Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder^*)

References

• Amatuzzi M.G., Northrop C., Liberman M.C., Thornton A., Halpin C., . Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care unit autopsies. Arch Otolaryngol Head Neck Surg. 2001; 127:629–636.
   PubMedGoogle Scholar
• Attias J., Raveh E., . Transient deafness in young candidates for cochlear implants. Audiol Neuro-otol. 2007; 12:325–333.
   PubMed Web of Science ®Google Scholar
• Berlin C.I., Hood L.J., Cecola R.P., Jackson D.F., Szabo P., . Does type I afferent neuron dysfunction reveal itself through lack of efferent suppression?. Hear Res. 1993; 65:40–50.
   PubMed Web of Science ®Google Scholar
• Berlin C.I., Bordelon J., St John P., Wilensky D., Hurley A., . Reversing click polarity may uncover auditory neuropathy in infants. Ear Hear. 1998; 19:37–41.
   PubMed Web of Science ®Google Scholar
• Berlin C.I., Hood L.J., Goforth-Barter L., Bordelon J., . Auditory neuropathy: three time courses after early identification. ARO Abstracts. 1999; 22:169.
   Google Scholar
• Berlin C.I., Hood L.J., Morlet T., Den Z., Goforth L., . The search for auditory neuropathy patients and connexin 26 patients in schools for the deaf. ARO Abstracts. 2000; 23.
   Google Scholar
• Berlin C.I., Hood L.J., Rose K., . On renaming auditory neuropathy as auditory dys-synchrony. Audiol Today. 2001a; 13:15–17.
   Google Scholar
• Berlin C.I., Jeanfreau J., Hood L.J., Morlet T., Keats B., . Managing and renaming auditory neuropathy as part of a continuum of auditory dys-synchrony. ARO Abstracts. 2001b.; 24:137.
   Google Scholar
• Berlin C.I., Morlet T., Hood L.J., . 2008. Management of individuals with auditory neuropathy spectrum disorder. D. Hayes, Y.S. Sininger, . Identification and Management of Infants and Young Children with Auditory Neuropathy Apectrum Disorder. Aurora, CO: The Children's Hospital.
   Google Scholar
• Berlin C.I., Hood L., Morlet T., Rose K., Brashears S., . Auditory neuropathy/dys-synchrony: diagnosis and management. Ment Retard Dev Disabil Res Rev. 2003; 9:225–231.
   PubMedGoogle Scholar
• Berlin C.I., Hood L.J., Morlet T., Wilensky D., St John P., . 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. J Am Acad Audiol. 2005; 16:546–553.
   PubMedGoogle Scholar
• Buchman C.A., Roush P.A., Teagle H.F., Brown C.J., Zdanski C.J., . Auditory neuropathy characteristics in children with cochlear nerve deficiency. Ear Hear. 2006; 27:399–408.
   PubMed Web of Science ®Google Scholar
• Burkard R., Trautwein P., Salvi R., . The effects of click level, click rate, and level of background masking noise on the inferior colliculus potential (ICP) in the normal and carboplatin-treated chinchilla. J Acoust Soc Am. 1997; 102:3620–3627.
   PubMed Web of Science ®Google Scholar
• Cacace A.T., Satya-Murti S., Grimes C.T., . Frequency selectivity and temporal processing in Friedreich's ataxia. Clinical aspects in two patients. Ann Otol Rhinol Laryngol. 1983; 92:276–280.
   PubMed Web of Science ®Google Scholar
• Ceranić B., Luxon L.M., . Progressive auditory neuropathy in patients with Leber's hereditary optic neuropathy. J Neurol Neurosurg Psychiatry. 2004; 75:626–630.
   PubMed Web of Science ®Google Scholar
• Cheng X., Li L., Brashears S., Morlet T., Ng S.S., . Connexin 26 variants and auditory neuropathy/dys-synchrony among children in schools for the deaf. Am J Med Genet. 2005; 139:13–18.
   PubMed Web of Science ®Google Scholar
• Chisin R., Perlman M, Sohmer H, . Cochlear and brainstem responses in hearing loss following neonatal hyperbilirubinemia. Ann Otol Rhinol. Laryngol. 1979; 88:352–357.
