Brainstem-Evoked Response Audiometry: Normative Data from the Preterm Infant

Cox, C. L.; Hack, Maureen; Metz, David A.

doi:10.3109/00206098109072682

Cited by 67 publications

(20 citation statements)

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“…14 In a few cases of HPS/aBS, hearing loss has been reported. 15 This might be related to a high incidence of sensorineural deafness (SND) in preterm infants (up to 10% 16,17 ), because HPS/aBS patients are born prematurely. However, Landau et al 18 described an association of "infantile Bartter syndrome" with SND in an inbred Bedouin kindred with at least 5 affected individuals.…”

mentioning

confidence: 99%

Hypokalemic Salt-Losing Tubulopathy With Chronic Renal Failure and Sensorineural Deafness

Jeck

Reinalter

Henne³

et al. 2001

Pediatrics

109

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ABSTRACT.Objective. To characterize a rare inherited hypokalemic salt-losing tubulopathy with linkage to chromosome 1p31.Methods. We conducted a retrospective analysis of the clinical data for 7 patients in whom cosegregation of the disease with chromosome 1p31 had been demonstrated. In addition, in 1 kindred, prenatal diagnosis in the second child was established, allowing a prospective clinical evaluation.Results. Clinical presentation of the patients was homogeneous and included premature birth attributable to polyhydramnios, severe renal salt loss, normotensive hyperreninemia, hypokalemic alkalosis, and excessive hyperprostaglandin E-uria, which suggested the diagnosis of hyperprostaglandin E syndrome/antenatal Bartter syndrome. However, the response to indomethacin was only poor, accounting for a more severe variant of the disease. The patients invariably developed chronic renal failure. The majority had extreme growth retardation, and motor development was markedly delayed. In addition, all patients turned out to be deaf.Conclusion. The hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome/antenatal Bartter syndrome. A pleiotropic effect of a single gene defect is most likely causative for syndromic hearing loss. Pediatrics 2001;108(1). URL: http:// www.pediatrics.org/cgi/content/full/108/1/e5; tubulopathy, Bartter syndrome, polyhydramnios, renal failure, syndromic deafness, pleiotropic gene.ABBREVIATIONS. HPS/aBS, hyperprostaglandin E syndrome/ antenatal Bartter syndrome; TAL, thick ascending limb of Henle's loop; PGE 2 , prostaglandin E 2 ; NKCC2, furosemide-sensitive Na-K-2Cl cotransporter; ROMK, renal outer-medullary potassium channel; SND, sensorineural deafness; GFR, glomerular filtration rate; BERA, brainstem-evoked response audiometry; PPN, partial parenteral nutrition. I nherited salt-losing tubulopathies with hypokalemic alkalosis involve an overlapping set of renal tubular disorders that can be subdivided into at least 3 phenotypes: 1) classic Bartter syndrome, 2) Gitelman syndrome, and 3) hyperprostaglandin E syndrome/antenatal Bartter syndrome (HPS/aBS). 1,2 Whereas patients with classic Bartter syndrome and Gitelman syndrome typically present in early infancy and childhood or adolescence, manifestation of HPS/aBS occurs in utero and the neonatal course is severe. 3 The first clinical sign is maternal polyhydramnios caused by fetal polyuria, which regularly results in premature birth between 28 and 34 weeks of gestation. 4,5 Postnatally, affected infants present with the typical pattern of impaired tubular reabsorption in the thick ascending limb of Henle's loop (TAL), including salt wasting, isosthenuric or hyposthenuric polyuria, and hypercalciuria with subsequent medullary nephrocalcinosis. 6 -8 Characteristically, endogenous formation of prostaglandin E 2 (PGE 2 ) is stimulated markedly, resulting in additional aggravation of saluretic polyuri...

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Hypokalemic Salt-Losing Tubulopathy With Chronic Renal Failure and Sensorineural Deafness

Jeck

Reinalter

Henne³

et al. 2001

Pediatrics

109

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“…Roberts et al (1982) also found no correlation between ABR test results and a number of risk factors, including intraventricular hemorrhage, hyaline membrane disease, asphyxia, hyperbilirubinemia, or aminoglycoside therapy. Cox, Hack, and Metz (1981) compared unselected preterm infants (32-40 weeks postconceptional age) with those scoring low (below 48) on the Hobel High Risk Screening Scales (Hobel, Hyvarinen, Okada, & Oh, 1973). No ABR differences could be attributed to perinatal, neonatal, or maternal conditions.…”

