Bone-anchored hearing aids (Baha) in congenital aural atresia: personal experience

Aims

Aural atresia is a congenital disease constituted by partial or complete lack of development of the external auditory canal, which is generally associated with malformations of the auricle and middle ear.Reconstruction of the auditory canal and correction of any deformities of the middle ear have yielded unpredictable results and variable functional outcomes, and there is a high rate of complications. Therefore, the use of bone-conduction hearing aids, such as the Baha, may represent a valid alternative for subjects who have conductive hearing loss with cochlear reserve that, as a rule, is fully conserved.The aim of this work is to reexamine our experience with the management of conductive and mixed hearing loss using the Baha system in children with bilateral aural atresia.

Methods

We examined 31 patients with bilateral congenital aural atresia in whom a Baha system had been implanted. The patients, 16 males and 15 females, were between 5 and 14 years of age (mean 8.7).The following parameters were assessed for each patient: mean preoperative air and bone conduction for frequencies between 0.5 and 4 kHz; mean preoperative threshold with conventional bone-conduction hearing aids; mean postoperative threshold with the Baha system; improvement in quality of life evaluated with the Glasgow Children's Benefit Inventory; rate and type of surgical complications.

Results

The mean preoperative air- and bone-conduction thresholds were 51.2 ± 12.5 and 14.1 ± 6.3 dB HL, respectively. The mean preoperative threshold with a conventional bone-conduction hearing aid was 29.3 ± 7.2 dB HL, and the mean postoperative threshold with the Baha system was 18.1 ± 7.5 dB HL. Quality of life improved for all operated patients.

Conclusions

The results of our study of the Baha system to treat patients with bilateral aural atresia were extremely satisfactory compared both with those of surgical reconstruction of the auditory canal and those of traditional bone-conduction hearing aids. Furthermore, great improvement was noted in quality of life, while the rate of complications was very low.Therefore, we are convinced that the Baha system is the treatment of choice for hearing loss due to bilateral congenital aural atresia.     

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.     

Complications of bone-anchored hearing aids in pediatric patients

Objective

To describe the range and rate of complications related to bone-anchored hearing aids in pediatric patients.

Method

We conducted a retrospective review all children 16 years of age or less who were implanted with a bone-anchored hearing aid from 2002 to 2009. The main outcome measures were post Baha^® implantation complications including infection, soft tissue hypertrophy, loss of osseointegration, and need for further surgery.

Results

Thirty-one patients were identified and 27 had sufficient follow up for complete analysis. Soft tissue reactions were seen in 24 patients (89%), half of which were considered minor. Ten patients (37%) had major complications defined as requiring revision surgery or removal of the implant. Soft tissue overgrowth and infection at the abutment required removal in 5 children (19%) and three implants (11%) failed to osseointegrate. Recurrent antibiotic treatment was required in eight patients (30%). Seven patients (26%) had a history of trauma and one of them lost the implant. To date, three of the children (11%) have been unable to use the device because of chronic infection and overgrowth of the abutment. There was a statistically significant increased risk for major complications in patients from socioeconomically deprived backgrounds and obesity appears to incur additional risk.

Conclusions

Complications of bone-anchored hearing aid implants are common in our experience and obesity and socioeconomic factors appear to contribute to a higher risk for complications. Frequent follow up and meticulous care of the implant site may minimize complications but can be challenging in this population.     

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.     

The Baha Softband. A new treatment for young children with bilateral congenital aural atresia

The Baha (bone-anchored hearing aid) Softband appears to be an effective mean of hearing rehabilitation for children with a congenital bilateral aural atresia who are too young for the amplification of a Baha on an implant. The aided hearing threshold with a Baha Softband is almost equal to that achieved with a conventional bone conductor. The speech development of the children studied with a Baha Softband is on a par with peers with good hearing.     

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.     

Bone-anchored hearing aid (Baha) in patients with Treacher Collins syndrome: tips and pitfalls

Objectives

Treacher Collins syndrome, also known as mandibulofacial dysostosis, is an autosomal dominant disorder of the cranio-facial morphogenesis affecting 1 of 50,000 live newborns. Most children with this disease present with bilateral, severe conductive hearing loss due to bilateral aural atresia. Auditory rehabilitation of these children can be effectively carried out with bone-anchored hearing aids (Baha). The aim of this retrospective study is to review the “Bambino Gesù” Children's Hospital's experience with Baha in Treacher Collins patients, highlighting the tips and pitfalls of Baha surgery in this particular population.

Methods

The clinical charts were reviewed of all children with a Treacher Collins syndrome diagnosis receiving a Baha in the “Bambino Gesù” Children's Hospital from January 1995 to January 2010. Data were collected concerning patients’ anagraphics and medical history, comorbidities, surgical technique, complications and functional outcome.

