Auditory function in women with autoimmune inner ear diseases and their offspring

Objective

The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.

Methods

Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.

Results

Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).

Conclusions

These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.     

Cochlear implant outcomes in children with motor developmental delay

Introduction

Multiple handicapped children and children with syndromes and conditions resulting additional disabilities such as cerebral palsy, global developmental delay and autistic spectrum disorder, are now not routinely precluded from receiving a cochlear implant. The primary focus of this study was to determine the effect of cochlear implants on the speech perception and intelligibility of deaf children with and without motor development delay.

Method

In a cohort study, we compared cochlear implant outcomes in two groups of deaf children with or without motor developmental delay (MDD). Among 262 children with pre-lingual profound hearing loss, 28 (10%) had a motor delay based on Gross Motor Function Classification (GMFC). Children with severe motor delays (classification scale levels 4 and 5) and cognitive delays were excluded. All children completed the Categories of Auditory Perception Scales (CAP) and Speech Intelligibility Rating (SIR) prior to surgery and 24 months after the device was activated.

Result

The mean age for the study population was 4.09 ± 1.86 years. In all 262 patients the mean CAP score after surgery (5.38 ± 0.043) had a marked difference in comparison with the mean score before surgery (0.482 ± 0.018) (P = 0.001). The mean CAP score after surgery for MDD children was 5.03, and was 5.77 for normal motor development children (NMD). The mean SIR score after surgery for MDD children was 2.53, and was 2.66 for NMD children. The final results of CAP and SIR did not have significant difference between NMD children versus MDD children (P > 0.05).

Conclusion

Regarding to the result, we concluded that children with hearing loss and concomitant MDD as an additional disabilities can benefit from cochlear implantation similar to those of NMD.     

Hearing aid functioning in the preschool setting: stepping back in time?

Purpose

In order for children with hearing impairment to receive maximum benefit from preschool programs, their amplification devices must be functioning appropriately. The purpose of this study, therefore, was to investigate the functionality of hearing aids in a preschool setting.

Methods

Forty-four hearing aids worn by 24 preschool children, whose ages ranged from three to five years, (mean age = 45.2 months, median age = 48 months) from four state funded aural/oral habilitation preschools were evaluated using listening checks and visual examinations. Hearing aids passing both evaluations were given a pass rating and those failing one or more of the evaluations were given a fail rating. Causes of fail rates were documented.

Results

Over half of the hearing aids tested were determined to have at least one or more problems (n = 27). These problems were detected using listening and visual checks.

Conclusions

Failure rates based on basic listening and visual checks among the hearing aids examined in this study demonstrated no improvement over those reported in the 1970's. The results re-emphasize the need for a national dialogue on this topic.     

Isolated malleus-handle fracture

Objective

Isolated malleus fracture is a rare ossicular injury with less than 80 cases published in the literature. The aim of this paper is to present a series of 3 patients with isolated malleus handle fracture, to review the literature and to discuss clinical presentation, imaging findings and treatment options.

Methods

We retrospectively a case series of 3 patients with isolated malleus handle fracture managed at a tertiary referral otologic center.

Results

The three patients related the same history of digitally manipulating the external auditory canal, leading to a sudden hearing loss and feeling of aural fullness. Meticulous otomicroscopy revealed the broken malleus with excessive movement of the distal part of the malleus on pneumatic otoscopy. High-resolution CT scan with multiplanar reconstruction along the axis of the malleus confirmed the fractures and displacements. One patient was treated conservatively and two underwent ossiculoplasty.

Conclusion

Malleus handle fracture should be systematically suspected in case of hearing loss and aural fullness of sudden onset after digital manipulation of the external auditory canal.     

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.     

Auditory evoked magnetic fields in children with functional hearing loss

Objectives

Abnormal cortical responses in patients with functional hearing loss were evaluated by magnetoencephalography, which can better separate bihemispherical activity than electroencephalography.

Methods

Auditory evoked fields in response to 1 kHz or 2 kHz tone bursts at 80 dB sound pressure level were measured by a helmet-shaped magnetoencephalography system in 22 patients with functional hearing loss (18 females, mean age 13.2 years) as well as 5 control subjects under 10 years old. Waveform, latency, and equivalent current dipole of N100m responses were used to evaluate activity in the bilateral auditory cortices.

Results

Abnormal N100m of the contralateral response to the stimulated ear, either absence or delayed latency in comparison to normal adult subjects, was found in 6 of the 7 patients with functional hearing loss aged 9 years or younger, but in only 3 of the 15 patients aged 10 years or older. However, such abnormalities were also observed in younger control subjects.

Conclusion

Auditory evoked field may be applied to objectively evaluate cortical auditory function in patients with functional hearing loss, but the normal findings for young children have not yet been established.     

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.     

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.     

Parental and program's decision making in paediatric simultaneous bilateral cochlear implantation: Who says no and why?

