Appropriate hearing screening in the pediatric patient with head trauma

Purpose

To develop an algorithm for the appropriate audiologic screening of in children with head trauma.

Methods

Participants were the first consecutive 50 children admitted to a children's hospital trauma service after October 1, 2005, whose injuries resulted in a Glasgow Coma Scale (GCS) score ≤13 and/or loss of consciousness (LOC) but no history of hearing loss. Screening tympanometry, otoacoustic emissions testing, and/or routine audiometric evaluation were performed as soon as possible after admission. Age, GCS score, Pediatric Trauma Score, Injury Severity Score, presence of head and neck soft tissue injury, temporal bone fracture, skull fracture not involving the temporal bone, midface/mandible fractures, intracranial abnormality on computed tomography, and cervical fracture were analyzed as risk factors for hearing loss.

Results

Seventeen (34%) of the 50 children had abnormal hearing test results. Fischer's exact test showed abnormal test results were most strongly related to temporal bone fracture (p = 0.0041), non-temporal bone skull fracture (p = 0.0211) and younger age (p = 0.0638).

Conclusions

Any child with head trauma and clinical or radiologic evidence of temporal bone fracture should have early hearing evaluation. Using the proposed algorithm to test children with head trauma and GCS ≤13 and/or LOC and age <3 years or any type of skull fracture may help identify children with hearing loss in a more cost effective manner.     

Eustachian tube function in patients with Meniere's disease

Objective

The aim of the present study was to clarify the relationship between Eustachian tube function and inner ear function, especially with respect to the hearing ability of patients with Meniere's disease.

Methods

Patients with Meniere's disease underwent nystagmic examinations and audiometric measurements, including hearing tests, tympanometry, and Eustachian tube function tests (sonotubometry). We compared the audiometric examination results of normal subjects to those of patients with Meniere's disease.

Results

Twenty-five percent of patients with Meniere's disease exhibited Eustachian tube dysfunction, but 92% displayed normal tympanometry findings. Their sonotubometry durations and amplitudes were not significantly different from those of normal subjects. However, the patients’ hearing level was significantly correlated to sonotubometry duration and amplitude. Our patients were classified according to the four stages of Meniere's disease: stage 1 (n = 9); stage 2 (n = 5); stage 3 (n = 8); and stage 4 (n = 2). The incidence of Eustachian tube dysfunction in these four groups of patients were 0% (0/9); 40% (2/5); 38% (3/8); and 50% (1/2), respectively.

Conclusion

Our study provides evidence demonstrating that treatment of Eustachian tube dysfunction may be useful in preventing the hearing of Meniere's patients from becoming worse.     

A normative study of otoacoustic emissions, ear asymmetry, and gender effect in healthy schoolchildren in Slovakia

Objective

The aim of this normative study was to examine cochlear status and possible ear asymmetry and gender effect in transient evoked and distortion product otoacoustic emissions in a group of healthy 12-year-old children in Slovakia.

Methods

Two hundred and twenty-nine 12-year-old children from Slovakia with normal hearing were included in this study. Adolescents with acute infection, abnormal otoscopic findings and abnormal tympanometry were excluded. Pure tone audiometry was performed in standard conditions in a sound proof room. Recordings of transient evoked (TE) and distortion product otoacoustic emissions (DPOAEs) were performed using an ILO 292 USB Echoport. Parameters of hearing thresholds and OAEs were compared using correlation analysis and Wilcoxon test.

Results

We found highly statistically significant associations between the hearing thresholds for the left and right ears. When comparing pure tone audiometry with OAEs no significant correlation was found. In TEOAE a significant gender effect and side effect in TEOAE SNR were found. On the other hand there was no side effect in TEOAE response level. In DPOAE neither gender nor side effects were determined.

Conclusions

This is the first comprehensive information on cochlear status among Slovak adolescents. The TEOAEs were significantly higher in girls than boys, but the ear asymmetry in TEOAE was not significant. For DPOAE responses ear asymmetry and gender did not play a role. The data obtained are a basis for population hearing screening, especially for hearing screening programs in infants and children in Slovakia. Moreover data from particular age group represent a link between data from infants and adults.     

