Maturation of auditory brainstem responses in young children with congenital monaural atresia.

OBJECTIVE: To date, the impact of conductive hearing loss on the auditory pathway at brainstem level has only been investigated in animal studies, which showed a species-specific delay of maturation. In this study, the functional maturation of auditory brainstem response (ABR) parameters in humans with unilateral atresia of the external auditory canal was investigated. METHODS: 42 newborns and toddlers ranging in age from 13 days to 11 months were included. The click-evoked ABR interpeak latencies (IPL) of the atretic ears and the contralateral ears with normal hearing were evaluated. The children had no comorbidities and had never been fitted with any kind of hearing aid. The absolute latencies (AL) and IPL of a matched control group were compared to the contralateral normally hearing ears of the children with unilateral atresia. RESULTS: The mean air-bone gap in the ears with atresia was 44 dB HL. Despite this partial acoustic deprivation, no significant difference between the IPLs of normal ears and ears with atresia could be detected. Both for AL and IPL, the differences between the normal ears and the control group were all within 1 standard deviation to the mean. CONCLUSION: The data showed that the monaural acoustic deprivation by a block of sound conduction does not produce any delay of functional maturation at brainstem level in this group of patients. With regard to the AL and IPL on brainstem level, no differences between the normal ears of children with unilateral atresia and children with bilateral normal hearing could be detected.

Evaluation of optimal masking levels in place-specific low-frequency chirp-evoked auditory brainstem responses.

The aim of the study is the experimental determination of the optimal required masking level for a given stimulus level when using a band limited "low-frequency chirp" in order to improve frequency and place specificity of auditory brainstem responses (ABRs). A low-frequency chirp (100-850 Hz) at stimulation levels between 40 and 80 dB normalized hearing level was presented to 12 normal hearing subjects. During presentation of each stimulus, the level of a high-pass noise with a low cutoff frequency of 1100 Hz was varied between 0 and 25 dB signal-to-noise ratio (SNR) by using 5 dB steps (at 0 dB SNR the same level of both the chirp and the masker in dB sound pressure level was presented). Measurements without masking were used as a reference. In all masking conditions, the latency of wave V was significantly increased compared to unmasked ABRs. The amplitude of wave V decreased when reaching the effective and therefore optimal masking level. Accordingly, in order to ensure place specificity of the ABR, ipsilateral masking is essential. At lower stimulus levels the SNR can be substantially increased (i.e., the masker level decreased) without loss of place specificity.

Electrocochleography in children with auditory synaptopathy/neuropathy: diagnostic findings and characteristic parameters.

INTRODUCTION: The early diagnosis of AS/AN in children remains challenging because it exclusively relies on the detection of OAE and/or CM, while ABR are pathologically changed or missing. The aim of our study was to ensure the diagnosis of AS/AN, demarcate it to an outer hair cell damage and possibly differentiate between pre- and postsynaptic pathologies. METHODS: We retrospectively evaluated the transtympanic ECochG results of ten children with AS/AN and compared them to a matched group with SNHL and without any signs of AS/AN. We analyzed the thresholds, latencies and - as a new parameter - the amplitude ratio between CAP and SP. RESULTS: CM and SP thresholds were significantly lower than CAP thresholds in AS/AN patients and significantly lower than SP and CM thresholds in SNHL patients with comparable CAP thresholds. The CAP/SP ratio of amplitudes in SNHL children was more than three times (significantly) higher than in AS/AN children. The cutoff value was set at 1.0 in order to differentiate between both groups with a 80-90% sensitivity and specificity. It was not possible to differentiate between a pre- and postsynaptic type of AS/AN in our collective. SUMMARY AND CONCLUSION: The ECochG can add valuable information for a precise differential diagnosis of AS/AN, especially in babyhood. We identified the CAP/SP ratio as a new parameter for differentiation between AS/AN and SNHL. When the CAP/SP ratio falls below 1.0, patients can be diagnosed AS/AN with high specificity and sensitivity. Significantly smaller SPL are needed to evoke SP and CM in the AS/AN group, thus showing the preserved hair cell function.

The correlation between ECochG parameters and early auditory behavior after cochlear implantation in children.

OBJECTIVE: The individual outcome after cochlear implantation in children with auditory synaptopathy/neuropathy (AS/AN) is difficult to predict. A tool for preoperative assessment would be helpful for counseling parents. This study evaluates the outcome after CI in children with AS/AN and with sensorineural hearing loss (SNHL), and correlates it with the preoperative ECochG results in order to find specific parameters of prognostic value. DESIGN: The improvement of auditory behavior after CI was retrospectively assessed using the LittlEARS questionnaire and quantified in a score (LS). This score was correlated with the CAP/SP ratio in the preoperative ECochG. The score was further correlated with the patient's age six months following CI. STUDY SAMPLE: Nine children with AS/AN were compared to nine children with SNHL. RESULTS: Both groups showed a significant improvement in LS following CI. There was a significant positive correlation between the CAP/SP ratio and the improvement in LS in all children. The correlation between age and LS was significantly negative in the SNHL group and positive in the AS/AN group. CONCLUSION: All children with AS/AN and SNHL benefit to a similar extent from CI. The preoperatively assessed CAP/SP ratio has a prognostic value for the development of auditory behavior following CI.