Results

23 Treacher Collins children were included. A two-stage surgery was adopted in 51% (n = 12) cases, while a one-stage approach was chosen in 49% (n = 11). 2 children underwent 1st stage surgery as they were younger than 5. There were neither cases of osseointegration failure, nor cases of traumatic implant loss. Overall, the local complication rate was not different in the one-stage and in the two-stage group. The functional gain was significantly better with the Baha than with the conventional bone-conduction hearing aids (p < 0.0002). There was no difference in terms of functional outcome between the 7 patients receiving Baha at an age younger than 5 years and the rest (p = 0.23).

Conclusions

Baha can provide effective auditory rehabilitation for children with Treacher Collins syndrome, as long as it is performed in a tertiary care center where a multidisciplinary approach to the frequent comorbidities is possible. The main challenge of Baha surgery in this population is the poor or irregular thickness of the patient's calvarial bone, which often makes it necessary to drill additional holes, to place the fixture in contact with the dura or to use materials for bone augmentation.     

Etiological diagnosis in the hearing impaired newborn: proposal of a flow chart

Objective

Most industrialized countries have introduced some form of universal newborn hearing screening program. Both identification and rehabilitation of hearing loss in newborns have evolved to an acceptable standard and the need for a standardized etiological protocol is emerging.

Methods

Extensive literature search to determine which investigations can help identifying the cause of congenital hearing loss and how to limit extensive testing in these children by taking into account the most prevalent causes.

Findings

A stepwise approach to detect the cause of hearing loss in children with congenital sensorineural hearing loss was developed.

Conclusion

In general it is advised to first rule out Cx26/Cx30 and infectious causes (cytomegalovirus and, if indicated, toxoplasmosis and rubella), and to preserve more extensive investigations for those children in whom these causes do not explain the hearing loss.     

Universal newborn hearing screening in the Champagne-Ardenne region: A 4-year follow-up after early diagnosis of hearing impairment

Introduction

Permanent congenital hearing loss is one of the most frequent congenital anomaly at birth. Universal newborn hearing screening (UNHS) was introduced in numerous countries in order to allow an early diagnosis and intervention for congenital hearing impairment.

Objective

First aim of this study is to evaluate the accuracy of early diagnosis of hearing impairment after UNHS. Second aim is to discuss the auditory intervention proposed after this diagnosis. Last aim is to evaluate the relevance of UNHS for early diagnosis and intervention.

Materials and methods

Prospective study. UNHS program was introduced in the entire French region of Champagne-Ardenne in January 2004. Forty-one children have benefited of an early diagnosis of hearing impairment until June 2007. They were included in an intervention program consisting of an audiometric follow-up and an auditory intervention. This program was conducted until June 2008.

Results

There were 28 males patients and 13 females patients. The diagnosis of hearing aid impairment was carried at an average age of 3.2-month. The auditory follow-up allowed confirming the initial diagnosis of deafness for the majority of the children as for their degree of hearing loss. Auditory intervention was heterogeneous depending on degree of hearing loss of the children.

Conclusion

This UNHS program demonstrates its validity and feasibility for early diagnosis and intervention of congenital hearing impairment. It brought a major impact on the management of congenital hearing impairment in Champagne-Ardenne.     

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.     

The role of dyslipidemia in sensorineural hearing loss in children

Objective

The relationship between dyslipidemia and hearing is controversial; especially in children, the available evidences are scarce. This study is designed to determine whether dyslipidemia is associated with sensorineural hearing loss in a group of 5-18-year-old children and adolescents.

Methods

Through an analytic cross-sectional study, records of all 5-18-year-old children who attended the pediatric endocrinology clinic of Loghman Hospital in Tehran, Iran, between April 2007 and April 2009 were reviewed. Records with a lipid profile were determined. Lipid profiles were repeated for eligible cases; they were enrolled if the results were the same as before (normal or dyslipidemic). Hearing loss, speech discrimination scores and lipid profiles were analyzed.

Results

When controlled for age and sex, no association between dyslipidemia and sensorineural hearing loss were found. There was also no statistically significant relationship between dyslipidemia and sensorineural hearing loss in different age groups.

Conclusions

Dyslipidemia seems to have no association with sensorineural hearing loss in 5-18 years old children according to this study.     

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.     

