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Otoacoustic Emissions: a New Method for Newborn Hearing Screening

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Otoacoustic Emissions: a New Method for Newborn Hearing Screening European Review for Medical and Pharmacological Sciences 2004; 8: 129-133 Otoacoustic emissions: a new method for newborn hearing screening P. SAURINI, G. NOLA*, D. LENDVAI Clinical Pedriatics Institute *Department of Otolaryngology, Audiology and Phoniatrics “G. Ferreri” “La Sapienza” University - Rome (Italy) Abstract. – Pediatric deafness is a handi- sorineural deafness due to genetic or congen- cap affecting approximately 2/1000 newborns. ital cause affects 1-2/1000 healthy newborns Currently, its diagnosis is markedly delayed, and involves from 4% to 5%3,4 of newborns since it occurs approximately at 24 to 36 months of age; at this age rehabilitation procedures (i.e., who show at birth one or more audiologic 2,5 acoustic prosthesis, speech therapy, psycholog- risk factors . At any rate, the prevalence of ical interventions on the family, or cochlear im- infant deafness is higher than that of other plants in the most serious situations) are unable congenital diseases, such as phenylketonuria to ensure a complete development of both the and hypothyroidism, for which newborns are voice and the speech, thus preventing the full routinely submitted to screening procedures. participation of the deaf child in social living. The turning point has taken place when meth- The period of “cerebral plasticity” is estab- ods and techniques were developed; they are lished in the first 3 years of life, during which aimed at the very early diagnosis of infantile complex and organized interneuronal circuits deafness and are based on the recordings of are developed. Peripheral acoustic input is otoacoustic emissions, that is, acoustic signals essential for the proper maturity of central of extremely weak intensity originating in the in- auditory pathways and allows to acquire both ner ear, which not only is a passive transducer, auditory memory and speech6,7. The deaf but is able to generate sounds also. Any lack of or any change in otoacoustic emissions is a ac- child has no chance to develop adequately a curate index of disabling deafness. The test un- normal ability to concentrate and pay atten- der study allows to perform selectively a mass tion. As a consequence, speech reception and screening on newborns (it is carried out 2 or 3 expression are limited, leading, in turn, to days after birth) since it is definetely non-inva- changes in emotional maturity, relational dif- sive, it is done very rapidly (a few seconds only), ficulties, and sociocultural integration8,9. In it is cost-effective and higly reliable. The new- born hearing screening is being accepted, at a view of such considerations, the top priority faster growing pace, by an increasing number of goal is the early discovery of genetic and/or health systems in the whole world. congenital deafness. Currently, the mean age of identification of pediatric deafness is still Key Words: rather high, being established at around 24-30 Otoacoustic emission, Distortion Product OtoAcoustic months10. The priority of an early diagnosis, Emissions (DPOAEs). aimed at a prompt intervention strategy capa- ble of operating during the period of cerebral plasticity, led the scientific community to multiply the efforts towards the definition of an effective program of newborn hearing screening. It is common knowledge that ap- Introduction proximately 50% of identified deafness in in- fants shows none of the 10 audiologic risk The pathogenesis of pediatric deafness is factors indicated by the Joint Committee on quite variable and often unknown. However, Infant Hearing Screening Assessment11,12. the prevalence of this auditory handicap in Therefore, a hearing screening performed on the infantile population is approximately the so called “audiologic risk” newborns on- 2/1000 infants1. Severe and/or marked sen- ly, implies the missed identification of about 129 P. Saurini, G. Nola, D. Lendvai one-half of neonatal deafness. During the last – Distortion products OAEs (DPOAEs) ob- few years, a number of studies13-20 have tained by the contemporaneous presenta- demonstrated the possibility of developing a tion of 2 sound stimuli (f1 and f2), or pri- neonatal audiologic screening based on the mary tones, bound together by a frequen- recordings of evoked otoacoustic emissions cy relationship; these emissions consist of (EOAEs). Evoked otoacoustic emissions are different frequencies with respect to ap- acoustic signals of non-linear type delivered plied primary stimuli resulting from the by the external ciliated cells (ECCs) of the combination of f1 and f2 as a result of ei- cochlea. Such cells are present in 100% of ther their difference or their summation. normally hearing individuals and are the ex- pression of a normal cochlear function; they Out of these 3 categories of otoacoustic are stable, reproducible, influenced by all the emissions, the SEOAEs, even though are cochlear nociceptive factors, and absent in present in around 94% of normal hearing hypoacusia higher than 40 