Hearing loss in congenital diaphragmatic hernia (CDH) survivors: is it as prevalent as we think?

PURPOSE: The incidence of sensorineural hearing loss (SNHL;>20 dB loss) in CDH survivors is debated. We evaluated long-term audiological outcomes at a single tertiary care center with ECMO capability and an established neonatal follow-up program. METHODS: With REB approval, records of CDH survivors from 2000 to 2010 were retrospectively analyzed. Demographic, postnatal, and audiometric information was gathered. All underwent auditory brainstem response (ABR) or otoacoustic emissions screening before discharge and complete audiological surveillance. Thirty-three patients were evaluated to age 4+ years with others continuing follow-up. RESULTS: Forty-three patient records were reviewed with 1 excluded (transferred to another institution). Median GA and BW were 39 weeks (35-41) and 3.1 kg (2-4), respectively. Median ventilation days were 10 (2-189) with 34 infants ventilated 5+ days. Sixteen (36%) received HFOV, 21 (49%) iNO, and 5 (12%) ECMO. The median time to CDH repair was 3 days (1-23), and 11 (26%) required patch repair. Nine infants (21%) received diuretics and oxygen after discharge. Audiological surveillance identified only one patient with SNHL (received HFO, iNO, and patch repair). CONCLUSION: Neonatal screening identifies CDH survivors at risk for hearing difficulties but must be followed with comprehensive testing until school age. The incidence of SNHL may be less than previously reported in this population.

Developmental trajectories of infants and toddlers with good initial presentation following moderate or severe traumatic brain injury: a pilot clinical assessment project.

OBJECTIVE: The purpose of this study was to review the feasibility and usefulness of instituting a clinical protocol of scheduled assessments for children after a moderate or severe traumatic brain injury (TBI) sustained before the age of 2 years and showing no immediate deficits at hospital discharge, as well as to explore the early developmental trajectories of these children. DESIGN: Exploratory analytical cohort study. SETTING: Pediatric Trauma Center Out-patient services. PARTICIPANTS: 31 children were followed within the clinical protocol of scheduled assessments. OUTCOME MEASURES: The protocol included an immediate post-injury clinical assessment of infants who sustained a TBI and follow-up assessments at the ages of 9 months, 18 months (if injured prior to that age), 30 months, and 42 months. Domains assessed at each scheduled visit included hearing, speech and language, motor performance, personal social abilities, and adaptive behaviors. RESULTS: Clinicians reported few difficulties with scheduling or administering the assessments, maintaining a 67% participation rate at the end of the follow-up period, thus demonstrating the feasibility of the protocol in this population. Scores on the majority of formal tests showed high variability and 15-20% of children presented with clinically significant motor and/or language delays. By 42 months of age, difficulties with adaptive behavior and personal social abilities were identified in our sample of children when compared to published norms. Qualitative clinical findings from professionals identified between 25-50% of children with potential attentional difficulties throughout the follow-up period. CONCLUSION: Findings from this study demonstrate the feasibility of implementing a clinical protocol of assessment for infants and toddlers who sustain a TBI before the age of 2 years and present with no impairments at the time of discharge from hospital. Developmental problems in this population appear to be easier to identify later in the toddler years as opposed to immediately following the TBI, emphasizing the importance of providing screening for developmental issues in this population prior to school entry.

Auditory neuropathy in siblings with Waardenburg's syndrome.

We report two siblings with a family history of Waardenburg's syndrome (WS) for whom the audiological profile corresponds to auditory neuropathy (AN). They have; (1) bilateral severe to profound hearing loss, (2) robust oto-acoustic emissions (OAEs) in both ears, and (3) no auditory evoked responses at 95 dBnHL bilaterally. Electrocochleography (ECochG) and auditory middle and late latency potentials were performed in one of the children. Results showed cochlear and neural activities in both ears. Central auditory responses were not conclusive. These children did not have any history of neonatal illness and one child was diagnosed with AN at the age of 3 weeks and the other at the age of 11 months.