Section: Discussionmentioning

confidence: 99%

Neurobehavioral and Neurophysiological Assessment of Healthy and "At-Risk" Full-Term Infants

Eldredge¹,

Salamy²

1988

Child Development

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The Neurological and Adaptive Capacity Score (NACS) and the auditory brainstem response (ABR) are measures that, respectively, provide neurobehavioral and neurophysiological information regarding the status of the central nervous system (CNS) in the neonatal period. We evaluated these measures as indices of CNS functioning in 15 neonates born at risk for neurological sequelae and 15 healthy controls. Significant group differences were observed on the NACS. The ABR failed to distinguish the groups, but abnormal responses were obtained from 1 severely asphyxiated infant. Measures used to define ABR abnormality were also correlated among risk infants. We concluded that the NACS is sensitive to mild but diffuse disturbances, whereas the ABR appears to reflect insults to the brainstem structures that are susceptible to damage from anoxia. Utilization of both tests provides more complete information regarding CNS integrity in neonates.

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“…Several studies have reported normative values for neonates (5,6,II ,15,22,25,28,45,47). The general purpose has been to determine what the expected responses are in order that abnormal or atypical findings become apparent.…”

Section: Protocolmentioning

confidence: 99%

“…The current literature pertinent to ABR testing of neonates and NICU populations is discussed and from several aspects (5)(6)(7)II,12,17,20,25,34,36,47 (27), Marshall et al (29), Hecox and Cone (21), Hecox et al (20) , Cox et al (8), and Roberts et al (35). ABR as a screening tool has been reported by Homing (24), Crowell et al (9), Hecox et al (20), and Salamy et al (37), while Simmons (43) and Galambos et al (17) have compared ABR with Crib-O-Gram testing.…”

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“…The summary will not attempt to review early ABR literature primarily due to excellent reviews that are already published. The interested reader, however, would find Downs (13) very thorough.The current literature pertinent to ABR testing of neonates and NICU populations is discussed and from several aspects (5)(6)(7) II,12,17,20,25,34,36,47). Weber (47) The influence of various factors on the ABR have been explored…”

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The Current Status of Auditory Brainstem Response Testing in Neonatal Populations

Cox

1984

Pediatr Res

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SummaryThe use of auditory brainstem response (ABR) for assessment of hearing in the neonate has not been without challenge. Although numerous articles have appeared, agreement regarding the utility of neonatal ABR testing does not exist. In review of the current studies and commentaries, a clear majority are favorable to neonatal ABR testing. These studies along with current test procedures are discussed. (38); procedures for the assessment of that potential were initiated. Since 1975, the research reported in the literature has grown exponentially each year. In spite of the large and increasing amount of research reported, sever! issues have remained unresolved, viz., the applicability, reliability, and validity of neonatal ABR testing. In fact, because of these and other factors, some authors have begun to seriously question using ABR with neonates (13,35,42).. The need for early identification of infants with hearing loss is apparent. Normal language, learning, and social skill acquisition is contingent upon hearing. Although the incidence of hearing impairment in the general neonatal population is relatively low (0,26%) (41), in NICU neonates the incidence is considerably higher (2-10%) (16, 39). Furthermore, the improved survival rate of neonates born weighing less than 1500 g (19, 31) has increased the pool of infants in which a high incidence of hearing impairment is seen (16). Because the need for early identification of hearing loss does exist and the current procedures that could identify hearing impairment in the neonate are open to question (ABR), a critical review of ABR testing is in order. The purpose of this article is to summarize the current ABR literature, both pro and con, and to draw conclusions regarding its use based on the literature and the experience of the writer. The summary will not attempt to review early ABR literature primarily due to excellent reviews that are already published. The interested reader, however, would find Downs (13) very thorough.The current literature pertinent to ABR testing of neonates and NICU populations is discussed and from several aspects (5)(6)(7) II,12,17,20,25,34,36,47). Weber (47) The influence of various factors on the ABR have been explored

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Brainstem-Evoked Response Audiometry: Normative Data from the Preterm Infant

Cited by 67 publications

References 15 publications

Hypokalemic Salt-Losing Tubulopathy With Chronic Renal Failure and Sensorineural Deafness

Hypokalemic Salt-Losing Tubulopathy With Chronic Renal Failure and Sensorineural Deafness

Neurobehavioral and Neurophysiological Assessment of Healthy and "At-Risk" Full-Term Infants

The Current Status of Auditory Brainstem Response Testing in Neonatal Populations

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