T. Morlet

Auditory neuropathy/dyssynchrony: Its diagnosis and management

Patients with auditory neuropathy/dyssynchrony exhibit no auditory brain stem response (ABR), no middle ear muscle response, and both normal otoacoustic emissions or normal cochlear microphonics. An absent or grossly abnormal ABR is not always associated with deafness. In contrast, a hearing loss of 30 dB or more usually predicts absent otoacoustic emissions, but normal emissions can be seen in some patients whose behavioral audiograms imply total deafness. This article reviews the underlying physiology that makes these tests both useful and potentially misleading, and recommends steps to be considered by primary care physicians and other professionals to compensate for the vulnerabilities of each of the procedures.

Evidence of Peripheral Hearing Asymmetry in Humans: Clinical Implications

Auditory system hemispheric asymmetry in language processing is well-established, and there are many indications of lateralization as of the peripheral auditory system i.e., as of the cochlea. The left ear is more susceptible to noise damage; tinnitus is more predominant there, while spontaneous otoacoustic emissions (SOAEs) are more often found in the right ear. The present study addressed the following two questions: Does this right-ear SOAE prevalence exist as early as preterm birth? Is there any functional asymmetry in the medial olivo-cochlear (MOC) efferent system, known to modulate outer hair cell contractions? The study involved 483 preterm neonates (gestional age: 24-37 weeks) and 70 right-handed adults (age: 18-31 years). In each ear, SOAEs and evoked otoacoustic emissions (EOAEs) were recorded and analysed, and, for the adults, functional MOC system assessment was made. Results showed SOAEs and EOAE amplitude to be right-predominant and in adults a right MOC functional predominance. These results indicate peripheral auditory system lateralisation, and an early origin thereof. The MOC system being thought to play a protective role, its physiological lateralisation may be relevant to the left prevalence of tinnitus and of auditory fatigue.

Functional Maturation of Cochlear Active Mechanisms and of the Medial Olivocochlear System in Humans

The aim of this study was to investigate the functional development of cochlear active mechanisms and of the medial efferent olivocochlear system. Otoacoustic emissions (evoked and spontaneous) were recorded in 42 preterm neonates (conceptional age ranging form 33 to 39 weeks) and a control group of 20 young normal-hearing adults. Medial olivocochlear system activity was examined by coupling evoked otoacoustic emission recording to a contralateral stimulation. Otoacoustic emission recordings were carried out using the Otodynamic ILO88 software and hardware. The stimuli were unfiltered clicks and the contralateral stimulation was broad band noise of 50 and 70 dBSPL delivered by an Adam generator. The results revealed the presence of EOAEs and SOAEs from at least 33 weeks in humans, suggesting that the functional maturation of the outer hair cells is nearly complete at that age. The study further revealed that the contralateral stimulation had no effect on evoked otoacoustic emissions in preterm neonates. The lack of activity observed in medial olivocochlear system indicated functional immaturity here, at least before full-term birth.

Spontaneous otoacoustic emissions in preterm neonates: prevalence and gender effects

A number of lines of evidence indicate that the human cochlea is fully functional as a mature sound transducer by 6 months of age. However, information about the development of the active cochlear mechanisms and notably the development of outer hair cell (OHC) activity is yet incomplete. Recording and analysis of otoacoustic emissions (OAEs), probably generated by the OHCs of the organ of Corti, have led to a better understanding, in humans, of how sounds are analysed in the cochlea by means of active mechanisms. Evoked OAEs (EOAEs) and spontaneous OAEs (SOAEs), when they can be recorded in full-term and preterm neonates, show different characteristics from those in adults, suggesting that maturation of the peripheral auditory system is incomplete at birth. To learn more about this maturation, using the best-established facts concerning SOAEs in adults, such as their greater prevalence in females and also in right ears, SOAEs were studied in more detail in 81 preterm neonates, from 30 to 40 weeks of conceptional age, all presenting bilateral EOAEs according to objective criteria. The first finding of this study was that SOAEs existed and could be recorded as of 30 weeks of conceptional age in humans. Some SOAE characteristics in preterm neonates, such as prevalence, peak number and acoustic frequencies, showed similarity with full-term neonates. Comparison of other criteria between the two populations, such as greater SOAE prevalence in right ears and higher SOAE peak number in females, suggested that these developmental factors emerge around term in humans. Comparison of SOAE characteristics between male and female preterms suggested that male preterms were less advanced in peripheral auditory development than were female preterms.

Development of human cochlear active mechanism asymmetry: involvement of the medial olivocochlear system?

To study the functional development of the medial olivocochlear system, transient-evoked otoacoustic emission suppression experiments were conducted in 73 ears of 38 pre-term and 11 full-term neonates. The continuous contralateral stimulation was a broad band white noise, presented at 70 dB SPL. Efferent suppression was determined by subtracting the without-contralateral stimulation condition from the with-contralateral stimulation condition. Across this population, a mean suppression effect of contralateral stimulation on transient-evoked otoacoustic emissions was found, with most of the suppression effect observed after 8 ms. The amount of suppression is linearly, positively correlated with the conceptional age. In the subgroup of bilaterally tested neonates, the suppression of transient-evoked otoacoustic emissions is similar in the right ear and the left ear in subjects whose conceptional age is less than 36 weeks and significantly higher in the right ear than in the left ear in older neonates. This last observation was seen at frequencies where transient-evoked otoacoustic emission amplitudes became higher in the right ear than in the left ear as the conceptional age increased, a finding already reported in adults. This study shows that the functional adult pattern of the medial efferent system, probably involved in the detection of signals in noise such as speech sounds, seems to appear gradually in neonates and represents one of the several arguments in favor of functional auditory lateralization in humans, with a right ear advantage.