Congenital incudostapedial anomalies in adult stapes surgery: a case-series review

Objectives

The management of developmental anomalies of the ossicular chain and otic capsule in stapes surgery tests the otologist's diagnostic skills and flexibility in technique and prosthesis choice. We review our experience in managing various incudostapedial developmental anomalies discovered during middle ear exploration for suspected otosclerosis in adult patients.

Design

A retrospective case-series review was used.

Setting

The study was set at a military tertiary referral center.

Patients and other Participants

A review of 185 stapes procedures yielded 8 cases of identified congenital anomalies of the stapes or incudostapedial complex including congenital absence of the oval window with malformed incus and stapes, columellar-type stapes with promontory fixation, stapes superstructure fixation to the fallopian canal, and malformed incus with stapes superstructure fixation to the promontory.

Interventions

The developmental anatomy and surgical technique in each case were reviewed. The surgical technique used in each case varied depending on the specific ossicular abnormality found and whether both the stapes and incus were affected.

Main outcome measures

Comparison of preoperative and postoperative audiometric testing results was performed.

Results

All 8 ears (7 patients) that underwent a stapes procedure for either an isolated congenital stapes anomaly or a combined incudostapedial anomaly had a favorable hearing outcome, with 7 ears improving to within 10-dB and 1 ear to within 13-dB air-bone gap on postoperative audiometry.

Conclusions

Despite unanticipated findings of congenital anomalies of the stapes and incus during middle ear exploration for conductive hearing loss, a flexible management approach can lead to successful hearing outcomes.     

Screening for pre-school and school-age hearing problems: European Consensus Statement

Objectives

To formulate consensus statement and policies on structured hearing screening programs in pre-school and school-age children in Europe. This consensus will be brought before the European Union's Member States as a working and effective program with recommendations for adoption.

Methods

A distinguished panel of experts discussed hearing screening of pre-school and school-age children during the 10th Congress of European Federation of Audiology Societies (EFAS), held in Warsaw, Poland, on June 22, 2011. The panel included experts in audiology, otolaryngology, communication disorders, speech language pathology, education and biomedical engineering.

Results

Consensus was reached on thirteen points. Key elements of the consensus, as described herein, are: (1) defining the role of pre-school and school screening programs in the identification and treatment of hearing problems; (2) identifying the target population; (3) recognizing the need for a quality control system in screening programs.

Conclusions

The European Consensus Statement on Hearing Screening of Pre-school and School-age Children will encourage the appropriate authorities of the various countries involved to initiate hearing screening programs of pre-school and school-age children.     

Measuring communicative performance with the German version of the FAPCI-instrument: normative data and longitudinal results

Objective

The German version of the Functioning after Paediatric Cochlear Implantation (FAPCI) inventory was designed to capture the communicative performance of cochlear implanted children. In order to be able to compare cochlear-implanted children to their normal hearing peers, normative growth curves were designed. Furthermore it was of interest how the communicative performance develops over time and whether it is influenced by age at implantation.

Method

A polynomial regression curve was fit to the data of 133 normal hearing children. This normative curve was compared to individual growth curves of 90 cochlear implanted children. The cochlear-implanted study sample was split up into four groups depending on the age at implantation.

Results

The normative growth curve increases from 1 year of age until saturation is reached with 3 years of age. The individual FAPCI trajectories of cochlear implanted children are heterogeneous, but in general they are delayed in comparison to the normative growth curve. “Early implanted children” follow the development of their normal hearing peers more closely than “later-implanted children”.

Conclusion

The German version of the FAPCI parental questionnaire constitutes an additional instrument in monitoring the communicative performance development of cochlear implanted children and allows for comparison to normal hearing peers.     

