Auditory Brainstem Pathology in Autism Spectrum Disorder: A Review.

Atypical responses to sound are common in individuals with autism spectrum disorder (ASD), and growing evidence suggests an underlying auditory brainstem pathology. This review of the literature provides a comprehensive account of the structural and functional evidence for auditory brainstem abnormalities in ASD. The studies reviewed were published between 1975 and 2016 and were sourced from multiple online databases.

Early childhood undernutrition increases risk of hearing loss in young adulthood in rural Nepal.

Background: Prevalence of young adult hearing loss is high in low-resource societies; the reasons for this are likely complex but could involve early childhood undernutrition.

Objective: We evaluated preschool childhood stunting, wasting, and underweight as risk factors for hearing loss in young adulthood in Sarlahi District, southern Nepal.

Design: Ear health was assessed in 2006-2008 in a cohort of 2193 subjects aged 16-23 y, who as children <60 mo of age participated in a 16-mo placebo-controlled, randomized vitamin A supplementation trial from 1989 to 1991.

Hearing loss is associated with decreased nonverbal intelligence in rural Nepal.

Objective: To evaluate the association between adolescent and young-adult hearing loss and nonverbal intelligence in rural Nepal.

Study Design: Cross-sectional assessment of hearing loss among a population cohort of adolescents and young adults.

Setting: Sarlahi District, southern Nepal.

Severe auditory processing disorder secondary to viral meningoencephalitis.

Objective: To describe auditory function in an individual with bilateral damage to the temporal and parietal cortex.

Design: Case report.

Study Sample: A previously healthy 17-year old male is described who sustained extensive cortical injury following an episode of viral meningoencephalitis.

Speech processing disorder in neural hearing loss.

Deficits in central auditory processing may occur in a variety of clinical conditions including traumatic brain injury, neurodegenerative disease, auditory neuropathy/dyssynchrony syndrome, neurological disorders associated with aging, and aphasia. Deficits in central auditory processing of a more subtle nature have also been studied extensively in neurodevelopmental disorders in children with learning disabilities, ADD, and developmental language disorders. Illustrative cases are reviewed demonstrating the use of an audiological test battery in patients with auditory neuropathy/dyssynchrony syndrome, bilateral lesions to the inferior colliculi, and bilateral lesions to the temporal lobes.

Childhood IQ, hearing loss, and maternal thyroid autoimmunity in the Baltimore Collaborative Perinatal Project.

Background: Maternal thyroid autoantibodies during pregnancy have been implicated in neurodevelopmental delays, including early childhood cognitive deficits. We evaluated whether maternal autoantibodies to thyroid peroxidase (TPOaAbs) during late pregnancy were associated with childhood intelligence quotient (IQ) scores in their offspring and how the children's TPOaAb-associated sensorineural hearing loss (HL) might affect the result.

Methods: We evaluated banked third-trimester sera corresponding to 1,733 children for whom childhood cognitive test scores and audiology data were available.

Sensorineural Hearing Loss following Carbon Monoxide Poisoning.

A case study is presented of a 17-year-old male who sustained an anoxic brain injury and sensorineural hearing loss secondary to carbon monoxide poisoning. Audiological data is presented showing a slightly asymmetrical hearing loss of sensorineural origin and mild-to-severe degree for both ears. Word recognition performance was fair to poor bilaterally for speech presented at normal conversational levels in quiet.

Vitamin A supplementation in preschool children and risk of hearing loss as adolescents and young adults in rural Nepal: randomised trial cohort follow-up study.

Objective: To determine whether vitamin A supplementation administered in the preschool years can lower the risk of hearing loss in adolescence and adulthood.

Design: Follow-up study of adolescents and young adults who, as preschool aged children in 1989, were enrolled into a cluster randomised, double blinded, placebo controlled trial of vitamin A supplementation.

Setting: South central, rural Nepal.

Hearing loss in osteogenesis imperfecta: characteristics and treatment considerations.

Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues.

Effects of sedation on auditory brainstem response in Rett syndrome.

Prolongation of the I-V interpeak latency intervals have been reported in Rett syndrome and other neurodevelopmental disorders. It has been suggested that the use of sedation may account for differences in the interpeak latency intervals when comparisons are made across diagnostic groups if sedated control groups are not used for the basis of comparison. This study examined the effects of sedation on auditory brainstem response interpeak latency intervals (i.

Prenatal antecedents of newborn neurological maturation.

Fetal neurobehavioral development was modeled longitudinally using data collected at weekly intervals from 24 to 38 weeks gestation in a sample of 112 healthy pregnancies. Predictive associations between 3 measures of fetal neurobehavioral functioning and their developmental trajectories to neurological maturation in the first weeks after birth were examined. Prenatal measures included fetal heart rate (FHR) variability, fetal movement, and coupling between fetal motor activity and heart rate patterning; neonatal outcomes include a standard neurologic examination (n = 97) and brainstem auditory evoked potential (BAEP; n = 47).

Prevalence of hearing loss and ear morbidity among adolescents and young adults in rural southern Nepal.

A cross-sectional survey was conducted among 3646 persons, 15 to 23 years of age, in the southern plains District of Sarlahi, Nepal, to assess the prevalence and severity of hearing loss and middle-ear dysfunction, and adverse effects of hearing loss on social integration. Between 2006 and 2008, subjects were enumerated and underwent otoscopic, tympanometric, and audiometric evaluations at central sites in villages. Hearing loss was diagnosed in subjects who failed a hearing screening and exhibited an average air conduction threshold value greater than or equal to 30 dBHL in the worse ear.

Infection and thyroid autoimmunity: A seroepidemiologic study of TPOaAb.