Speech understanding in quiet and in noise with the bone-anchored hearing aids Baha Compact and Baha Divino

CONCLUSIONS: Speech understanding is better with the Baha Divino than with the Baha Compact in competing noise from the rear. No difference was found for speech understanding in quiet. Subjectively, overall sound quality and speech understanding were rated better for the Baha Divino. OBJECTIVES: To compare speech understanding in quiet and in noise and subjective ratings for two different bone-anchored hearing aids: the recently developed Baha Divino and the Baha Compact. PATIENTS AND METHODS: Seven adults with bilateral conductive or mixed hearing losses who were users of a bone-anchored hearing aid were tested with the Baha Compact in quiet and in noise. Tests were repeated after 3 months of use with the Baha Divino. RESULTS: There was no significant difference between the two types of Baha for speech understanding in quiet when tested with German numbers and monosyllabic words at presentation levels between 50 and 80 dB. For speech understanding in noise, an advantage of 2.3 dB for the Baha Divino vs the Baha Compact was found, if noise was emitted from a loudspeaker to the rear of the listener and the directional microphone noise reduction system was activated. Subjectively, the Baha Divino was rated statistically significantly better in terms of overall sound quality.     

A comparison of audiometric and objective methods in hearing screening of school children. A preliminary study

Objective

In newborn hearing screening, one exclusively applies objective hearing testing methods - based on evoked potentials and/or on otoacoustic emissions. However, when testing school children, one can consider both audiometric and electrophysiological methods. The choice of methods is determined by the aims of the program. If one wants to detect conductive hearing losses, impedance audiometry seems to be the method of choice.

Methods

The aim of this study was to compare test performance measures from audiometric and objective methods (OAEs and impedance audiometry), in the hearing screening of school children. Screening protocols were applied on a group of 190 children of about 12 years of age (6th grade of primary school).

Results

For a single application of a screening procedure, the best performance was observed in the automated four-tone audiometry, followed by the tympanometry and the TEOAE-based procedures. Screening performance was enhanced using a combination of automated and impedance audiometry. A four-tone audiometry test combined with tympanometry gives a sensitivity of 65%, and the PPV of 46%, which are reasonable values, acceptable for practical use. The use of a TEOAE protocol degrades the overall performance of screening.

Conclusions

Screening of school children is feasible with a combination of automated audiometry and tympanometry with time requirements equal to 3 min per subject.     

Prevalence of c.35delG and p.M34T mutations in the GJB2 gene in Estonia

Objective

The purpose of this study was to determine the prevalence of c.35delG and p.M34T mutations in the GJB2 gene among children with early onset hearing loss and within a general population of Estonia.

Methods

Using an arrayed primer extension assay, we screened 233 probands with early childhood onset hearing loss for 107 different mutations in the GJB2 gene. We then looked for the two most common mutations, c.35delG and p.M34T, in a population of 998 consecutively born Estonian neonates to determine the frequency of these mutations in the general population.

Results

In 115 (49%) of the patients with early onset hearing loss, we found a mutation in at least one allele of the GJB2 gene. Seventy-three (31%) were homozygous for the c.35delG mutation, seven (3%) were homozygous for the p.M34T mutation, and five (2%) had c35delG/p.M34T compound heterozygosity. Other six identified mutations in GJB2 gene occurred rarely. Among the 998 anonymous newborn samples, we detected 45 who were heterozygous for c.35delG, 2 individuals homozygous for c.35delG, and 58 who were heterozygous for p.M34T. Additionally, we detected two c.35delG/p.M34T compound heterozygotes.

Conclusion

The most common GJB2 gene mutations in Estonian children with early onset hearing loss were c.35delG and p.M34T, with c.35delG accounting for 75% of GJB2 alleles. The carrier frequency for c.35delG and p.M34T in a general population of Estonia was 1 in 22 and 1 in 17, respectively, and was higher than in most other countries.     

Findings from multidisciplinary evaluation of children with permanent hearing loss

Objectives

To describe clinical findings from a multidisciplinary program for children with permanent hearing loss (PHL).

Methods

Retrospective chart review at a tertiary care children's hospital. Patients: Two hundred patients charts were selected from the population of 260 children with permanent hearing loss presenting between July 2005 and December 2006. Main outcome measures: PHL etiology; radiographic findings; clinical findings by genetics, ophthalmology, developmental pediatrics, speech pathology, and aural rehabilitation.

Results

Etiology of hearing loss was determined in 60% of subjects. Genetic causes of hearing loss were identified or presumed (positive history of first degree relative with hearing loss) in 27% of the children. Structural ear anomalies were found in 20% of children. Among the 36% of children with CNS imaging, abnormal findings were noted in 32%. There were a high rate of ophthalmological findings (53%) among children seen by ophthalmology (n = 105). Neurodevelopmental evaluations were completed in 58% of subjects and clinically significant findings were noted in 68%. Of the 61% of children who receiving received speech/language evaluations, 77% required intervention. Over half of the 40% of subjects who had an aural rehabilitation evaluation needed therapy. There were not significant differences in rates of findings for children with mild or unilateral hearing loss as compared to children with more severe degrees of hearing loss.

