Yvonne Sininger

Lateral asymmetry in the ABR of neonates: evidence and mechanisms.

Lateralized processing of auditory stimuli occurs at the level of the auditory cortex but differences in function between the left and right sides are not clear at lower levels of the auditory system. The current study is designed to (1) investigate asymmetric auditory function at the ear and brainstem in human infants and (2) investigate possible mechanisms for asymmetry at these levels. Study 1 evaluated auditory brainstem responses (ABRs) in response to high and low-level clicks presented to the right and left ears of neonates. Wave V was significantly larger in amplitude and waves III and V were shorter in latency when the ABR was generated in the right ear. Study 2 investigated two possible mechanisms of such asymmetry by (a) using contralateral white noise masking to activate the medial olivocochlear system and (b) increasing stimulus rate to reveal neural conduction and synaptic mechanisms. ABR wave V, evoked by clicks to the left ear, showed a greater reduction in amplitude with contralateral noise than the response evoked from the right ear. No systematic asymmetries in ABR latencies or amplitudes were found with increased stimulus rate. We conclude that (1) the click-evoked ABR in neonates demonstrates asymmetric auditory function with a small but significant right ear advantage and (2) asymmetric activation of the medial olivocochlear system, specifically greater contralateral suppression of ABR produced by the left ear, is a possible mechanism for asymmetry.

Infant hearing loss and connexin testing in a diverse population

Purpose: Previous studies of connexin-related hearing loss have typically reported on mixed age groups or adults. To further address epidemiology and natural history of connexin-related hearing loss, we conducted a longitudinal study in an ethnically diverse cohort of infants and toddlers under 3 years of age. Our study compares infants with and without connexin-related hearing loss to examine differences in the prevalence of connexin and non-connexin-related hearing loss by ethnic origin, detection by newborn hearing screening, phenotype, neonatal risk factors, and family history. This is the first study to differentiate infants with and without connexin-related hearing loss.Methods: We enrolled 95 infants with hearing loss from whom both exons of Cx26 were sequenced and the Cx30 deletion was assayed. Demographic, family history, newborn hearing screening data, perinatal, and audiologic records were analyzed.Results: Genetic testing identified biallelic Cx26/30 hearing loss-associated variants in 24.7% of infants with a significantly lower prevalence in Hispanic infants (9.1%). Eighty-two infants underwent newborn hearing screening; 12 infants passed, 3 had connexin-related hearing loss. No differences in newborn hearing screening pass rate, neonatal complications, or hearing loss severity were detected between infants with and without connexin-related hearing loss. Family history correlates with connexin-related hearing loss.Conclusions: Connexin-related hearing loss occurs in one quarter of infants in an ethnically diverse hearing loss population but with a lower prevalence in Hispanic infants. Not all infants with connexin-related hearing loss fail newborn hearing screening. Family history correlates significantly with connexin-related hearing loss. Genetic testing should not be deferred because of newborn complications. These results will have an impact on genetic testing for infant hearing loss.

A prospective, longitudinal study of the impact of GJB2/GJB6 genetic testing on the beliefs and attitudes of parents of deaf and hard-of-hearing infants.

There are limited data on the impact of incorporating genetic counseling and testing into the newborn hearing screening process. We report on results from a prospective, longitudinal study to determine the impact of genetic counseling and GJB2/GJB6 genetic testing on parental knowledge, attitudes, and beliefs about genetic testing. One hundred thirty culturally hearing parents of 93 deaf or hard‐of‐hearing children ages 0–3 years primarily identified through newborn hearing screening received pre‐ and post‐test genetic counseling for GJB2 and GJB6. Parents completed questionnaires following pre‐test counseling, and 1‐ and 6‐month post‐test result disclosure. Results indicate that following pre‐test counseling all parents perceived benefits to genetic testing. While parents who received positive results continued to perceive benefits from testing, perceived benefit declined among parents who received inconclusive or negative results. Parents did not perceive genetic testing as harmful following pre‐test counseling or receipt of test results. Parents who received positive test results performed better in understanding recurrence and causation of their childs deafness and indicated greater interest in prenatal genetic testing than those who received inconclusive or negative test results. Parents felt that pediatricians and audiologists should inform parents of genetic testing availability; however, there was no consensus on timing of this discussion. Thus culturally hearing parents do not perceive genetic testing of their deaf or hard‐of‐hearing infants/toddlers as harmful; they feel that primary care providers should discuss genetic testing with them; and positive genetic test results with genetic counseling give rise to better understanding and perceived benefit than negative or inconclusive results.