Infectious agents have been implicated as triggers of autoimmunity. Prospective epidemiologic studies of infection with specific pathogens and the subsequent elevation of specific autoantibodies are difficult and costly to conduct. As a result, a solid body of evidence regarding this theoretically intriguing connection remains to be accrued.

Audiological findings in osteogenesis imperfecta.

Background: Osteogenesis imperfecta (OI) is an inherited bone and connective tissue disorder associated with the lifelong occurrence of frequent fractures following even mild trauma. Hearing loss is frequently reported in patients with OI.

Purpose: This investigation is a retrospective study of measurements of tympanometry, acoustic reflexes, and transient evoked otoacoustic emissions in a sample of patients with OI grouped according to age.

Auditory function in adrenomyeloneuropathy.

Auditory brainstem responses (ABR), ipsilateral and contralateral acoustic reflexes and the masking level difference for speech (MLD) were studied in 29 patients with adrenomyeloneuropathy (AMN). Abnormalities were seen for all ABR components with Waves V and III affected to the greatest degree. For male patients with AMN, the I-III, III-V and I-V interpeak latency intervals were abnormal for a majority of patients.

Maternal thyroid autoantibodies during the third trimester and hearing deficits in children: an epidemiologic assessment.

Elevated maternal thyroid autoantibodies during pregnancy are linked to infertility, miscarriage, and neurodevelopmental deficits such as in cognitive function. It has not been established whether autoantibodies to thyroid peroxidase are associated with sensorineural hearing loss (SNHL). The authors tested stored third-trimester maternal serum specimens of 1,736 children for thyroid peroxidase autoantibodies (TPOaAb) by using an enzyme-linked immunosorbent assay technique.

Auditory brainstem response findings and peripheral auditory sensitivity in adrenoleukodystrophy.

Measurements of the auditory brainstem response (ABR) were obtained in 96 individuals with X-linked adrenoleukodystrophy (X-ALD). The patients were divided into five diagnostic groups on the basis of neurologic diagnosis. The five groups were cerebral childhood and adolescent, pure adrenomyeloneuropathy (pure AMN), adrenomyeloneuropathy cerebral (AMN cerebral), Addison's only and symptomatic female heterozygotes.

Construct validity of the auditory continuous performance test for preschoolers.

Development of diagnostic instruments directed toward neuropsychological assessment of preschoolers lags significantly behind those available for school-age children (DeWolfe, Byrne, & Bawden, 2000). This is particularly true for measures of executive function (EF). The Auditory Continuous Performance Test for Preschoolers (ACPT-P; Mahone, Pillion, & Hiemenz, 2001) is a computerized, Go-No-go test developed to measure selected EF skills in preschoolers.

Characteristics of auditory agnosia in a child with severe traumatic brain injury: a case report.

We present a case that is unusual in many respects from other documented incidences of auditory agnosia, including the mechanism of injury, age of the individual, and location of neurological insult. The clinical presentation is one of disturbance in the perception of spoken language, music, pitch, emotional prosody, and temporal auditory processing in the absence of significant deficits in the comprehension of written language, expressive language production, or peripheral auditory function. Furthermore, the patient demonstrates relatively preserved function in other aspects of audition such as sound localization, voice recognition, and perception of animal noises and environmental sounds.

Age and task parameters in continuous performance tests for preschoolers.

66 children (M=56.2 mo., SD=10.

Prevalence of hearing loss in Rett syndrome.

This study examined the auditory status of females with Rett syndrome (RS) using auditory brainstem response (ABR) testing and measures of acoustic admittance. Prevalence of hearing impairment in 81 female patients (age range 1 year 3 months to 39 years 8 months; mean age 8 years 7 months, SD 6 years 4 months) was evaluated with ABR measurements. If the ABR latencies at 20dB nHL exceeded those of the normative group, a slight hearing loss was diagnosed.

Auditory brainstem response findings in Rett syndrome: stability over time.

Objective: To determine whether changes consistent with the presence of white matter neurodegenerative disease would be observed in the auditory brainstem response (ABR) in Rett syndrome in conjunction with age advancement.

Study Design: Initial and follow-up ABR findings were analyzed in a sample of 27 female patients with Rett syndrome. The interval between ABR tests ranged from 1 to 9 years.

Auditory brainstem responses in Rett syndrome: effects of hyperventilation, seizures, and tympanometric variables.

This study examined the effects of tympanometric variables, stage of disease, hyperventilation, and seizures on the auditory brainstem response (ABR) in Rett syndrome (RS). Thirty-four female children with RS ranging in age from 2 years, 3 months to 15 years, 7 months participated in the study. ABRs and tympanograms were recorded from all of the subjects.

Distortion product otoacoustic emission and auditory brain stem response measures of pediatric sensorineural hearing loss with islands of normal sensitivity.

Objective: To assess the accuracy of: 1) distortion product otoacoustic emission (DPOAE) measures for the identification of frequencies at which auditory sensitivity is normal or near normal; and 2) click and nonmasked tone burst-evoked auditory brain stem response (ABR) thresholds for behavioral threshold estimation for children with sensorineural hearing loss characterized by islands of normal sensitivity.

Design: DPOAEs and ABRs were recorded from five hearing-impaired and eight normal-hearing pediatric ears. The accuracy with which DPOAEs permitted identification of frequencies at which elevated hearing thresholds were present was examined.

Effects of frequency modulation on absolute, masked, and reflex thresholds.

Behavioral and acoustic reflex thresholds were determined for five normal-hearing subjects in response to carrier signals of 500 and 2000 Hz which were unmodulated or modulated sinusoidally at rates of 2, 20, and 200 times per second with frequency deviations (delta f) of 30, 100, and 300 Hz. Behavioral (absolute and masked) thresholds were determined using an adaptive two-alternative forced-choice procedure. Acoustic reflex thresholds were determined by visual inspection of stored reflex waveforms.