   PubMed Web of Science ®Google Scholar
• Coats A.C., Martin J.L., . Human auditory nerve action potentials and brain stem evoked responses. Effects of audiogram shape and lesion locations. Arch Otolaryngol. 1977; 103:605–622.
   PubMed Web of Science ®Google Scholar
• Corley V.M., Crabbe L.S., . Auditory neuropathy and a mitochondrial disorder in a child: case study. J Am Acad Audiol. 1999; 10:484–488.
   PubMedGoogle Scholar
• Cullen J.K.Jr, Ellis M.S., Berlin C.I., Lousteau R.J., . Human acoustic nerve action potential recordings from the tympanic membrane without anesthesia. Acta Otolaryngol. 1972; 74:15–22.
   PubMed Web of Science ®Google Scholar
• Dallos P., Fakler B., . Prestin, a new type of motor protein. Nat Rev Mol Cell Biol. 2002; 3:104–111.
   PubMed Web of Science ®Google Scholar
• Dallos P., Zheng J., Cheatham M.A., . Prestin and the cochlear amplifier. J Physiol. 2006; 576:37–42.
   PubMed Web of Science ®Google Scholar
• Delmaghani S., del Castillo F.J., Michel V., Leibovici M., Aghaie A., . Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy. Nat Genet. 2006; 38:770–778.
   PubMed Web of Science ®Google Scholar
• Deltenre P., Mansbach A.L., Bozet C., Christiaens F., Barthelemy P., . Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions. Audiol. 1999; 38:187–195.
   PubMed Web of Science ®Google Scholar
• Foerst A., Beutner D., Lang-Roth R., Huttenbrink K.B., von Wedel H., . Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing loss. Int J Pediatr Otorhinolaryngol. 2006; 70:1415–1422.
   PubMed Web of Science ®Google Scholar
• Forli F., Mancuso M., Santoro A., Dotti M.T., Siciliano G., . Auditory neuropathy in a patient with mitochondrial myopathy and multiple mtDNA deletions. J Laryngol Otol. 2006; 120:888–891.
   PubMed Web of Science ®Google Scholar
• Gibson W.P., Sanli H., . Auditory neuropathy: an update. Ear Hear. 2007; 28:102S–106S.
   PubMed Web of Science ®Google Scholar
• Hall J.W.3rd, Smith S.D., Popelka G.R., . Newborn hearing screening with combined otoacoustic emissions and auditory brainstem responses. J Am Acad Audiol. 2004; 15:414–425.
   PubMedGoogle Scholar
• Hinchcliffe R., Osuntokun B.O., Adeuja A.O., . Hearing levels in Nigerian ataxic neuropathy. Audiol. 1972; 11:218–230.
   PubMedGoogle Scholar
• Kaga K., Nakamura M., Shinogami M., Tsuzuku T., Yamada K, . Auditory nerve disease of both ears revealed by auditory brainstem responses, electrocochleography and otoacoustic emissions. Scand Audiol. 1996; 25:233–238.
   PubMed Web of Science ®Google Scholar
• Kemp D.T., . Stimulated acoustic emissions from within the human auditory system. J Acoust Soc Am. 1978; 64:1386–1391.
   PubMed Web of Science ®Google Scholar
• Kiang N.Y., Liberman M.C., Sewell W.F., Guinan J.J., . Single unit clues to cochlear mechanisms. Hear Res. 1986; 22:171–182.
   PubMed Web of Science ®Google Scholar
• Konrádsson K.S., . Bilaterally preserved otoacoustic emissions in four children with profound idiopathic unilateral sensorineural hearing loss. Audiol. 1996; 35:217–227.
   PubMedGoogle Scholar
• Korzybski A., . Science and Sanity: An Introduction to Non-Aristotelian Systems and General Semantics. Connecticut: The International Non-Aristotelian Library Publishing Company1958.
   Google Scholar
• Kraus N., Ozdamar O., Stein L., Reed N., . Absent auditory brain stem response: peripheral hearing loss or brain stem dysfunction?. Laryngoscope. 1984; 94:400–406.
   PubMed Web of Science ®Google Scholar
• Kraus N., Bradlow A.R., Cheatham M.A., Cunningham J., King C.D., . Consequences of neural asynchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol. 2000; 1:33–33.