Characteristics of hearing impairment in Yemeni children with chronic suppurative otitis media: A case-control study

Background

Chronic suppurative otitis media (CSOM) is a serious disorder particularly in low resource settings. It can lead to disabling hearing impairment and sometimes life-threatening infective complications.

Objective

The aim of the present study was to describe the characteristics of hearing impairment associated with CSOM in Yemeni children.

Methods

A case-control study of 75 children with CSOM and 74 healthy controls. Hearing was assessed by behavioural testing and audiometry.

Results

Cases had lower academic performance than controls (OR 15.31, 95% CI 1.99-322.14, p < 0.001). Disabling hearing impairment >30 dB was present in 51.5% (right ear) and 66.7% (left ear) of children with CSOM.

Conclusion

Disabling hearing impairment was identified as a major health problem in these Yemeni children with CSOM. There is a need for investment to reduce the burden of CSOM and its complications in these communities. Greater attention to the chronic disabling effects of CSOM in children is required in poor communities and low resource settings.     

Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease

Objectives

In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use.

Methods

Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied.

Results

Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p < 0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p < 0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value = 2.034; p = 0.033).

Conclusions

To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.     

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree

Objective

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type.

Methods

Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15 dB hearing loss averaged over 0.5, 1 and 2 kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4 kHz, and classified into four categories: mild, moderate, severe and profound.

Results

Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss.

Conclusions

The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease.     

Evaluation of cochlear involvement by transient evoked otoacoustic emission test in children with Crimean-Congo hemorrhagic fever

Objective

The aim of this study was to investigate cochlear involvement in child patients with Crimean-Congo hemorrhagic fever (CCHF) disease.

Methods

Twenty-eight CCHF disease patients (56 ears) and 26 sex- and age-matched healthy control subjects (52 ears) were included in the study. Pure-tone audiometry at frequencies 0.25, 0.5, 1, 2, 4, and 6 kHz, immittance measures including tympanometry and acoustic reflex testing, and transient evoked otoacoustic emission (TEOAE) testing were performed in the patients and controls.

Results

The proportion with a result of ‘fail’ for the TEOAE test in the CCHF patients was not statistically significant from the control group (p > 0.05).

Conclusions

CCHF disease does not impair cochlear function in children. The clinical course of CCHF among children seems to be milder than in adults.     

Hearing assessment in high-risk congenital diaphragmatic hernia survivors

Objective

To report results of audiometric evaluations in high-risk congenital diaphragmatic hernia survivors and their exposure to audiological risk factors (mechanical ventilation, high frequency oscillation, aminoglycoside therapy and neuromuscular blocking agents).

Design

All newborns with high-risk congenital diaphragmatic hernia born between January 2003 and June 2009 were treated consecutively at the Neonatal Intensive Care Unit, Pediatric Hospital, University of Padova. Thirty-two survived and 26 of them underwent formal audiological evaluation (tonal and speech audiometry, otoacoustic emission, and immitance measurements) and follow up.

Results

Twenty-one children had normal hearing; 4 had conductive hearing loss, which was successfully treated; and 1 had severe sensorineural hearing loss and suffers from Turner syndrome.

Conclusions

Our series revealed a lower prevalence of sensorineural hearing loss in high-risk congenital diaphragmatic hernia survivors than in other studies, suggesting that the association between hearing loss and congenital diaphragmatic hernia has yet to be accurately defined and fully elucidated.     

Audiological chronological findings in children with congenital anomalies of the central nervous system

Objective

To determine the frequency of hearing impairment in children with congenital anomalies of the central nervous system (CNS) by using detailed audiological evaluation methods.

Methods

The patients were 78 children with congenital anomalies of the CNS with a mean age of 29.5 months. They had been observed for a mean period of 38.5 months. Hearing levels were evaluated behavioral observation audiometry (BOA), visual reinforcement audiometry (VRA) and distortion product otoacoustic emissions (DPOAEs) were performed. Auditory brainstem responses (ABRs) and computed tomography (CT) scans of the temporal bone were performed in the cases in which the minimum response levels (MRLs) were above 30 dBHL. All cases were assessed in terms of developmental age.

