Sensory systems experience a period of intrinsically generated neural activity before maturation is complete and sensory transduction occurs. Here we review evidence describing the mechanisms and functions of this \'spontaneous\' activity in the auditory system. Both ex vivo and in vivo studies indicate that this correlated activity is initiated by non-sensory supporting cells within the developing cochlea, which induce depolarization and burst firing of groups of nearby hair cells in the sensory epithelium, activity that is conveyed to auditory neurons that will later process similar sound features. This stereotyped neural burst firing promotes cellular maturation, synaptic refinement, acoustic sensitivity, and establishment of sound-responsive domains in the brain. While sensitive to perturbation, the developing auditory system exhibits remarkable homeostatic mechanisms to preserve periodic burst firing in deaf mice. Preservation of this early spontaneous activity in the context of deafness may enhance the efficacy of later interventions to restore hearing.

UNASSIGNED: To investigate speech recognition in school-age children with early-childhood otitis media (OM) in conditions with noise or speech maskers with or without interaural differences. To also investigate the effects of three otologic history factors.
UNASSIGNED: Using headphone presentation, speech recognition thresholds (SRTs) were measured with simple sentences. As maskers, stationary speech-shaped noise (SSN) or two-talker running speech (TTS) were used. The stimuli were presented in a monaural and binaural condition (SSN) or a co-located and spatially separated condition (TTS). Based on the available medical records, overall OM duration, OM onset age, and time since the last OM episode were estimated.
UNASSIGNED: 6-13-year-olds with a history of recurrent OM (N = 42) or without any ear diseases (N = 20) with normal tympanograms and audiograms at the time of testing.
UNASSIGNED: Mixed-model regression analyses that controlled for age showed poorer SRTs for the OM group (Δ-value = 0.84 dB, p = 0.009). These appeared driven by the spatially separated, binaural, and monaural conditions. The OM group showed large inter-individual differences, which were unrelated to the otologic history factors.
UNASSIGNED: Early-childhood OM can affect speech recognition in different acoustic conditions. The effects of the otologic history warrant further investigation.

Over the last three decades, insects have been utilized to provide a deep and fundamental understanding of many human diseases and disorders. Here, we present arguments for insects as models to understand general principles underlying hearing loss. Despite ∼600 million years since the last common ancestor of vertebrates and invertebrates, we share an overwhelming degree of genetic homology particularly with respect to auditory organ development and maintenance. Despite the anatomical differences between human and insect auditory organs, both share physiological principles of operation. We explain why these observations are expected and highlight areas in hearing loss research in which insects can provide insight. We start by briefly introducing the evolutionary journey of auditory organs, the reasons for using insect auditory organs for hearing loss research, and the tools and approaches available in insects. Then, the first half of the review focuses on auditory development and auditory disorders with a genetic cause. The second half analyses the physiological and genetic consequences of ageing and short- and long-term changes as a result of noise exposure. We finish with complex age and noise interactions in auditory systems. In this review, we present some of the evidence and arguments to support the use of insects to study mechanisms and potential treatments for hearing loss in humans. Obviously, insects cannot fully substitute for all aspects of human auditory function and loss of function, although there are many important questions that can be addressed in an animal model for which there are important ethical, practical and experimental advantages.

OBJECTIVE: To investigate the effect of the interval between bilateral cochlear implantation on the development of bilateral peripheral auditory pathways as revealed by the electrically evoked auditory brainstem response (EABR).
METHODS: Fifty-eight children with profound bilateral sensorineural hearing loss were recruited. Among them, 33 children received sequential bilateral cochlear implants (CIs), and 25 children received simultaneous bilateral CIs. The bilateral EABRs evoked by electrical stimulation from the CI electrode were recorded on the day of second-side CI activation.
RESULTS: The latencies of wave III (eIII) and wave V (eV) were significantly shorter on the first CI side than on the second CI side in children with sequential bilateral CIs but were similar between the two sides in children with simultaneous bilateral CIs. Furthermore, the latencies were prolonged from apical to basal channels along the cochlea in the two groups. In children with sequential CIs, the inter-implant interval was negatively correlated with the eV latency on the first CI side and was positively correlated with bilateral differences in the eIII and eV latencies.
CONCLUSIONS: Unilateral CI use promotes the maturation of ipsilateral auditory conduction function. However, a longer inter-implant interval results in more unbalanced development of bilateral auditory brainstem pathways. Bilateral cochlear implantation with no or a short interval is recommended.