   PubMed Web of Science ®Google Scholar
• Lee J.S.M., McPherson B., Yuen K.C.P., Wong L.L.N., . Screening for auditory neuropathy in a school for hearing impaired children. Int J Pediatr Otorhinolaryngol. 2001; 61:39–46.
   PubMed Web of Science ®Google Scholar
• Loundon N., Marcolla A., Roux I., Rouillon I., Denoyelle F., . Auditory neuropathy or endocochlear hearing loss?. Otol Neurotol. 2005; 26:748–754.
   PubMed Web of Science ®Google Scholar
• McMahon C.M., Patuzzi R.B., Gibson W.P., Sanli H., . Frequency-specific electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy exist. Ear Hear. 2008; 29:314–325.
   PubMed Web of Science ®Google Scholar
• Merchant S.N., McKenna M.J., Nadol J.B.Jr, Kristiansen A.G., Tropitzsch A., . Temporal bone histopathologic and genetic studies in Mohr-Tranebjaerg syndrome (DFN-1). Otol Neurotol. 2001; 22:506–511.
   PubMed Web of Science ®Google Scholar
• Miyamoto R.T., Kirk K.I., Renshaw J., Hussain D., . Cochlear implantation in auditory neuropathy. Laryngoscope. 1999; 109:181–185.
   PubMed Web of Science ®Google Scholar
• Moore B.C., Huss M., Vickers D.A., Glasberg B.R., Alcántara J.I., . A test for the diagnosis of dead regions in the cochlea. Br J Audiol. 2000; 34:205–224.
   PubMed Web of Science ®Google Scholar
• Moore B.C., . Dead regions in the cochlea: conceptual foundations, diagnosis, and clinical applications. Ear Hear. 2004; 25:98–116.
   PubMed Web of Science ®Google Scholar
• Moser T., Strenzke N., Meyer A., Lesinski-Schiedat A., Lenarz T, . Diagnosis and therapy of auditory synaptopathy/neuropathy. HNO. 2006; 54:833–839.
   PubMed Web of Science ®Google Scholar
• Podwall A., Podwall D., Gordon T.G., Lamendola P., Gold A.P., . Unilateral auditory neuropathy: case study. J Child Neurol. 2002; 17:306–309.
   PubMed Web of Science ®Google Scholar
• Prieve B.A., Gorga M.P., Neely S.T., . Otoacoustic emissions in an adult with severe hearing loss. J Speech Hear Res. 1991; 34:379–385.
   PubMedGoogle Scholar
• Rance G., Beer D.E., Cone-Wesson B., Shepherd R.K., Dowell R.C., . Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear. 1999; 20:238–252.
   PubMed Web of Science ®Google Scholar
• Rance G., McKay C., Grayden D., . Perceptual characterization of children with auditory neuropathy. Ear Hear. 2004; 25:34–46.
   PubMed Web of Science ®Google Scholar
• Rance G., . Auditory neuropathy/dys-synchrony and its perceptual consequences. Trends Amplif. 2005; 9:1–43.
   PubMedGoogle Scholar
• Rance G., Barker E.J., Sarant J.Z., Ching T.Y., . Receptive language and speech production in children with auditory neuropathy/dyssynchrony type hearing loss. Ear Hear. 2007; 28:694–702.
   PubMed Web of Science ®Google Scholar
• Rance G., Barker E.J., . Speech perception in children with Auditory Neuropathy/Dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol. 2008; 29:179–182.
   PubMed Web of Science ®Google Scholar
• Rance G., Fava R., Baldock H., Chong A., Barker E., Corben L, Delatycki MB, . Speech perception ability in individuals with Friedreich ataxia. Brain. 2008; 131(Pt 8):2002–2012.
   PubMed Web of Science ®Google Scholar
• Rapin I., Gravel J.S., . Auditory neuropathy: a biologically inappropriate label unless acoustic nerve involvement is documented. J Am Acad Audiol. 2006; 17:147–150.
   PubMed Web of Science ®Google Scholar
• Rea P.A., Gibson W.P.R., . Evidence for surviving outer hair cell function in congenitally deaf ears. Laryngoscope. 2003; 113:230–234.
   Web of Science ®Google Scholar
• Santarelli R., Arslan E., . Electrocochleography in auditory neuropathy. Hear Res. 2002; 170:32–47.