Results

A total of 14.1% (11/78) of the children with congenital anomalies of the CNS were initially diagnosed with bilateral sensorineural hearing loss (SNHL). However, the hearing levels of nine of them improved by the time of the last diagnosis. Therefore, the patients with bilateral SNHL were only 2.6% (2/78) of the total patients with congenital anomalies of the CNS at last diagnoses. As shown by our results, many children with bilateral SNHL at initial diagnosis showed improved ABR thresholds and behavioral hearing thresholds with age. In this series, the use of hearing aids was arranged for six patients. However, four patients stopped using hearing aids when their hearing threshold levels improved. In two cases, there were no changes in hearing levels and the children continued using hearing aids.

Conclusion

Our results suggest that hearing level recovery can occur in some children with CNS anomalies. Confirmation of hearing loss in children with congenital anomalies of the CNS takes a long time. There are improvements in hearing loss during the observation period. Therefore periodic assessment of hearing is important.     

National Newborn Hearing Screening Program in Turkey: Struggles and implementations between 2004 and 2008

Objective

In this review, we have presented the data of our National Newborn Hearing Screening Program (NNHSP) with total 764,352 newborns those screened in last five years.

Methods

National Newborn Hearing Screening Program (NNHSP) has been conducted in Turkey since the year 2003. National Newborn Hearing Screening Program (NNHSP) had begun at the end of 2003 only in 1 center. After birth, in the third day, Transient Evoked Otoacoustic Emissions (TEOAEs) test criteria and if necessary, auditory brain response (ABR) testing evaluation methods were applied to newborn. The children diagnosed with hearing loss were further referred for advanced treatment and rehabilitation to advanced audiologic centers.

Results

After five years of carrying out the program (between 2004 and 2008) a total number of 764,352 newborns were screened for hearing impairment. In the year 2008, National Newborn Hearing Screening Program (NNHSP) had given the chance for 2136 children with various types of hearing loss (320 with unilateral and 417 with bilateral hearing loss) to detect and refer to more experienced centers for further treatment.

Conclusions

Our results indicate that the necessity of newborn hearing screening is an indispensable issue. We have been targeted to develop National Newborn Hearing Screening Program (NNHSP) till given chance to access for every newborn in Turkey in next five years.     

Non-organic hearing loss in childhood

Objective

The present study aimed to investigate the etiology, symptoms, diagnosis and prognosis of pediatric patients with non-organic hearing loss (NOHL), and to heighten awareness of this disorder among physicians.

Methods

Between January 2000 and July 2009, we retrospectively reviewed the medical records of 47 pediatric patients (aged 6-18 years of age) diagnosed with NOHL. The diagnosis was made when there were audiometric discrepancies between the subjective and objective hearing thresholds of the patient in the absence of any organic disease.

Results

Eighteen patients presented with unilateral hearing loss, and 29 showed bilateral hearing loss. Five patients received steroid treatment before the correct diagnosis was made, and six had secretory otitis media and underwent a tympanostomy tube placement.

Conclusion

If physicians are unaware of the possibility of NOHL; they may misdiagnose children with idiopathic sudden sensorineural hearing loss and administer high-dose steroid treatments or exploratory tympanotomies. Otoacoustic emissions are abolished when NOHL patients have secretory otitis media. In these cases, after tympanostomy tube placement, they should undergo objective electrophysiologic examinations to reevaluate NOHL.     

Hearing status in children with congenital cytomegalovirus: up-to-6-years audiological follow-up

Objective

To evaluate the audiological outcome of children with congenital cytomegalovirus infection.

Methods

In a prospective study, the hearing of ninety seven congenitally cytomegalovirus-infected children, born between January 2003 and July 2009, was systematically evaluated until the age of six, applying the Flemish CMV protocol. Depending on the age of the child, the protocol provides hearing evaluation by objective-, play- or conventional audiometry. Symptomatic children with hearing loss at birth were treated with ganciclovir, if parents consented.

