Glasgow Children's Benefit Research Articles

Sacral nerve stimulation leads to long-term improvement in fecal incontinence and quality of life for children with functional and organic defecation disorders.

Our objective was to evaluate long-term outcomes of sacral nerve stimulation (SNS) for children with functional and organic defecation disorders. We performed a prospective study of children <21 years of age who started SNS treatment between 2012 and 2018. We recorded demographics, medical history, and diagnostic testing. We obtained measures of symptom severity and quality of life at baseline and follow up at 1, 6, 12, 24, 36, 48, and ≥60 months. Successful response was defined as bowel movements >2 times/week and fecal incontinence (FI) <1 time/week. Families were contacted to administer the Glasgow Children's Benefit Inventory and to evaluate patient satisfaction. We included 65 patients (59% female, median age at SNS 14 years, range 9-21) with median follow-up of 32 months. Thirty patients had functional constipation (FC), 15 had non-retentive FI (NRFI), and 16 had an anorectal malformation (ARM). The percentage with FI <1 time/week improved from 30% at baseline to 64% at 1 year (p < 0.001) and 77% at most recent follow-up (p < 0.001). Patients with FC, NRFI, and ARM had sustained improvement in FI (p = 0.02, p < 0.001, p = 0.02). Patients also reported fewer hard stools (p = 0.001). Bowel movement frequency did not improve after SNS. At most recent follow-up, 77% of patients with a functional disorder and 50% with an organic disorder had responded (p = 0.03). Nearly all families reported benefit. SNS led to sustained improvement in FI regardless of underlying etiology, but children with functional disorders were more likely to respond than those with organic disorders.

Improvement in quality of life with continuous positive airway pressure outweighs the treatment burden in children with obstructive sleep apnea.

Continuous positive airway pressure (CPAP) for treatment of obstructive sleep apnea (OSA) may pose a significant burden on families. We assessed the impact of CPAP for children on quality of life (QOL) and caregiver treatment burden. Prospective cohort study of children commencing outpatient CPAP in a specialist sleep centre 2020-2022. Questionnaires regarding sleep-related symptoms (PROMIS Pediatric Sleep Disturbance and Sleep-Related Impairment), QOL (OSA-18, QI-Disability), caregiver burden (Caregiver Strain Questionnaire) and overall health impact (Glasgow Children's Benefit Inventory) were completed by caregivers at CPAP commencement and 6 weeks later. Twenty-sixpatients completed follow-up (7 female; median age 11.4 year, baseline obstructive apnea hypopnea index 10.3/h; 77% overweight or obese, 73% comorbidity other than obesity). OSA-related QOL (OSA-18) significantly improved at follow-up (p < 0.01), as did child general QOL (p < 0.001), sleep disturbance (p < 0.01) and sleep-related impairment (p < 0.001). Caregivers mostly rated CPAP as beneficial to their child's health but 19% rated CPAP as harmful or having no effect. Caregiver strain reduced at follow-up (p < 0.001) and benefit outweighed inconvenience (p < 0.0001) in 81%. CPAP adherence was correlated with overall health impact (r = 0.67, p < 0.01) but not with caregiver rating of inconvenience. CPAP resulted in improvements in QOL and sleep-related symptoms, and reduced caregiver strain. Perceived benefits outweighed the burden of treatment for most but not all families. CPAP adherence was moderately correlated with family-reported measures of benefit but not related to perceived inconvenience. This study provides reassuring evidence regarding the benefits and impacts of CPAP for children, many of whom already have complex health care needs.

Comparison of Quality of Life Outcomes for Percutaneous Versus Transcutaneous Implantable Hearing Devices: A Systematic Review and Meta-analysis.

