J Am Acad Audiol 2000; 11(06): 337-347
DOI: 10.1055/s-0042-1748063
Case Report

Auditory Neuropathy: Case Study with Hyperbilirubinemia

Jeffrey L. Simmons
Boys Town National Research Hospital, Omaha, Nebraska
,
Kathryn Laudin Beauchaine
Boys Town National Research Hospital, Omaha, Nebraska
› Author Affiliations

Abstract

Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the Internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5–year period that includes audiologic data and communication development results.

Abbreviations: AN = auditory neuropathy, CI = cochlear implant, CM = cochlear microphonic, DPOAE = distortion product otoacoustic emissions, DSL = Desired Sensation Level, IHC = inner hair cell, MRI = magnetic resonance imaging, OAE = otoacoustic emission, OHC = outer hair cell, SS = standard scores, TC = total communication



Publication History

Article published online:
08 April 2022

© 2000. American Academy of Audiology. This article is published by Thieme.

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  • REFERENCES

  • Berlin CI, Hood LJ, Cecola RP, Jackson DF, Szabo P. (1993). Does type I afferent neuron dysfunction reveal itself through lack of efferent suppression? Hear Res 65:40–50.
  • Berlin CI, Bordelon J, St. John P, Wilensky D, Hurley A, Kluka E, Hood LJ. (1998). Reversing click polarity may uncover auditory neuropathy in infants. Ear Hear 19:37–47.
  • Berlin CI, Hood LJ, Goforth-Barter L, Bordelon J. (1999). Clinical application of auditory efferent studies. In: Berlin CI, ed. The Efferent Auditory System: Basic Science and Clinical Applications. San Diego: Singular, 105–124.
  • Cevette MJ, Robinette MS, Carter J, Knops JL. (1995). Otoacoustic emissions in a sudden unilateral hearing loss associated with multiple sclerosis. J Am Acad Audiol 6:197–202.
  • Chisin R, Perlman M, Sohmer H. (1979). Cochlear and brain stem responses in hearing loss following neonatal hyperbilirubinemia. Ann Otol 88:352–357.
  • Corley VM, Crabbe LS. (1999). Auditory neuropathy and a mitochondrial disorder in a child: case study. J Am Acad Audiol 10:484–488.
  • Davis H. (1976). Principles of electric response audiometry. Ann Otol Rhinol Laryngol 85(Suppl 28):62–63.
  • Davis H, Hirsch HK. (1979). Slow brainstem response for low frequency audiometry. Audiology 18:445—461.
  • Deltenre P, Mansbach AL, Bozet C, Clercx A, Hecox KE. (1997). Auditory neuropathy: a report on three cases with early onsets and major neonatal illnesses. Electroencephalogr Clin Neurophysiol 104:17–22.
  • Deltenre P, Mansbach AL, Bozet C, Chritiaens F, Barthelmy P, Paulissen D, Renglet T. (1999). Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions. Audiology 38:187–195.
  • Doyle KJ, Sininger Y, Starr A. (1998). Auditory neuropathy in childhood. Laryngoscope 108:1374–1377.
  • Gorga MP, Stelmachowicz PG, Barlow SM, Brookhouser PE. (1995). Case of recurrent, reversible, sudden, sensorineural hearing loss in a child. J Am Acad Audiol 6:163–172.
  • Gorga MP, Neely ST, Ohlrich B, Hoover B, Redner J, Peters J. (1997). From laboratory to clinic: a large scale study of distortion product otoacoustic emissions in ears with normal hearing and ears with hearing loss. Ear Hear 18:440–155.
  • Gupta AK, Mann SBS. (1998). Is auditory brainstem response a bilirubin neurotoxicity marker? Am J Otolaryngol 19:232–236.
  • Harrison RV. (1998). An animal model of auditory neuropathy. Ear Hear 19:355–361.
  • Hood LJ. (1998). Auditory neuropathy: what is it and what can we do about it? Hear J 51(8):10–18.
  • Jerger J, Ali A, Fong K, Tseng E. (1992). Otoacoustic emissions, audiometric sensitivity loss, and speech understanding: a case study. J Am Acad Audiol 3:283–286.
  • Katona G, Buki B, Zsolt F, Pytel J, Simon-Nagy E, Hirschberg J. (1993). Transitory evoked otoacoustic emission (TEOAE) in a child with profound hearing loss. Int J Pediatr Otorhinolaryngol 26:263–267.