   PubMed Web of Science ®Google Scholar
• Santarelli R., Starr A., Michalewski H.J., Arslan E, . Neural and receptor cochlear potentials obtained by transtympanic electrocochleography in auditory neuropathy. Clin Neurophysiol. 2008; 119(5):1028–1041.
   PubMed Web of Science ®Google Scholar
• Shallop J.K., Peterson A., Facer G.W., Fabry L.B., Driscoll C.L., . Cochlear implants in five cases of auditory neuropathy: postoperative findings and progress. Laryngoscope. 2001; 111:555–562.
   PubMed Web of Science ®Google Scholar
• Shivashankar N., Satishchandra P., Shashikala H.R., Gore M., . Primary auditory neuropathy—an enigma. Acta Neurol Scand. 2003; 108:130–135.
   PubMed Web of Science ®Google Scholar
• Silman S., Gelfand S.A., . The relationship between magnitude of hearing loss and acoustic reflex threshold levels. J Speech Hear Disord. 1981; 46:312–316.
   PubMedGoogle Scholar
• Sininger Y.S., . Identification of auditory neuropathy in infants and children. Sem Hear. 2002; 23:193–200.
   Google Scholar
• Starr A., Picton T.W., Sininger Y., Hood L.J., . & Berlin, C.I . Auditory neuropathy. Brain. 1996; 119:741–753.
   PubMed Web of Science ®Google Scholar
• Starr A., Sininger Y., Winter M., Derebery M.J., Oba S., . Transient deafness due to temperature-sensitive auditory neuropathy. Ear Hear. 1998; 19:169–179.
   PubMed Web of Science ®Google Scholar
• Starr A., Sininger Y.S., Pratt H., . The varieties of auditory neuropathy. J Basic Clin. Physiol Pharmacol. 2000; 11:215–230.
   PubMedGoogle Scholar
• Starr A., Michalewski H.J., Zeng F.G., Fujikawa-Brooks S., Linthicum F., . Pathology and physiology of auditory neuropathy with a novel mutation in the MPZ gene (Tyr145->Ser). Brain. 2003; 126:1604–1619.
   PubMed Web of Science ®Google Scholar
• Starr A., Zeng F.G., Michalewski H.J., Moser T., . Perspectives on auditory neuropathy: Disorders of inner hair cell, auditory nerve, and their synapseA.I. Basbaum, A. Kaneko, G.M. Shepherd, G. Westheimer, . The Senses: A Comprehensive Reference P. Dallos, D. Oertel Academic PressSan Diego; Vol 3:397–412.
   Google Scholar
• Sutton G.J., . Usefulness of acoustic reflexes still unproven in newborns and young infants. Letter to the Editor. J Amer Acad Audiol. 2007; 18:187–189.
   PubMed Web of Science ®Google Scholar
• Turner C.W., Chi S.L., Flock S., . Limiting spectral resolution in speech for listeners with sensorineural hearing loss. J Speech Lang Hear Res. 1999; 42:773–784.
   PubMed Web of Science ®Google Scholar
• Varga R., Kelley P.M., Keats B.J., Starr A., Leal S.M., . Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet. 2003; 40:45–50.
   PubMed Web of Science ®Google Scholar
• Varga R., Avenarius M.R., Kelley P.M., Keats B.J., Berlin C.I., . OTOF mutations revealed by genetic analysis of hearing loss families including a potential temperature sensitive auditory neuropathy allele. J Med Genet. 2006; 43:576–581.
   PubMed Web of Science ®Google Scholar
• Withnell R.H., . Brief report: the cochlear microphonic as an indication of outer hair cell function. Ear Hear. 2001; 22:75–77.
   PubMed Web of Science ®Google Scholar
• Worthington D.W., Peters J.F., . Quantifiable hearing and no ABR: paradox or error?. Ear Hear. 1980; 1:281–285.
   PubMed Web of Science ®Google Scholar
• Zeng F.G., Oba S., Garde S., Sininger Y., Starr A., . Temporal and speech processing deficits in auditory neuropathy. Neuroreport. 1999; 10:3429–3435.
   PubMed Web of Science ®Google Scholar
• Zeng F.G., Kong Y.Y., Michalewski H.J., Starr A., . Perceptual consequences of disrupted auditory nerve activity. J Neurophysiol. 2005; 93:3050–3063.
   PubMed Web of Science ®Google Scholar