Connexin 26 variants and auditory neuropathy/dys-synchrony among children in schools for the deaf.

Genetic and auditory studies of 731 children with severe‐to‐profound hearing loss in US schools for the deaf and 46 additional children receiving clinical services for hearing loss ranging from moderate to profound demonstrated that mutations in the connexin 26 (GJB2) and connexin 30 (GJB6) genes explain at least 12% of those with nonsyndromic sensorineural deafness. Otoacoustic emissions (OAEs) testing to detect functional outer hair cells indicated that 76 of the children had emissions and therefore may have (as yet unconfirmed) auditory neuropathy/dys‐synchrony (AN/AD). Five of these children with OAEs were GJB2 homozygotes or compound heterozygotes with the genotypes 35delG/35delG, W77X/W77X, 35delG/360delGAG, 35delG/V95M, and V84M/M34T. In particular, unilateral AN/AD was confirmed in a child with moderate hearing loss and the 35delG/V95M genotype. Detecting OAEs in individuals with GJB2 mutations suggests that lack of functional gap junctions as a result of GJB2 mutations does not necessarily destroy all outer hair cell function.

Contralateral Suppression of Evoked Otoacoustic Emissions and Detection of a Multi-Tone Complex in Noise

Although some findings suggest that auditory efferent fibers are involved in perception in noise, their function remains controversial. The contralateral suppression of evoked otoacoustic emissions (EOAEs) has recently provided a means of exploring the medial olivocochlear system (MOCS) in humans. In an experiment based on this paradigm, the present study examined the relationships between variations of both EOAEs and detection-in-noise thresholds, induced in the same subjects by a contralateral 50-dB-SPL broad-band noise masker. EOAEs were recorded in response to a burst of a multitone complex composed of 1, 1.5 and 2-kHz components. The detection thresholds of this 3-component complex were measured at 2 ipsilateral noise levels: 50 and 70 dB SPL. The main finding was a significant correlation between EOAE suppression and threshold variations under contralateral masking. A relationship was also found between the contralateral suppression of EOAEs and threshold variation induced by the increase in ipsilateral noise level. These findings support the notion that the MOCS is involved in the detection of multicomponent stimuli in noise.

Spontaneous and evoked otoacoustic emissions in pre-term and full-term neonates: is there a clinical application?

In neonates and infants, hearing impairment leads to impaired language and cognitive development. For that reason, early detection of this sensory deficit is of outstanding importance, particularly in pre-term neonates, who constitute a high risk population in regard to very early acquired hearing loss. Evoked (EOAE) and spontaneous otoacoustic emission (SOAE) recording in 93 pre-term and full-term neonates revealed that this technique is potentially useful for auditory screening in neonatology units. EOAEs and SOAEs can be recorded successfully from 30 weeks of conceptional age. SOAEs were found to be prevalent in females and presented higher peak numbers in right than in left ears. Furthermore, SOAE incidence in pre-term and full-term neonates was found to be high in EOAE positive ears, associated with strong and robust EOAEs.

Contralateral auditory stimulation and otoacoustic emissions: a review of basic data in humans.

The influence of contralateral auditory stimulation on otoacoustic emissions (OAE), spontaneous OAE, evoked OAE and acoustic distortion products, can be summarized as follows: (1) alteration (mainly a decrease) of OAE amplitude; (2) alteration of response spectrum (upward shift frequency of SOAE); (3) alteration of phase; (4) effect dependent on intensity of contralateral stimulation; (5) effect inversely dependent on intensity of ipsilateral stimulation; (6) frequency specificity of the suppressive effect. Involvement of the medial olivocochlear bundle is highly probable but one cannot exclude a double pathway including also the acoustic reflex.

Auditory screening in high-risk pre-term and full-term neonates using transient evoked otoacoustic emissions and brainstem auditory evoked potentials

The present report concerns a 3 year, 8 month hearing screening in 1531 high-risk neonates by means of two successive transient evoked otoacoustic emission (TEOAE) recordings followed, in cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE 1 and 2 and BAEP testing, 1361 infants (88.9%) were declared normal, and 170 (11.1%) suspected of hearing loss. Of these 170, 58 showed bilateral and 26 unilateral impairment. Definite hearing loss on ORL consultation was diagnosed in 14 infants (0.9% of the screened population as a whole); 22 are still being followed, while 86 (5.6%) failed to consult for diagnosis. The mean age on diagnosis of definite hearing loss was 9.9 +/- 4.9 (range 4-20) months. Several auditory function risk factors proved more frequent in deaf than in normal children. Our results show that early hearing loss screening in at-risk neonates needs to be pursued.