Clinical and Audiological Features in Auditory Neuropathy

Madden, Colm; Rutter, Michael; Hilbert, Lisa; Greinwald, John H.; Choo, Daniel I.

doi:10.1001/archotol.128.9.1026

Cited by 201 publications

(212 citation statements)

References 22 publications

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“…A subsequent study from the US reported a prevalence rate of 5.1% in a population of children with hearing loss. 8 Although the rate of SNHL in the NICU infant population is 10 times more common than for normal term infants, the prevalence of AN in this same population is not established. Stein 14 reported an occurrence of AN of 4 per 100 in a consecutive series of admissions to an infant special care nursery.…”

Section: Discussionmentioning

confidence: 99%

“…8,3,15 Bilirubin is a known neuronal toxin, and it may play a role in the etiology of AN. [16][17][18][19] In a recent study, Berg et al 12 reported an association between AN and elevated peak total bilirubin levels.…”

Section: Discussionmentioning

confidence: 99%

“…Among children with diagnosed hearing loss, the prevalence rate of AN is high, ranging between 5.1 and 14.6 per 100. 3,[8][9][10] There have been case reports since the 1970s of patients presenting with symptoms consistent with AN; however, it was not until 1996 that AN was first defined by Starr et al 11 Thus, there is a paucity of information regarding prevalence rates of AN among neonates. Berg et al 12 recently showed that infants in the intensive care nursery are at significantly higher risk for the development of AN.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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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%

“…Retrospective clinical studies have suggested that low birth weight and prematurity are risk factors for AN (Madden et al 2002;Beutner et al 2007;Xoinis et al 2007;Teagle et al 2010). Of course, prematurity is associated with a long list of comorbid factors including hyperbilirubinemia, hypoxia, necrotizing enterocolitis, bronchopulmonary dysplasia (Beutner et al 2007;Teagle et al 2010), and other clinical conditions also noted in the NICU charts of the premature infants in this study (Tables 1 and 2).…”

Section: Full-term Casementioning

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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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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A novel missense mutation in a C2 domain ofOTOF results in autosomal recessive auditory neuropathy

2005

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Screening of 12 Turkish families with apparently autosomal recessive nonsyndromic sensorineural deafness without GJB2 and mtDNA m.1555A > G mutations for 11 previously mapped recessive deafness loci showed a family in which hearing loss cosegregated with the DFNB9 (OTOF) locus. Three affected children were later found to carry a novel homozygous c.3032T > C (p.Leu1011Pro) mutation in the OTOF gene. Both parents were heterozygous for the mutation. p.Leu1011Pro alters a conserved leucine residue in the C2D domain of otoferlin. Pure tone audiometry of the family showed severe to profound sensorineural hearing loss (with U-shape audiograms) in children, and normal hearing in the parents. Otoacoustic emissions and auditory brainstem response (ABR) suggested the presence of auditory neuropathy in affected individuals.

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Clinical and Audiological Features in Auditory Neuropathy

Cited by 201 publications

References 22 publications

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

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

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

A novel missense mutation in a C2 domain ofOTOF results in autosomal recessive auditory neuropathy

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