To compare quality of life (QOL) outcomes of percutaneous and transcutaneous bone conduction devices (pBCD and tBCD, respectively). Pubmed, Scopus, CINAHL. A systematic review was performed searching for English language articles from inception to March 15, 2023. Studies reporting QOL outcomes measured using a validated tool following implantation of either pBCDs or tBCDs were considered for inclusion. QOL outcomes included scores for Glasgow Benefit Inventory, Glasgow Children's Benefit Inventory, Abbreviated Profile of Hearing Aid Benefit, and the Speech, Spatial, and Qualities of Hearing Scale. A meta-analysis of continuous measures was performed. A total of 52 articles with 1,469 patients were included. Six hundred eighty-nine patients were implanted with pBCDs, and the remaining 780 were implanted with tBCDs. Average Glasgow Benefit Inventory scores for the tBCD group (33.0, 95% confidence interval [22.7-43.3]) were significantly higher than the pBCD group (30.9 [25.2-36.6]) (Δ2.1 [1.4-2.8], p < 0.0001). Mean Glasgow Children's Benefit Inventory scores (Δ3.9 [2.0-5.8], p = 0.0001) and mean gain in Abbreviated Profile of Hearing Aid Benefit scores (Δ5.6 [4.8-6.4], p < 0.0001) were significantly higher among patients implanted with tBCDs than those implanted with pBCDs. Patients implanted with tBCDs also had significantly higher gains on the Speech (Δ1.1 [0.9-1.3], p < 0.0001), Spatial (Δ0.8 [0.7-0.9], p < 0.0001), and Qualities of Hearing (Δ1.2 [1.1-1.3], p < 0.0001) portions of the Speech, Spatial, and Qualities of Hearing Scale than those implanted with pBCDs. Patients implanted with transcutaneous devices had better QOL outcomes than those implanted with percutaneous devices.

An Assessment of Quality-of-Life Following Tissue Expansion in Pediatric Patients

Introduction: There is limited data on the effects of tissue expansion (TE) surgery on quality-of-life (QOL) in pediatric patients. Evidence-based information may help clinicians, patients, and their families with treatment decision-making and post-operative expectations. This study explores QOL following TE in pediatric patients. Methods: A post-intervention QOL survey and retrospective chart review was performed. Patients who underwent TE at a pediatric tertiary hospital between October 2004 and March 2020 completed the Glasgow Children's Benefit Inventory or the Glasgow Benefit Inventory. Total scores range from −100(worsened QOL) to +100(improved QOL). Participants were also asked if they would recommend TE to other patients with the same indication. Results: The response rate was 38% (n = 20; 14 females, 6 males). The median QOL score was +17[−2,+49]. Higher QOL scores were found for TE indications of scar (+49) and alopecia (+40), compared to giant congenital melanocytic nevus (−2). Higher scores were also associated with shorter time in active treatment ( r = −0.65) and fewer complications ( r = −0.56). 18/20 participants would recommend TE. Participants whose QOL improved (n = 11, 55%) reported increased self-confidence, fitting in with peers, and improved function. Participants with unchanged or decreased QOL (n = 9, 45%) described a negative experience with residual scarring. However, 7/9 with unchanged or decreased QOL would still recommend TE. Conclusions: This preliminary descriptive study demonstrated variable QOL following TE. Patient and treatment-related factors impact QOL outcomes. Despite the range in QOL outcomes, the majority of participants would recommend TE. Further research evaluating QOL following TE is necessary to better understand this relationship.

TCOF1 Gene variation in Treacher Collins syndrome and evaluation of speech rehabilitation after bone bridge surgery

Objective:By analyzing the clinical phenotypic characteristics and gene sequences of two patients with Treacher Collins syndrome（TCS）, the biological causes of the disease were determined. Then discuss the therapeutic effect of hearing intervention after bone bridge implantation. Methods:All clinical data of the two family members were collected, and the patients signed the informed consent. The peripheral blood of the proband and family members was extracted, DNA was extracted for whole exome sequencing, and Sanger sequencing was performed on the family members for the mutation site.TCOF1genetic mutations analysis was performed on the paitents. Then, the hearing threshold and speech recognition rate of family 2 proband were evaluated and compared under the sound field between bare ear and wearing bone bridge. Results:In the two pedigrees, the probands of both families presented with auricle deformity, zygomatic and mandibular hypoplasia, micrognathia, hypotropia of the eye fissure, and hypoplasia of the medial eyelashes. The proband of Family 1 also presents with specific features including right-sided narrow anterior nasal aperture and dental hypoplasia, which were consistent with the clinical diagnosis of Treacher Collins syndrome. Genetic testing was conducted on both families, and two heterozygous mutations were identified in the TCOF1 gene: c. 1350_1351dupGG（p. A451Gfs*43） and c. 4362_4366del（p. K1457Efs*12）, resulting in frameshift mutations in the amino acid sequence. Sanger sequencing validation of the TCOF1 gene in the parents of the proband in Family 1 did not detect any mutations. Proband 1 TCOF1 c. 1350_1351dupGG heterozygous variants have not been reported previously. The postoperative monosyllabic speech recognition rate of family 2 proband was 76%, the Categories of Auditory Performance（CAP） score was 6, and the Speech Intelligibility Rating（SIR） score was 4. Assessment using the Meaningful Auditory Integration Scale（MAIS） showed notable improvement in the patient's auditory perception, comprehension, and usage of hearing aids. Evaluation using the Glasgow Children's Benefit Inventory and quality of life assessment revealed significant improvements in the child's self care abilities, daily living and learning, social interactions, and psychological well being, as perceived by the parents. Conclusion:This study has elucidated the biological cause of Treacher Collins syndrome, enriched the spectrum of TCOF1 gene mutations in the Chinese population, and demonstrated that bone bridge implantation can improve the auditory and speech recognition rates in TCS patients.