Impaired Timing and Frequency Discrimination in High-functioning Autism Spectrum Disorders

Individuals with autism spectrum disorders (ASD) frequently demonstrate preserved or enhanced frequency perception but impaired timing perception. The present study investigated the processing of spectral and temporal information in 12 adolescents with ASD and 15 age-matched controls. Participants completed two psychoacoustic tasks: one determined frequency difference limens, and the other determined gap detection thresholds. Results showed impaired frequency discrimination at the highest standard frequency in the ASD group but no overall difference between groups. However, when groups were defined by auditory hyper-sensitivity, a group difference arose. For the gap detection task, the ASD group demonstrated elevated thresholds. This supports previous research demonstrating a deficit in ASD in temporal perception and suggests a connection between hyper-sensitivity and frequency discrimination abilities.

Ethnic differences in parental perceptions of genetic testing for deaf infants.

As genetic testing becomes an integral part of the evaluation of deaf infants and children, it is important to understand parental views on genetic testing. The purpose of this study is to examine parental reasons for, and beliefs about, genetic testing for deafness in early-identified infants, and to determine if they differ as a function of ethnicity. We present baseline data collected from 56 Caucasian, 59 Hispanic, and 24 Asian parents of deaf children participating in a longitudinal, prospective study on genetic testing for connexin-related deafness. The overall finding is that reasons for, and beliefs about, genetic testing for deafness varied as a function of ethnicity. Virtually all parents sought genetic testing to understand why their child is deaf. However, Asian and/or Hispanic parents were more likely than Caucasian parents to view family planning, helping with their child’s medical care, and helping the family as other important reasons for testing, and were more likely than Caucasian parents to perceive genetic testing to be useful for these purposes. Asian and Hispanic parents were more likely than Caucasian parents to perceive genetic testing in harmful terms. Genetic testing fulfills a cognitive need for parents to understand why their child is deaf, yet differences in responses suggest that Asian and Hispanic parents may seek testing for other purposes. Understanding different perspectives on genetic testing for deafness will enhance genetic counselors’ cultural competence and facilitate the pre-test genetic counseling session.

Dichotic listening after cerebral hemispherectomy: Methodological and theoretical observations ☆

We examined two commonly used dichotic listening tests for measuring the degree of hemispheric specialization for language in individuals who had undergone cerebral hemispherectomy: the consonant-vowel (CV) nonsense syllables and the fused words (FW) tests, using the common laterality indices f and lambda. Hemispherectomy on either side resulted in a massive contralateral ear advantage, demonstrating nearly complete ipsilateral suppression of the left ear in the right hemispherectomy group but slightly less complete suppression of the right ear in the left hemispherectomy group. The results are consistent with the anatomical model of the ear advantage [Kimura, D. (1961). Most syllables or words are reported for the ear contralateral to the remaining hemisphere, while few or none are reported for the ear ipsilateral to the remaining hemisphere. In the presence of competing inputs to the two ears, the stronger contralateral ear-hemisphere connection dominates/suppresses the weaker ipsilateral ear-hemisphere connection. The lambda index was similar in the two tests but the index f was higher in the CV than the FW test. Both indices of the CV test were sensitive to side of resection, higher in the right hemispherectomy than in the left hemispherectomy groups.

Laterality of basic auditory perception.