Case report of a child with otoacoustic emissions and profound hearing loss in whom otoacoustic emissions were preserved after cochlear implantation.

The management of patients characterised by the presence of otoacoustic emissions and/or cochlear microphonics suggesting normal outer hair cell function in conjunction with absent or grossly abnormal auditory brainstem responses is often associated with particularly poor response to amplification. Cochlear implantation has been shown to be an option in affected patients. Here, we report a case of successful cochlear implantation and preserved otoacoustic emissions in a child suffering from this hearing disorder.

Risk factors for auditory neuropathy/auditory synaptopathy.

AIMS: It was the aim of this study to describe risk factors in auditory neuropathy/auditory synaptopathy (AN/AS). METHODS: Between 1997 and 2005, we diagnosed 37 children with AN/AS. They underwent a critical chart review for risk factors and etiological coincidences in this idiosyncratic disorder. RESULTS: Eighteen neonates had a history of prematurity and low birth weight. Hyperbilirubinaemia was present in 13 children. Three patients had evidence of infection during pregnancy, and AN/AS was associated with complex syndromal diseases in 2 cases. A congenital, familial pattern was seen in 2 siblings. Seven patients had idiopathic AN/AS. CONCLUSION: Rather than being a single etiological entity, AN/AS comprises a spectrum of risk factors and associated problems affecting the cochlea and the auditory pathway. This study shows that the majority of AN/AS in children is the result of perinatal problems and is not genetic in origin. Hyperbilirubinaemia is a common and etiologically significant finding in infants suffering from AN/AS. Thus, early hearing screening for AN/AS including transient evoked otoacoustic emissions and auditory brainstem response assessment among neonates with risk factors for AN/AS is crucial in order to better manage patients suffering from this disorder.

Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing loss.

OBJECTIVE: To examine the prevalence of auditory neuropathy/synaptopathy (AN/AS) in a cohort of children with profound hearing loss. METHODS: From 1997 until 2004, 5190 children, aged 1-15 years, whose hearing ability was uncertain or who had risk factors for hearing impairment were investigated with subjective and objective hearing tests. Three thousand four hundred and fifteen from these children were screened for AN/AS using pure-tone audiometry, impedance measurement, transient evoked otoacoustic emissions (TEOAE) and click-evoked auditory brainstem responses (ABR). RESULTS: From 3415 patients who participated in an ABR and TEOAE assessment, 379 children showed absent or elevated (> or = 80 dB nHL) ABR thresholds. Within this group we found 32 cases with evidence of AN/AS via visible TEOAE and/or cochlear microphonics (CM) coupled with absent ABR. In the remaining 3036 children, AN/AS, could be ruled out by means of detectable ABR-thresholds and coherent findings in pure-tone audiometry and TEOAE assessment. This results in a prevalence of AN/AS of 0.94% within the group at risk for hearing loss, compared to 8.44% among profoundly hearing impaired children. CONCLUSION: This study shows that AN/AS is a common finding in the population of hearing impaired infants. In the majority of our AN/AS children (50%, n=16), an early audiological diagnosis was made under the age of 12 months. Therefore, clinicians and other health care professionals should generally be sensitised for AN/AS in infants, so that an appropriate treatment can promptly be initiated. Further research on clinical and pathophysiological aspects is necessary to better identify and manage patients suffering from AN/AS.

Hearing loss in early infancy affects maturation of the auditory pathway.

The influence of early cochlear hearing loss on maturation of the auditory pathway was studied by measuring auditory brainstem responses (ABR). In a retrospective study, 85 children with normal hearing (46 males, 39 females; age range 2 months to 14 years) and 165 children with binaural cochlear hearing impairment (89 males, 76 females; age range 1 month to 16 years) were examined. A significant positive correlation (p<0.001) between the degree of hearing loss and interpeak latencies I-V (IPL(I-V)) of the ABR was observed. No significant correlation (p=0.85) was found between hearing loss and interpeak latencies I-III (IPL(I-III)). These findings can be interpreted as indicating a marked delay in maturation of higher brainstem structures due to reduced auditory input during infancy. The correlation differs notably from results of comparable studies of adults published in recent literature. This leads to the assumption that the developing human brain is particularly sensitive to auditory deprivation. Thus, our results indicate the importance of a normal acoustic environment during sensitive periods in early childhood to ensure normal hearing and speech development.