Objective

To evaluate initial candidacy for bilateral simultaneous cochlear implantation in children.

Design

Prospective case series.

Setting

Tertiary academic pediatric hospital.

Participants

As part of our research protocol all children eligible for cochlear implantation were assessed for suitability to receive a simultaneous bilateral implant. Over a 12-month period (January to December 2007) 78 children received a total of 95 cochlear implants. Children with sequential second implants (24), revision cases (4), and out of province recipients (4) were excluded. The remaining 46 patients were assessed for bilateral simultaneous implantation.

Main outcome measures

Team/parental decision to proceed with bilateral simultaneous implantation.

Results

17 children (37%) received simultaneous bilateral implants. 29 children (63%) were not considered suitable for simultaneous bilateral implantation. Reasons included developmental delay (10), residual borderline hearing in the second ear (9), parental/patient refusal (6), abnormally poor speech development for age (2), and abnormal cochlear anatomy precluding implantation (2). None were considered unsuitable for the more prolonged operative procedure on medical grounds.

Conclusions

Although bilateral implantation is thought to produce the optimal auditory outcome, not all patients are suitable, nor do all parents wish to proceed, when assessed for simultaneous implantation. Some of these patients are likely to be candidates for sequential bilateral implantation in due course.     

Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening

Objective

The goal of this study was to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Shanghai, China.

Methods

Between October 2009 and September 2010, 21,427 preschool children were enrolled from five areas of Shanghai, who had passed newborn hearing screening. Children were screened for delayed-onset hearing loss with pediatric audiometers. Children with positive results in initial and re-screening tests were assessed audiologically and for risk indicators according to the Year 2007 Statement of the American Joint Committee on Infant Hearing (JCIH).

Results

During the study period, 445 children (2.08%) were referred for audiologic assessment; 16 (0.75/1000, 95% CI 0.38-1.12) had permanent delayed-onset hearing loss. Of these, five (0.23/1000) had bilateral moderate hearing loss; seven (0.33/1000) had mild bilateral hearing loss; and four (0.19/1000) had unilateral moderate or mild hearing loss. JCIH risk indicators were established for six children (6/16, 37.5%), including parental concern regarding speech and language developmental delay, neonatal intensive care unit with assisted ventilation or hyperbilirubinemia, recurrent otitis media with effusion, craniofacial malformation, and family history (n = 1 for each). The remaining ten (62.5%) had no related risk factors.

Conclusions

A significant proportion of preschool children have undiagnosed delayed-onset hearing loss. Hearing screening in preschool is recommended for an early detection.     

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.     

BAHA results in children at one year follow-up: A prospective longitudinal study

Objective

To evaluate audiometric and clinical results of children fitted with a bone-anchored hearing aid with specific emphasis on speech discrimination in different sound environments after one year of use.

Methods

We performed a prospective longitudinal study. Seventeen patients between the ages of 5 and 18 years old were included. All patients underwent a complete tonal and vocal evaluation at four pre-determined intervals between the pre-operative period and one-year of bone-anchored hearing aid (BAHA) use. Basic pure-tone average and speech reception threshold were measured in different sound environments. Speech discrimination improvement was tested with the voice originating from the side of the BAHA-fitted ear and with the voice originating from a source directly in front of the patient. These measures were repeated with confounding noise facing the patient then from the side of the affected ear. All tonal and vocal evaluations were performed pre-operatively, the day of processor insertion, 6 months and 12 months after processor insertion.A variance analysis was performed to compare differences in hearing gain with BAHA over time.

Results

Hearing gain with BAHA was clinically and statistically significant at all intervals. Conventional tonal evaluation revealed significantly improved hearing gain after BAHA insertion compared with pre-operative testing with BAHA (26.3 dB vs. 17.3 dB), and this improvement was maintained at one year (27.9 dB). Speech discrimination gain at one year was better than immediately post-insertion (21.9% vs. 11.7%). Maximal gain with BAHA was found with the voice originating from the side of the affected ear and with confounding noise facing the patient (27.1% at one year), whereas the least gain was found in a silent room with the voice coming from straight ahead (11.9% at one year).

Conclusions

Pure-tone average gain at one year post-insertion was similar to immediate post-insertion gain. BAHA aids speech discrimination most when the voice originates from the side of the affected ear with confounding noise facing the patient. Speech discrimination gain improves with time, suggesting an underlying learning process. The best BAHA gain in speech discrimination occurred with background noise.     

The submucous cleft palate: Diagnosis and therapy

Objectives

To investigate age of diagnosis, typical symptoms, finding of the palate, therapy options and accompanying diseases.

Methods

A retrospective analysis of 439 patients with symptomatic submucous cleft palate (SMCP), who received a veloplasty operation (butterfly suture technique developed by Haase) was made.