Thyroid hormone deficiency can lead to abnormal auditory development of varying severity. Retardation of morphological development, including delays in degeneration of Kölliker\'s organ and subsequent delayed formation of the inner sulcus, along with delayed opening of the tunnel of Corti and malformation of the tectorial membrane, was consistently observed in an antithyroid drug-induced congenital hypothyroidism rodent model. Abnormal morphological development could partly explain impaired adult auditory function. However, whether the development of inner hair cell ribbon synapses is influenced by hypothyroidism remains unclear. In the present study, we characterize the normal degeneration pattern of Kölliker\'s organ along the basal-to-apical axis. Then, we verified the retardation of morphological development in congenital hypothyroid mice. Using this model, we found that twisted collagen is present in the major tectorial membrane and delayed separation from supporting cells affects the minor tectorial membrane. Finally, we found that the number of synaptic ribbons was not significantly altered but the ribbon synapse maturation process was significantly impaired in congenital hypothyroid mice. We conclude that thyroid hormone is involved in structural development of the tectorial membrane and the ribbon synapse maturation process.

Congenitally deaf children who undergo cochlear implantation before 1 year of age develop their auditory skills faster than children who are implanted later. In this longitudinal study, a cohort of 59 implanted children were divided into two subgroups according to their ages at implantation-below or above 1 year old-and the plasma levels of matrix metalloproteinase-9 (MMP-9), brain-derived neurotrophic factor (BDNF), and pro-BDNF were measured at 0, 8, and 18 months after cochlear implant activation, while auditory development was simultaneously evaluated using the LittlEARs Questionnaire (LEAQ). A control group consisted of 49 age-matched healthy children. We identified statistically higher BDNF levels at 0 months and at the 18-month follow-ups in the younger subgroup compared to the older one and lower LEAQ scores at 0 months in the younger subgroup. Between the subgroups, there were significant differences in the changes in BDNF levels from 0 to 8 months and in LEAQ scores from 0 to 18 months. The MMP-9 levels significantly decreased from 0 to 18 months and from 0 to 8 months in both subgroups and from 8 to 18 months only in the older one. For all measured protein concentrations, significant differences were identified between the older study subgroup and the age-matched control group.

To assess changes and deficits in language and auditory exposures consequent to preterm birth and neonatal intensive care unit stay compared with exposures in utero among typically developing fetuses.
We analyzed over 23 000 hours of auditory exposure data in a cohort study of 27 typically-developing fetuses and 24 preterm infants. Extrauterine exposures for fetuses were captured by having pregnant women wear 24-hour audio recording devices. For preterm infants, recording devices were placed in the infant\'s crib. Multilevel linear regressions were conducted to test for group differences and effects of infant sex, maternal education, and mother\' occupation. A linear mixed-effects model was used to test for an effect of speaker gender.
Fetuses were exposed to an estimated 2.6 ± 1.8 hours/day of nearby, predominantly female language, nearly 5 times greater than 32 ± 12 minutes/day estimated for preterm infants (P < .001). Preterm infants had greater daily exposure to electronic sounds (5.1 ± 2.5 vs 1.3 ± 0.6 hours; P < .001) and noise (4.4 ± 2.1 vs 2.9 ± 2.8 hours; P < .05), with 4.7 ± 3.9 hours/day of silence. Language and extrauterine sound exposure for fetuses showed a marked day/night cyclical pattern, with low exposure during nighttime hours, but preterm infants\' exposures showed significantly less change across the 24-hour cycle (P < .001). Maternal occupation requiring frequent communication predicted greater language exposure (P < .05).
Our findings provide the first comparison of preterm infant auditory exposures to typically-developing fetuses. Some preterm infants may incur deficits of over 150 hours of language exposure over the preterm period. Given known effects of prenatal/preterm language exposure on neurobehavioral outcomes, this magnitude of deficit is alarming.

