Hearing threshold estimation by Auditory Steady State Responses (ASSR) in children

Hearing threshold identification in very young children is always problematic and challenging. Electrophysiological testing such as auditory brainstem responses (ABR) is still considered the most reliable technique for defining the hearing threshold. However, over recent years there has been increasing evidence to support the role of auditory steady-state response (ASSR). Retrospective study. Forty-two children, age range 3-189 months, were evaluated for a total of 83 ears. All patients were affected by sensorineural hearing loss (thresholds ≥ 40 dB HL according to a click-ABR assessment). All patients underwent ABRs, ASSR and pure tone audiometry (PTA), with the latter performed according to the child’s mental and physical development. Subjects were divided into two groups: A and B. The latter performed all hearing investigations at the same time as they were older than subjects in group A, and it was then possible to achieve electrophysiological and PTA tests in close temporal sequence. There was no significant difference between the threshold levels identified at the frequencies tested (0.25, 0.5, 1, 2 and 4 kHz), by PTA, ABR and ASSR between the two groups (Mann Whitney U test, p < 0.05). Moreover, for group A, there was no significant difference between the ASSR and ABR thresholds when the children were very young and the PTA thresholds subsequently identified at a later stage. Our results show that ASSR can be considered an effective procedure and a reliable test, particularly when predicting hearing threshold in very young children at lower frequencies (including 0.5 kHz)

Auditory Neuropathy Spectrum Disorder in the White Sutton Syndrome

White Sutton Syndrome is a rare autosomal dominant disorder resulting from a de novo mutation of Pogo Transposable Element Derived with Zinc Finger domain gene. The phenotype is characterized by a wide spectrum of cognitive dysfunction and developmental delays. Hearing loss is frequently mentioned as one of the symptoms of this rare disease, but details are usually scant. We report a case of a male child affected by White Sutton Syndrome and sensorineural hearing loss, with audiological findings of an auditory neuropathy spectrum disorder, a dysfunction of the auditory pathway with preserved cochlear outer hair cell function. Up to date, the present case is the first description of hearing loss due to an auditory neuropathy spectrum disorder in White Sutton Syndrome. A comprehensive audiological assessment is therefore mandatory in all White Sutton Syndrome patients in order to recognize a possible auditory neuropathy disorder and then avoid misdiagnosis, or erroneous clinical management

Hearing Loss in Children: Clinical-Epidemiological Data from Two Different Provinces of the Same Region

Background: In many countries, neonatal hearing screening programs (NHS) have been available for many years; however, because of the presence of hearing loss at late onset, early hearing detection programs (EHDP) have been implemented. The aim of this study was to evaluate all cases of infantile hearing loss under the care of two different provinces of a regional health service since the introduction of NHS. Methods: Clinical data (the presence of audiological risk factors, age at which children are placed under the care of health service, entity of hearing loss, treatment, and exposure to bilingualism) were retrospectively collected during the period from 1 January 2012 to 31 December 2018, starting from the IT management system used in all of the regional neuropsychiatric services. Results: In total, 124 children were included-116 cases failed the screening, 1 case had an untraceable result, and 7 cases (5.6%) had hearing screening that passed. Most of the children were placed under the care of a neuropsychiatric infantile and adolescence (NPIA) service within the first year of life. The main differences across the two provinces concerned the percentages of audiological risk factors and the number of unilateral hearing loss cases. Conclusion: In order to plan and manage hearing rehabilitation programs for children in the best way, it is very important to know the local clinical-epidemiological features of the population