Patient reported outcome measures in unilateral aural atresia treated using a transcutaneous bone conduction implant (The Cochlear Baha Attract®)

Unilateral aural atresia (UAA) is a congenital condition that is associated with maximal conductive hearing loss. The primary objective of this study was to assess the long-term compliance, complications, and quality of life of pediatric patients with UAA who had transcutaneous bone conduction hearing implants (TCBI). Retrospective consecutive case series at a single centre between 2014 and 2018. Inclusion criteria consisted of UAA patients between 5 to 17 years-old receiving the Cochlear Baha Attract ® device. Demographic and audiologic data was extracted from charts. A prospective telephone survey was done, with patients and their families completing the Glasgow Children's Benefit Inventory (GCBI). Basic descriptive statistics, paired t-tests, and a univariate analysis were completed. Data was successfully collected from all 9 eligible children who received the Cochlear Baha Attract ® device for UAA (100%). The mean follow-up duration was 33 months after TBCI (9-60 months). The mean daily use was 7.7h/day. Pure tone average and mean speech in noise scores were both significantly improved when comparing the unaided condition to the aided condition with TCBI (p<0.001). The majority (89%) of patients had an improvement in GCBI; the median GCBI score was +14.6, indicating overall positive benefit. A linear regression showed no demographic variables were significant for mean daily use or GCBI scores. This preliminary study showed that patients with a TCBI for UAA had high long-term compliance and daily usage rates. TCBI improved the quality of life for the majority of patients and significantly improved hearing measures.

Underinsurance in children is associated with worsened quality of life after cochlear implantation

ImportanceResearch has suggested that early cochlear implantation is associated with improved language outcomes. Select studies demonstrate that this translates into a higher quality of life following implantation. Previous work from our group has shown that underinsurance represents a risk factor for worsened auditory and language outcomes for implantees. However, to our knowledge, the effect of insurance status on quality of life outcomes following cochlear implantation has not been evaluated. ObjectiveTo assess quality of life outcomes for children receiving cochlear implants, accounting for age at implantation, insurance status, gender, surgeon, number of implants and duration of follow-up since implantation. DesignA retrospective study using the Glasgow Children's Benefit Inventory (GCBI), a validated questionnaire measuring quality of life across four domains: learning, emotion, vitality and physical heath. Multivariate linear regression was used to examine the effects of age at implantation, insurance status, number of implants, sex, surgeon, and duration of follow-up on GCBI scores. Age at implantation was assessed as both a continuous and dichotomous variable, comparing children implanted by 12 months of age with those implanted after 12 months. SettingChildren's National Health System in Washington, DC, a tertiary academic referral center. ParticipantsThe GCBI was administered telephonically to parents/guardians of prelingually deaf children aged 2–16 years who received cochlear implants at the center between January 1, 2008 and December 31, 2018. ResultsOf 169 prelingually deafened implantee children who met inclusion criteria, parents/guardians of 64 (37.9%) responded to the questionnaire. After excluding children with late implantation (≥7 years age at CI) and missing GCBI responses, the final analytic sample consisted of 57 children. The mean age (SD) of the children at the time of the study was 3.3 (1.9) years, 63.2% were publicly insured, and 73.7% were implanted after 12 months of age. Average duration of follow-up was 3.9 (2.8) years. On a scale of −100 to +100, GCBI scores ranged from 41.7 to 95.8 (mean (SD), 64.0 (10.3)). Public health insurance (β, −5.8 [95% CI, −10.6 to −0.01]), and older age at the time of implantation (β, −0.1 [95% CI, −0.3 to 0.0]), particularly implantation following 12 months of age (p < 0.05), were significantly associated with lower GCBI scores after implantation. ConclusionPublicly insured recipients of cochlear implants and children implanted at an older age, particularly after 12 months of age, experienced significantly lower quality of life measures.