Results

Seventy children had a pass on initial screening, 27 had unilateral or bilateral hearing loss. Within the normal hearing group, one asymptomatic and two symptomatic children developed late-onset hearing loss. Within the group with hearing loss, 8 children received ganciclovir, while 8 symptomatic and 11 asymptomatic children did not receive ganciclovir. As for the treated group, 37.5% of the children had stable hearing loss, one child had progressive and one child had fluctuating hearing loss. Improvement of hearing threshold occurred in 37.5% of the children. Among the untreated symptomatic children, hearing loss remained stable in 50%, while progression occurred in 37.5%. In the group of asymptomatic children with hearing loss, hearing loss was most commonly stable (72.7%).Within the group of normal hearing ears at birth (n = 156), there is a significant better progression in pure tone average for ears of asymptomatic subjects in comparison to ears of symptomatic subjects (p ≤ 0.0001). As for the group of ears with hearing loss at birth (n = 38), analysis shows no evidence for a difference in pure tone average progression between the different groups (p = 0.38).

Conclusions

Cytomegalovirus infection may cause hearing loss, in both symptomatic and asymptomatic children. Our data show a significant difference, between both groups, in the progression of pure tone average of normal hearing ears at birth, in favor of the asymptomatic children. This is not the case for ears with hearing loss at birth. However, this may be due to the small number of ears in this group. Our data show the tendency that treatment with ganciclovir increases the likelihood of improvement and reduces the likelihood of deterioration of the hearing.     

Threshold prediction in children with sensorioneural hearing loss using the auditory steady-state responses and tone-evoked auditory brain stem response

Objective

The purpose of this study was to compare ASSRs to tone-evoked ABR and to behavioral thresholds obtained on follow-up audiometry at 500, 1000, 2000, and 4000 Hz in infants and young children.

Methods

The study included 17 infants and young children ages between 2 months and 3 years old, with sensorineural hearing loss. The ASSRs thresholds were compared with the tone-evoked ABR thresholds, and with the behavioral thresholds obtained on follow-up audiometry.

Results

The correlation of tone-evoked ABR and ASSRs thresholds at 500, 1000, 2000 and 4000 Hz was 0.91, 0.76, 0.81, 0.89, respectively. ASSRs and behavioral hearing thresholds obtained on follow-up were highly correlated, with Pearson r values exceeding 0.94 at each of the test frequencies.

Conclusions

Multiple ASSRs have strong correlations to tone-evoked ABR and to behavioral thresholds obtained during follow-up in hearing impaired infants and young children. These results might be useful in order to provide further evidence for the use of multiple ASSRs, as an alternative tool to tone-evoked ABR, although further data are still required.     

Measuring communicative performance with the FAPCI instrument: preliminary results from normal hearing and cochlear implanted children

Objective

: To develop preliminary “growth curves” of Functioning after Pediatric Cochlear Implantation (FAPCI) scores using a cross-sectional sample of normal hearing children and to compare these curves to trajectories of FAPCI scores in children receiving cochlear implants.

Methods

: Quantile regression was used to develop growth curves from the FAPCI scores of a cross-sectional sample of 82 normal hearing children (age range 7 months-5 years). Trajectories of FAPCI scores from a longitudinal cohort of 75 children with cochlear implants (age range 1-5 years) were compared to these growth curves.

Results

: FAPCI scores were positively associated with increasing age in normal hearing children with a rapid increase in scores observed at earlier ages followed by a plateau at age 3 years. FAPCI trajectories for cochlear-implanted children varied with age at implantation and did not reach a plateau until age 5-6 years.

Conclusion

: Normal hearing children demonstrated increasing FAPCI scores with age, and these preliminary growth curves allow for the interpretation of a cochlear-implanted child's FAPCI scores in comparison to normal hearing children. Additional research using a larger, longitudinal cohort of normal hearing children will be needed to develop definitive normative FAPCI trajectories.