Results

SMCP was initially diagnosed at the mean age of 4.9 years. Main symptoms were hyper nasal speech (51%) and conductive hearing loss (45%), which resolved after veloplasty (often in combination with adenotomy and insertion of ventilation tubes). Typical findings of the palate were a lack of posterior nasal spine (68%) and bifid uvula (59%). Following surgery 17.1% required speech therapy and 5.5% needed velopharyngoplasty due to continuing hyper nasal speech.

Conclusion

SMCP is often diagnosed very late, though symptoms of velopharyngeal insufficiency (hyper nasal speech, Eustachian tube dysfunction) and bifid uvula are present. We therefore recommend that all patients with such findings are examined by an appropriate specialist such as Phoniatrics, Otolaryngologist and Oral-Maxillofacial-Surgeon so that early diagnosis and palatoplasty can be performed. The veloplasty operation (butterfly suture technique) can be recommended as a safe therapy for velopharyngeal insufficiency for patients with symptomatic SMCP.     

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.     

Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss

Auditory neuropathy is a challenging disorder and needs special habilitative/rehabilitative approach. This study aimed to detect its prevalence among infants and young children with severe to profound hearing loss. 112 infants and young children with age ranged 6-32 months were examined and diagnosed as having severe to profound hearing loss and were referred for hearing aid fitting. Those infants were reassessed in our centers for detecting cases with auditory neuropathy. The study group was subjected to immittancemetry, behavioral observation audiometry, ABR and cochlear microphonics.

Results

15 patients were found to have auditory neuropathy according to our criteria for diagnosis.

Conclusions

The prevalence of AN in the study group was 13.4%. CM were recommended to be tested routinely during ABR assessment whenever abnormal results are obtained.     

Qualitative analysis of parents’ experience with receiving the news of the detection of their child's hearing loss

Objective

Despite the fact that clinicians are responsible for delivering bad news, they have been shown to lack both confidence and skill in performing this basic task. The time immediately after the detection of childhood hearing loss is perceived as stressful. We conducted a qualitative study to assess parents’ experiences with receiving the bad news of the detection of their child's hearing loss.

Study design

Semi-structured interviews were conducted with families of children with hearing loss identified during early childhood.

Setting

A rehabilitation center treating pre-school children in the north of Israel.

Patients

14 families/parents of children diagnosed prior to the implementation of a universal screening program.

Main outcome measure

Parents’ perceptions of the manner in which the information regarding the detection of their child's hearing loss was given, and what their feelings were at the time.

Results

50% of parents expressed dissatisfaction with the process of the breaking of the bad news. ABR is perceived by parents and health professionals alike as the definitive moment of diagnosis. The emotions experienced by parents at the moment of the breaking of the bad news were predominantly shock and upset. The meaning of the news was perceived differently under different circumstances. Information given bluntly, without empathy, was a frequent complaint. Parents repeatedly stated the importance of the formulation of a plan for the future.

Conclusions

Qualitative enquiry provided valuable information. Effective strategies for the breaking of bad news should become an integral part of universal neonatal screening programs.     

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.     

Newborn hearing screening on infants at risk

Objectives

This article presents the results of newborn hearing screenings on infants at risk of hearing impairment at the French University Hospital of Besançon from 2001 to 2007.

Materials and methods

All newborns at risk of hearing impairment were tested according to the method recommended by the Joint Committee on Infant Hearing (JCIH): a two-step automated oto-acoustic emissions (AOAE) program, completed by an auditory brainstem response (ABR) for the positive diagnosis of hearing impairment. The screening started with AOAE on the third day of life, at the earliest. If one or both ears did not have AOAE, the infant was re-tested at which time, should the AOAE again be positive, ABR was performed. When the ABR threshold was 40 dB or more, the infant was referred to an audiologist specialized in infant deafness for diagnosis confirmation and management.

Results

Over the period, 1461 infants were screened, among whom 4.55% were diagnosed as deaf or hard of hearing. Nearly 10% of the infants were lost to follow up. Forty-six children had a sensorineural hearing impairment, of which 34 were bilateral and were managed before the age of 6 months. The risk factors for sensorineural hearing loss were (in order of statistical significance): severe birth asphyxia; neurological disorder; syndromes known to be associated with hearing loss; TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes) infections; family history of deafness; age at the time of screening; and the association of 2 or more risk factors. However, birth weight inferior to 1500 g and premature birth before the 34th week of pregnancy did not show a statistically significant influence on sensorineural hearing loss. Craniofacial anomalies (mostly cleft palate and ear aplasia) were a significant factor for conductive hearing loss.

Conclusion

Our selected hearing screening on infants at risk allowed 60 deaf children access to early management. However, too many children were lost to follow up; which revealed that better information regarding risk of hearing loss must be provided to parents and paramedics and universal newborn screening needs to be performed. The most important result of this study is that in a population of hearing impaired children, with an impairment incidence close to what is commonly reported, the association of several risk factors proves to be a significant additional risk factor for hearing impairment.