Correlation of quality of life with speech and hearing performance after pediatric cochlear implantation

ObjectivesThis study aimed to determine the correlation of the quality of life (QOL) with the Categories of Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales in children after cochlear implantation. MethodsThis cross-sectional study was conducted from November 2018 to February 2020 at the otolaryngology department at a tertiary referral center. Patients aged ≤16 years who had received cochlear implants (CIs) at our center were consecutively included in this study. Parents were asked to complete the Glasgow Children's Benefit Inventory questionnaire, and auditory and speech assessments were performed by the speech therapists at our center. The correlations of Glasgow Children's Benefit Inventory results with the objective data from the CAP and SIR assessment tools were analyzed. ResultsSeventy patients were included in this study. The mean age at implantation was 3 years and 9 months. The mean Glasgow Children's Benefit Inventory score was 52.23 (standard deviation = 23.99), indicating a positive benefit in QOL. There was a statistically significant correlation of the QOL questionnaire score with the CAP score (r = 0.40, p = 0.008), but no correlation was found between the QOL questionnaire score and the SIR score. ConclusionWe recommend that cochlear implant patients should undergo an evaluation that incorporates auditory, language, and QOL assessment tools to gain a more comprehensive understanding of their progress.

Outcome of Auricular Reconstruction with Porous Polyethylene Frameworks: Our 10 Years of Experience with 113 Children and Adults.

This study evaluates the surgical outcome and patient benefit after auricular reconstruction with porous polyethylene frameworks and temporoparietal fascia flaps in both children and adults. A total of 161 patients who had undergone auricular reconstruction between 2003 and 2014 by doctors in our department were asked to answer both a validated health-related quality-of-life questionnaire (Glasgow Benefit Inventory or Glasgow Children's Benefit Inventory) and a questionnaire reporting satisfaction, complaints, and complications with the reconstructed pinna. Furthermore, postoperative surgical complications were recorded. A total of 113 patients (89 individuals older than 14 and 24 children under the age of 14) returned the questionnaires. About 80% of the patients were satisfied with the results of their auricular reconstruction. The main complaints concerned scars and the shape. Patients' quality of life was correlated with their postoperative aesthetic satisfaction. Thus, framework extrusion was the principal factor affecting patients' satisfaction, whereas treatable postoperative complications did not interfere with the overall quality of life. Interestingly, children indicated greater benefit from auricular reconstruction than older patients. With regard to the patient's feeling of being physically healthy after auricular reconstruction, partial pinna reconstruction and reconstruction without simultaneous hearing restoration should be very carefully considered. Auricular reconstruction using a porous polyethylene framework significantly increases the patients' health-related quality of life and leads to significant patient satisfaction, especially in the case of younger patients.

Gastric electrical stimulation improves symptoms and need for supplemental nutrition in children with severe nausea and vomiting: A ten-year experience.

Gastric electrical stimulation (GES) is a promising therapy for children with severe nausea and vomiting, but information on long-term outcomes is limited. We sought to evaluate the long-term efficacy and safety of GES and to describe patient benefit and satisfaction. Using a prospective registry, we identified patients aged <21years treated with GES at our institution between 2009 and 2019. Encounters were selected at baseline prior to GES and at follow-up at 1month, 12months, and the most recent visit. We compared symptoms, route of nutrition, and medication usage at baseline to follow-up timepoints. Factors associated with improvement were evaluated. We recorded complications and need for battery replacement. We contacted families to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire. Eighty-five patients (68.2% female, median age 15.8years) completed a trial of temporary GES due to severe nausea and vomiting. Seventy-seven (90.6%) had a positive response and underwent permanent stimulator placement. Use of tube feeding or parenteral nutrition (PN) decreased from 72.7% at baseline to 29.9% at the most recent follow-up (p<0.001). Higher baseline vomiting severity was associated with the ability to stop supplemental nutrition by 1month (p<0.05). Fourteen patients (18.2%) had complications, primarily due to stimulator-associated discomfort, and 29 (37.7%) required battery replacement. Median GCBI was +52.1, indicating health-related benefit. Children with severe nausea and vomiting treated with GES experienced significant and durable improvement in symptom severity and their ability to tolerate oral nutrition.