Laterality (left–right ear differences) of auditory processing was assessed using basic auditory skills: (1) gap detection, (2) frequency discrimination, and (3) intensity discrimination. Stimuli included tones (500, 1000, and 4000 Hz) and wide-band noise presented monaurally to each ear of typical adult listeners. The hypothesis tested was that processing of tonal stimuli would be enhanced by left ear (LE) stimulation and noise by right ear (RE) presentations. To investigate the limits of laterality by (1) spectral width, a narrow-band noise (NBN) of 450-Hz bandwidth was evaluated using intensity discrimination, and (2) stimulus duration, 200, 500, and 1000 ms duration tones were evaluated using frequency discrimination. A left ear advantage (LEA) was demonstrated with tonal stimuli in all experiments, but an expected REA for noise stimuli was not found. The NBN stimulus demonstrated no LEA and was characterised as a noise. No change in laterality was found with changes in stimulus durations. The LEA for tonal stimuli is felt to be due to more direct connections between the left ear and the right auditory cortex, which has been shown to be primary for spectral analysis and tonal processing. The lack of a REA for noise stimuli is unexplained. Sex differences in laterality for noise stimuli were noted but were not statistically significant. This study did establish a subtle but clear pattern of LEA for processing of tonal stimuli.

Deaf Genetic Testing and Psychological Well-Being in Deaf Adults

Limited data suggest that enhanced self-knowledge from genetic information related to non-medical traits can have a positive impact on psychological well-being. Deaf individuals undertake genetic testing for deaf genes to increase self-knowledge. Because deafness is considered a non-medical trait by many individuals, we hypothesized that deaf individuals receiving a genetic explanation for why they are deaf will experience increased psychological well-being. We report results from a prospective, longitudinal study to determine the impact of genetic testing (GJB2, Cx26; GJB6, Cx30) on perceived personal control (PPC), anxiety, and depression in deaf adults (N = 209) assessed following pre-test genetic counseling as well as 1-month and 6-months following test result disclosure. Participants were classified as Cx positive (n = 82) or Cx negative/inconclusive (n = 127). There was significant evidence for Cx group differences in PPC and anxiety over time (PPC: Cx group*time interaction p = 0.0007; anxiety: Cx group*time interaction p = 0.002), where PPC scores were significantly higher, and anxiety scores were significantly lower for the Cx positive group relative to the negative/inconclusive group following test result disclosure. Compared to pre-test, PPC scores increased at 1-month (p = 0.07) and anxiety scores decreased at 6-months (p = 0.03) for the Cx positive group. In contrast, PPC scores decreased (p = 0.009, p < 0.0001) and anxiety scores increased (p = 0.09, p = 0.02) for the Cx negative/inconclusive group at 1- and 6-months post test result disclosure. Genetic testing for deaf genes affects the psychological well-being of deaf individuals. Increasing deaf adults’ access to genetic testing may potentially enhance self-knowledge and increase psychological well-being for those who receive a genetic explanation, which could offer downstream health benefits.

Comment on “Ear Asymmetries in middle-ear, cochlear, and brainstem responses in human infants” [J. Acoust. Soc. Am. 123, 1504–1512]

Following Sininger and Cone-Wesson [Science 305, 1581], Sininger and Cone-Wesson [Hear. Res. 212, 203-211], Keefe et al. [J. Acoust. Soc. Am. 123(3), 1504-1512] described ear asymmetries in middle ear, cochlear, and brainstem responses of infants. Keefe et al. state that their data do not support the findings of Sininger and Cone-Wesson [Science 305, 1581] who found asymmetries in evoked otoacoustic emissions and auditory brainstem responses and proposed that stimulus-directed asymmetries in processing may facilitate development of hemispheric specialization. The Keefe et al. findings, in fact, replicated and extended the findings of Sininger and Cone-Wesson (2004, 2006) and support, rather than refute, the conclusions. Keefe et al. controlled neither the background noise nor averaging time across test conditions (ear or otoacoustic emission type) and thus their separate analyses of signal and noise magnitude exceed the limitations imposed by the data collection methods.