decibels. subjects, are the least used in clinical practice Depending on the presence or absence of in view of the complexity of their recording. an external stimulation, otoacoustic emissions TEOAEs are the best known otoacoustic (OAEs) may be classified in two main cate- emissions (Figure 1); in their behalf a wide gories: spontaneous OAEs and evoked OAEs. field of clinical application was quickly A) Spontaneous OAEs (SOAEs) are ob- gained as early as the 1980s. tained without any sound stimulation. They Morphologic stability and reproducibility are narrow band signals, single or more often are the main features of these responses26,27, multiple, unilateral or bilateral. They can be even though great differences may be found measured in 40% to 70% of normal ears21,22, among individuals as well as between the ears and show a sinusoidal shape similar to the typ- of the same individual; often the contralateral ical shape of pure tones. In the majority of the ear shows a rather similar response pattern. instances they are found in a frequency range These signals, in normal hearing ears, are from 1 to 2 KHz; it has been supposed, there- present in 98% to 100% of the cases, inde- fore, that they originate from the intermedi- pendently of sex and age29,30. ate-apical portion of the cochlea. Their origin DPOAEs (Figure 2) are the result of an ac- has been ascribed to a double mechanism: a tive process of intermodulation at cochlear physiologic mechanism23,24 linked both to the level mediated by the ECCs and are obtained spontaneous activity of the ECCs and to the by simultaneously signaling two stimulating activity chemically and/or electrically induced pure external tones, called primary, namely under steady control of the olivocochlear bun- f1 and f2; the former is deemed to be an ex- dle. The second mechanism, indicated as ternal tone whose frequencies are lower than pathologic25, would be related to a sharply de- those of the latter. limited “focal” change or damage of the ECCs, whereas in this situation the producers of SOAEs would be the intact cells adjoining the damaged areas. Both the conditions of the test and the characteristics of the measuring instrumentation used influence, to a significant extent, the stability of the intensity level. B) EOAEs are subdivided into the follow- ing categories, depending on the characteris- tics of the administered stimulus: – Simultaneous EOAEs (SEOAEs) ob- tained by means of continuous sonorous stimuli; – Transient EOAEs (TEOAEs) obtained by means of cliks or tone-bursts, and better known as cochlear echoes, or Figure 1. Transient evoked otoacoustic emissions Kemp echoes, or transient echoes; (TEOAEs). 130 Otoacoustic emissions: a new method for newborn hearing screening DPOAE responses and of TEOAEs are con- cerned, it is appropriate to specify and em- phasize the importance of the middle ear; in fact, the cochlea produces an acoustic emis- sion of very low intensity and therefore a sig- nificant otoacoustic emission measured in the ear canal is obtained as a result of a ret- rograde vibration of the ear drum through the auditory ossicles chain. As a conse- quence, from what has been said above it can be deduced that only a healthy middle ear may be able to acoustically coupling, in an effective way, the cochlea with the air of the ear canal; thus a normal function of the mid- dle ear is essential for the recording of nor- Figure 2. Distortion Products Otoacoustic Emissions mal otoacoustic emissions. (DPOAEs). Evoked otoacoustic emissions, with partic- ular reference to TEOAEs and DPOAEs, must be considered by now as a powerful Commonly studied DPOAEs correspond means for study and a valuable clinical test to the so called “tones of cubic difference” for audiologic pathological conditions. resulting from the combination 2f1-f231,32. Otoacoustic emissions are the only specific In fact, they represent the distortion prod- method of investigation, for the direct and uct of the highest intensity in both humans objective study of cochlear mechanisms and and animals, one of the most stable and dynamics. In general, otoacoustic emissions certainly that which is linked to the active are simple to perform, absolutely non-inva- non linear micromechanisms of the cochlea. sive and non-traumatic and thus can be car- Such a feature makes such a distortion ried out in newborns during sleep; they are product particularly sensitive and quite ear- well accepted by grown up children. The lim- ly vulnerable with respect to the harmful ited cost of the instrumentation used as well stimuli of toxic, traumatic and degenerative as the short time required to perform an ex- type involving the cochlea. By properly amination are additional items on behalf of varying the frequency values of either pri- this diagnostic method. mary tone it is possible to obtain combina- Among the international experiences, that tions of different frequency; as a conse- are points of reference for the use of quence, different cochlear areas, both to- TEOAEs as a method for newborn hearing wards the apex and towards the base, may screening, we would like to mention the fol- be explored objectively.
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