  • Konradsson KS. (1996). Bilaterally preserved otoacoustic emissions in four children with profound idiopathic unilateral sensorineural hearing loss. Audiology 35:217–227.
  • Kraus Ν, Özdamar Ö, Stein L, Reed N. (1984). Absent auditory brain stem response: peripheral hearing loss or brain stem dysfunction? Laryngoscope 94:400–406.
  • Laccourreye L, Francois M, Tran Ba Huy E, Narcy P. (1996). Bilateral evoked otoacoustic emissions in a child with bilateral profound hearing loss. Ann Otol Rhinol Laryngol 105:286–288.
  • Lutman ME, Mason SM, Sheppard S, Gibbon KP. (1989). Differential diagnostic potential of otoacoustic emissions: a case study. Audiology 28:205–210.
  • Perlman M, Fainmesser P, Sohmer H, Tamari H, Wax Y, Pevsmer B. (1983). Auditory nerve-brainstem evoked responses in hyperbilirubinemic neonates. Pediatrics 72:658–664.
  • Prieve BA, Gorga MP, Neely ST. (1991). Otoacoustic emissions in an adult with severe hearing loss. J Speech Hear Res 34:379–385.
  • Rance G, Beer DE, Cone-Wesson B, Shepherd RK, Dowell RC, King AM, Rickards FW, Clark GM. (1999). Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 20:238–252.
  • Robinette MS, Durrant JD. (1997). Contributions of evoked otoacoustic emissions in differential diagnosis of retrocochlear disorders. In: Robinette MS, Glattke TJ, eds. Otoacoustic Emissions: Clinical Applications. New York: Thieme, 205–232.
  • Ross M, Lerman J. (1971). Word Intelligibility by Picture Identification—WIPI. St. Louis: Auditec.
  • Seewald RC, Cornelisse LE, Ramji KV, Sinclair ST, Moodie KS, Jamieson DG. (1997). DSL υ 4.1 for Windows: A Software Implementation of the Desired Sensation Level (DSL [i/o]) Method for fitting linear gain and Wide-Dynamic-Range Compression Hearing Instruments. London, ON: Hearing Health Care Research Unit.
  • Sininger YS. (1998, February). Auditory Neuropathy. Presentation at the 28th Annual Mid-South Conference on Communicative Disorders, Memphis, TN.
  • Sininger YS, Starr A. (1997, April). Auditory neuropathy in children. American Speech-Language-Hearing Association Special Interest Divisions, Hearing and Hearing Disorders in Childhood 7(1):6–11.
  • Sininger YS, Hood LJ, Starr A, Berlin CI, Picton TW. (1995). Hearing loss due to auditory neuropathy. Audiology Today 7(2): 10–13.
  • Sininger YS, Trautwein PG, Shallop JK, Fabry LB, Starr A. (1999). Electrical activation of the auditory nerve in patients with auditory neuropathy [on-line]. ARO Abstracts. http://www.aro.org/archives/1999/675.html.
  • Starr A, McPherson D, Patterson J, Don M, Luxford W, Shannon R, Sininger Y, Tonakawa L, Waring M. (1991). Absence of both auditory evoked potentials and auditory percepts dependent on timing cues. Brain 114:1157–1180.
  • Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. (1996). Auditory neuropathy. Brain 119:741–753.
  • Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michaelewski HJ. (1998). Transient deafness due to temperature-sensitive auditory neuropathy. Ear Hear 19:169–179.
  • Vohr BR, Lester B, Rapisardi G, O'Dea C, Brown L, Puecker M, Cashore W, Oh W. (1989). Abnormal brain-stem function (brain-stem auditory evoked response) correlates with acoustic cry features in term infants with hyperbilirubinemia. J Pediatr 115:303–308.
  • Widen JE, Ferraro JA, Trouba SE. (1995). Progressive neural hearing impairment: case report. J Am Acad Audiol 6:217–224.
  • Worthington D, Peters J. (1980). Quantifiable hearing and no ABR: paradox or error? Ear Hear 1:281–285.
  • Yellin MW, Jerger J, Fifer RC. (1989). Norms for disproportionate loss in speech intelligibility. Ear Hear 10:231–234.
  • Zeng F-G, Oba S, Garde S, Sininger Y, Starr A. (1999). Temporal and speech processing deficits in auditory neuropathy. Neuroreport 10:3429–3435.