Clinical Findings for a Group of Infants and Young Children with Auditory Neuropathy

Rance, Gary; Beer, David E.; Cone‐Wesson, Barbara; Shepherd, Robert K.; Dowell, Richard C.; King, Alison; Rickards, Field; Clark, Graeme M.

doi:10.1097/00003446-199906000-00006

Cited by 420 publications

(449 citation statements)

References 67 publications

(59 reference statements)

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“…The finding was surprising since selective inner hair cell loss is an uncommon pathology in both the human and animal literature. The finding was also intriguing because all three cases were preterm babies and because there are many reports in the literature of an association between AN and prematurity (Rance et al 1999;Madden et al 2002;Beutner et al 2007;Xoinis et al 2007).…”

Section: Introductionmentioning

confidence: 90%

“…This clinical condition has been termed auditory neuropathy (AN;Starr et al 1996Starr et al , 2000, even though it could logically arise from either damage/degeneration of the cochlear nerve or selective damage/degeneration of the inner hair cells, which provide the exclusive synaptic drive to 95% of the cochlear nerve fibers (Spoendlin 1972). Depending on the size and nature of the population sampled, estimates of the prevalence of AN range anywhere from 5% to 15% of those with sensorineural hearing loss (Rance et al 1999;D'Agostino and Austin 2004).…”

Section: Introductionmentioning

confidence: 99%

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Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

Amatuzzi

Liberman

Northrop

2011

JARO

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Premature birth is a well-known risk factor for sensorineural hearing loss in general and auditory neuropathy in particular. However, relatively little is known about the underlying causes, in part because there are so few relevant histopathological studies. Here, we report on the analysis of hair cell loss patterns in 54 temporal bones from premature infants and a control group of 46 bones from full-term infants, all of whom spent time in the neonatal intensive care unit at the Hospital de Niños in San Jose, Costa Rica, between 1977 and 1993. The prevalence of significant hair cell loss was higher in the preterm group than the full-term group (41% vs. 28%, respectively). The most striking finding was the frequency of selective inner hair cell loss, an extremely rare histopathological pattern, in the preterm vs. the full-term babies (27% vs. 3%, respectively). The findings suggest that a common cause of non-genetic auditory neuropathy is selective loss of inner hair cells rather than primary damage to the cochlear nerve.

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Section: Introductionmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

Amatuzzi

Liberman

Northrop

2011

JARO

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“…These risk factors include positive family history, hyperbilirubinemia, exposure to ototoxic medications, hypoxia, birth asphyxia and intracranial hemorrhages, including intraventricular hemorrhage. [2][3][4][5][6][7] The exact anatomical location of abnormality or injury and the pathophysiology of AN are poorly understood. As such, there are no specific risk factors to distinguish AN from cochlear hearing loss.…”

Section: Introductionmentioning

confidence: 99%

Extremely low birth weight infants are at high risk for auditory neuropathy

et al. 2007

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Auditory neuropathy (AN) is a condition in which transmission of sound to the brain is abnormal. This is reflected as an electrophysiologic profile of normal otoacoustic emissions (OAE), with abnormal auditory brainstem evoked responses (ABR). Functionally speech perception is impaired and management strategies remain controversial. AN can be missed if high-risk newborns are screened for hearing loss with only OAE testing. The rate of sensorineural hearing loss (SNHL) in high-risk nursery infants is 10 times greater compared with normal term newborns. Therefore, we hypothesize that infants from the neonatal intensive care unit (NICU) are at significantly higher risk for AN than normal term infants.Objective: The objective of this study is to establish a prevalence rate and characterize risk factors for NICU graduates who demonstrate the AN electrophysiologic profile.Study Design: This retrospective study examined infants admitted to the NICU at Kapi'olani Medical Center for Women and Children in Honolulu, HI from 1999 through 2003. Infants were screened with automated ABR. Diagnostic testing and OAE were performed before discharge if the ABR was abnormal. Hospital courses of 24 AN, 71 SNHL and 95 gestational age (GA)-matched control infants with normal hearing were reviewed.Result: With a SNHL prevalence of 16.7/1000, the rate for AN was 5.6/1000 NICU infants. Compared to infants with SNHL, infants with AN were significantly younger (GA 28.3±4.8 AN vs 32.9±5.2 weeks SNHL, P<0.0001) and smaller (BW 1318±894 AN vs 1968±1006 g SNHL).Nearly two-thirds of the AN infants were ELBW and had significantly longer hospital stays compared to SNHL infants of the same birth weight group. Exposure to furosemide, aminoglycosides, vancomycin or dexamethasone was associated with increased AN but not SNHL. Peak bilirubin level correlated with SNHL but not AN.Conclusion: Low birth weight NICU infants are at significant risk for AN. ELBW infants are at significantly higher risk for both AN and SNHL.Infants admitted to the NICU should be routinely screened by automated ABR and if abnormal, further evaluation should be started before hospital discharge. Early identification of AN will result in better understanding of this disorder and lead to the development of appropriate intervention strategies.

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“…High precision in action potential timing is also the basis for accurate processing of interaural time differences (ITD) (Finlayson and Caspary 1991;Batra et al 1997;Tollin and Yin 2005) that might potentially be impaired in the presently investigated children with an auditory discrimination deficit. Similar impairments are also seen in patients suffering from AN (Starr et al 1996;Rance et al 1999). Other investigations also emphasize reduced speed of neuronal transmission causing deficits in temporal processing, for example, temporal resolution, detection of amplitude modulation, and temporal aspects of frequency discrimination (Rance et al 2004;Zeng et al 2005).…”

Section: Auditory Impairmentsmentioning

confidence: 61%

Auditory Processing Disorders with and without Central Auditory Discrimination Deficits

Ludwig

Fuchs

Kruse

et al. 2014

JARO

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Auditory processing disorder (APD) is defined as a processing deficit in the auditory modality and spans multiple processes. To date, APD diagnosis is mostly based on the utilization of speech material. Adequate nonspeech tests that allow differentiation between an actual central hearing disorder and related disorders such as specific language impairments are still not adequately available. In the present study, 84 children between 6 and 17 years of age (clinical group), referred to three audiological centers for APD diagnosis, were evaluated with standard audiological tests and additional auditory discrimination tests. Latter tests assessed the processing of basic acoustic features at two different stages of the ascending central auditory system: (1) auditory brainstem processing was evaluated by quantifying interaural frequency, level, and signal duration discrimination (interaural tests). (2) Diencephalic/telencephalic processing was assessed by varying the same acoustic parameters (plus signals with sinusoidal amplitude modulation), but presenting the test signals in conjunction with noise pulses to the contralateral ear (dichotic signal/ noise tests). Data of children in the clinical group were referenced to normative data obtained from more than 300 normally developing healthy school children. The results in the audiological and the discrimination tests diverged widely. Of the 39 children that were diagnosed with APD in the audiological clinic, 30 had deficits in auditory performance. Even more alarming was the fact that of the 45 children with a negative APD diagnosis, 32 showed clear signs of a central hearing deficit. Based on these results, we suggest revising current diagnostic procedure to evaluate APD in order to more clearly differentiate between central auditory processing deficits and higher-order (cognitive and/or language) processing deficits.

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Clinical Findings for a Group of Infants and Young Children with Auditory Neuropathy

Cited by 420 publications

References 67 publications

Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

Selective Inner Hair Cell Loss in Prematurity: A Temporal Bone Study of Infants from a Neonatal Intensive Care Unit

Extremely low birth weight infants are at high risk for auditory neuropathy

Auditory Processing Disorders with and without Central Auditory Discrimination Deficits

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