Conclusions

Interdisciplinary medical evaluation of children with PHL allows for the identification and treatment of clinically significant ophthalmologic, neurodevelopmental, genetic, and speech/language disorders. A high rate of CNS and temporal bone abnormalities were identified. These findings provide an understanding of the importance of considering thorough medical and developmental evaluations among children who are deaf/hard of hearing.     

Benefit of Baha in the elderly with single-sided deafness

The objective of this study is to determine the benefits of bone-anchored hearing aid (Baha) contralateral routing of signal (CROS) in the older adult population with single-sided deafness. Five questionnaires [general usage questionnaire, Glasgow benefit inventory (GBI), Abbreviated profile of hearing aid benefit (APHAB), Nijmegen cochlear implant questionnaire and the hearing handicap inventory for the elderly-screening version (HHIE-S)] were used to evaluate Baha use. Consecutive patients over 60 years of age with SSD fitted with a Baha CROS between April 1990 and April 2007 not using a conventional hearing aid in the better-hearing ear were identified. Nine out of 11 patients (82 %) were still using their Baha CROS, and 7 of the 11 patients (64 %) were still satisfied. The patients experienced no to little problems with handling and cleaning of the device. The GBI scores show good benefit in domains total (14 ± 11) and general (19 ± 17). The APHAB shows that, overall, 3 out of the 11 patients (27 %) experienced significant benefit, while all others experienced no significant benefit and no drawbacks. The HHIE-S shows that the patients experienced severe (18 %), mild to moderate (46 %) or no handicap (36 %) when using the Baha CROS. In conclusion, the benefit of a Baha CROS for elderly patients with SSD is evident in the majority of patients.     

Bone-anchored hearing aid A single-stage procedure in children

Objective

The bone-anchored hearing aid (BAHA) is an essential part of the armamentarium of the modern otolaryngologist dealing with ear disorders. A two-stage approach for implantation in children is recommended by the manufacturers. In our practice we implant BAHA in children as a single procedure. We describe our technique and results.

Method

We performed a review of all children having a BAHA between 1997 and 2005. Surgery was performed by taking a post-auricular split skin graft and excising the underlying soft tissues. Drilling, tapping and fixture placement were performed as per the manufacturer's instructions. The skin graft was then sutured in place and perforated in its centre and the abutment placed. Ten to twelve weeks were allowed for osseointegration before the hearing aid attachment.

Results

Thirty children were implanted. Age at implantation ranged from 3 to 15 years (mean 9.1 and median 8.1 years). Main indications included recurrent otorrhoea, conductive hearing loss and aural atresia. Follow-up was at 4-6 weeks review initially, curtailing to 6 monthly reviews and then annual review. Early complications of skin infection occurred in two patients. Late complications such as skin hypertrophy and chronic infection occurred in two patients. Two patients lost the fixture due to trauma, both were subsequently reimplanted. There was no incidence of implant failure to osseointegrate. Twenty-eight children (93%) implanted wear their BAHAs.

Conclusion

Our results show that a single-stage technique is associated with a low rate of early complications, with no reports of fixture loss due to osseointegration failure.     

Children with hearing impairment--living with cochlear implants or hearing aids

Objective

The aim of this study was to enhance knowledge about the life circumstances of children with cochlear implants or hearing aids, regarding daily functioning and attitude to the impairment.

Methods

Data were obtained from 36 children with cochlear implants and 38 children with hearing aids via study-specific questionnaires with fixed answer alternatives. The questions covered (1) usage of aids and related factors, (2) hearing in different everyday situations, (3) thoughts about the children's own hearing and others’ attitudes to it, and (4) choice of language. The data were analyzed using SPSS, and presented via the theoretical frame of the International Classification of Functioning, Disability and Health, Child and Youth version (ICF-CY).

Results

Children with CI and HA functioned equally well in daily life, but there were also certain differences. Symptoms from neck and shoulders were more common among children with hearing aids than among children with cochlear implants (p < .001). Children with hearing aids used their aids significantly less often than those with cochlear implants (p < .001). The participation variables showed that children with hearing aids had significantly more hearing problems in team sports (p = .033) and outdoor activities (p = .019), in comparison to children with cochlear implants. The two groups had similar thoughts regarding their own hearing, mostly considering it not to be a problem. They also did not generally think that other people found their hearing to be a problem.

Conclusions

Children with cochlear implants and children with hearing aids have, in some aspects, equally good functioning in everyday life situations. However, certain differences were found in dimensions of functioning, regarding neck and shoulder pain, usage of aids and sign language, and hearing problems in some activities.