears revealed by auditory brainstem responses, electrocochleography and otoacoustic emissions. Scand Audiol 25:233–238
Rapin I, Gravel J (2003) “Auditory neuropathy”: physiologic and pathologic evidence calls for more diagnostic specificity. Int J Pediatr Otorhinolaryngol 67:707–728
Kaga M, Kon K, Uno A, et al (2002) Auditory perception in auditory neuropathy: clinical similarity with auditory verbal agnosia. Brain Dev 24:197–202
Kaga M (1999) Language disorders in Landau-Kleffner syndrome. J Child Neurol 14: 118–122
Kaga M, Shindo M, Kaga K (2000) Long-term follow-up of auditory agnosia as a sequel of herpes encephalitis in a child. J Child Neurol 15:626–629
Kaga M (1992) Development of sound localization. Acta Paediatr Jpn 34:134–138
Berlin CI, Bordelon J, St John P, et al (1998) Reversing click polarity may uncover auditory neuropathy in infants. Ear Hear 19:37–47
Nobutoki T, Sasaki M, Fukumizu M, et al (2006) Fluctuating hearing loss, episodic headache, and stroke with platelet hyperaggregability: coexistence of auditory neuropathy and cochlear hearing loss. Brain Dev 28:55–59
Starr A, Sininger Y, Winter M, et al (1998) Transient deafness due to temperature-sensitive auditory neuropathy. Ear Hear 19:169–179
Saito Y, Sakurakawa N, Sasaki M, et al (1998) A case of alternating hemiplegia of childhood with cerebellar atrophy. Pediatr Neurol 19:65–68
Kon K, Inagaki M, Kaga M, et al (2000) Otoacoustic emission in patients with neurological disorders who have auditory brainstem response abnormality Brain Dev 22:327–335
Zeng FG, Oba S, Garde S, et al (1999) Temporal and speech processing deficits in auditory neuropathy. Neuroreport 10:3429–435
Shellhaas RA, Smith SE, O’Tool E, et al (2006) Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics 118:704–709
Sheykholeslami K, Kaga K, Kaga M (2001) An isolated and sporadic auditory neuropathy (auditory nerve disease): report of five patients. J Laryngol Otol 115:530–534
Butinar D, Starr A, J Zidar J, et al (2008) Auditory nerve is affected in one of two different point mutations of the neurofilament light gene. Clin Neurophysiol 119:367–375
Rance G, Beer DE, Cone-Wesson B, et al (1999). Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 20:238–252
Walton J, Gibson WP, Sanli H, et al (2008) Predicting cochlear implant outcomes in children with auditory neuropathy. Otol Neurotol 29:302–309
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Kaga, M., Inagaki, M., Kon, K., Uno, A., Nobutoki, T. (2009). Diagnosis of Auditory Neuropathy (AN) in Child Neurology. In: Kaga, K., Starr, A. (eds) Neuropathies of the Auditory and Vestibular Eighth Cranial Nerves. Springer, Tokyo. https://doi.org/10.1007/978-4-431-09433-3_13
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DOI: https://doi.org/10.1007/978-4-431-09433-3_13
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-09432-6
Online ISBN: 978-4-431-09433-3
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