Auditory and verbal abilities of children with early cochlear implant (CI) surgery usually take some time to develop, and a reference index of early development for horizontal comparisons is urgently needed to guide the mapping process and adjust rehabilitation programs. Therefore, the aim of this study was to establish a reference value for early auditory preverbal skills development in children with CI and investigate the developmental curve and influencing factors for these children. The LittlEARS® Auditory Questionnaire (LEAQ) scores of 287 eligible Chinese participants were obtained at 1, 2, 3, 6, 9, 12, 18, 24, 28, and 36 months interval after CI activation. The median and standard deviation of the LEAQ score of each hearing stage for these children with different activation ages were calculated to establish the reference values. Quadratic regression was conducted to fit the expected developmental curve of the LEAQ score (y=-0.057x2+2.55x+5.45 [0 to 20 months]). With a linear mixed-effects model, we found that the receptive auditory behavior increased rapidly after CI activation, while expressive language skills developed at a steady rate. We also found that the following factors all significantly influenced the LEAQ: the duration of CI use, the development quotient, age of implantation and activation, and the presence of large vestibular aqueduct syndrome or auditory neuropathy spectrum disorder. The reference values and the expected developmental curve for the LEAQ in children with CI established by the present study provides guidance to clinicians and parents as well as realistic expectations regarding language and speech outcomes.

Using the Danish \'børneDAT\' corpus, the current study aimed to (1) collect normative masked speech recognition data for 6-13-year-olds in conditions with and without interaural difference cues, (2) evaluate the test-retest reliability of these measurements, and (3) compare two widely used measures of binaural/spatial benefit in terms of the obtained scores. Seventy-four children and 17 young adults with normal hearing participated. Using headphone presentation, speech recognition thresholds (SRTs) were measured twice at two separate visits in four conditions. In the first two conditions, børneDAT sentences were presented in diotic stationary speech-shaped noise, with the sentences either interaurally in-phase (\'N0S0\') or interaurally out-of-phase (\'N0S180\'). In the other two conditions, børneDAT sentences were simulated to come from 0° azimuth and two running speech maskers from either 0° azimuth (\'co-located\') or ±90° azimuth (\'spatially separated\'). In relative terms, the children achieved lower SRTs in stationary noise than in competing speech, whereas the adults showed the opposite pattern. 12-13-year-old children achieved adult-like performance in all but the co-located condition. Younger children showed generally immature speech recognition abilities. Test-retest reliability was highest for the SRTs in stationary noise and lowest for the spatial benefit scores. Mean benefit was comparable for the two measures and participant groups, and the two sets of scores were not correlated with each other. Developmental effects were most pronounced in the conditions with interaural difference cues. In conclusion, reference data for the børneDAT corpus obtained under different acoustic conditions are available that can guide future research and potential clinical applications.

UNASSIGNED: The processing of auditory temporal information is important for the extraction of voice pitch, linguistic information, as well as the overall temporal structure of speech. However, many aspects of its early development remain poorly understood. This paper reviews the development of auditory temporal processing during the first year of life when infants are acquiring their native language.
UNASSIGNED: First, potential mechanisms of neural immaturity are discussed in the context of neurophysiological studies. Next, what is known about infant auditory capabilities is considered with a focus on psychophysical studies involving non-speech stimuli to investigate the perception of temporal fine structure and envelope cues. This is followed by a review of studies involving speech stimuli, including those that present vocoded signals as a method of degrading the spectro-temporal information available to infant listeners.
UNASSIGNED: This review suggests that temporal resolution may be well developed in the first postnatal months, but that the ability to use and process the temporal information in an efficient way along the entire auditory pathway is longer to develop. Those findings have crucial implications for the development of language abilities, especially for infants with hearing impairment who are using cochlear implants.