Evaluation of unilateral and bilateral bimodal bone conduction hearing intervention in patients with bilateral microtia-atresia

Objectives: To evaluate the therapeutic effects of unilateral/bilateral bone conduction hearing rehabilitation in patients with bilateral microtia accompanied with severe conductive hearing loss following staged auricle reconstruction and bonebridge implantation. Methods: Thirty-two patients, including 20 males and 12 females, with an average age of 11.8, who received surgical treatment in Peking Union Medical College Hospital (PUMCH) from March, 2016 to January, 2020 with bilateral microtia-atresia were included. Hearing thresholds, speech perception and high-resolution CT of the temporal bone were evaluated prior to surgery and individualized surgery plans (staged auricle reconstruction and bonebridge implantation) were made. Hearing thresholds and speech perception in quiet and noise (SNR = 5 dB) using unilateral Bonebridge were tested two weeks after the implantation surgery when the Bonebridge was activated and at 3th, 6th, 12th month after activation. Hearing thresholds and speech perception were also tested at least three months after the activation of the Bonebridge under three conditions: unaided, unilateral Bonebridge, and bilateral bone conduction hearing devices (Bonebridge plus contralateral ADHEAR). The international hearing aid assessment questionnaire (IOI-HA) and Glasgow children's benefit questionnaire were used to evaluate the subjective benefits of the patients. SPSS 21.0 software was used for statistical analysis. Results: Among these 32 patients, nine were conducted Bonebridge implantation surgery before auricle reconstruction, six were simultaneously with auricle reconstruction and 17 were implanted after auricle reconstruction surgery. Compared with unaided, the mean hearing thresholds (0.5, 1, 2, and 4 kHz) and speech perception following unilateral BCHD and bilateral BCHD attachment were improved significantly (P<0.05 each). The speech perceptin in noise of bilateral BCHD was better than unilateral (P<0.05 each). The modified questionnaire revealed high levels of patient satisfaction following use of both unilateral and bilateral devices. Conclusions: Individulized surgical procedures involving auricle reconstruction and Bonebridge implantation are safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. Speech perception in noise is better following bilateral BCHD than unilateral BCHD attachment.

Evaluation of Cognitive and Social Behavior After Adenotonsillectomy in Children.

To evaluate improvement in cognitive and social behavior of children after adenotonsillectomy. This prospective, observational study was done on 50 patients between November 2016 to September 2019 with clinical features suggestive of adenoid and palatine tonsillar hypertrophy (grade 3, 4) at department of otorhinolaryngology in our institute. We used 10 questions of Glasgow Children's Benefit Inventory (GCBI), to evaluate the cognitive and social behavior of children after adenotonsillectomy. After surgery, we evaluated cognitive functions (distractibility, learning, concentration and irritability) and social behavior (Progress and development, liveliness, family harmony, fun with friends, happiness and leisure) of children. p value of comparison at 1month and 6months postoperative was significant (< 0.05) for all questions of GCBI. Adenotonsillectomy definitely have positive impact on cognitive and social behavior of children. So early surgical intervention in form of adenotonsillectomy is recommended in children having clinical symptoms of adenotonsillar hypertrophy and sleep disordered breathing.

Long-term evaluation of development in patients with bilateral microtia using softband bone conducted hearing devices

ObjectiveThe ability of early intervention with softband bone conducted hearing device (BCHD) to ensure normal development of speech, language and psychosocial situations remains undetermined. We aimed to evaluate auditory and speech development, as well as psychosocial situations of children with bilateral microtia fitted with a softband BCHD for 3–5 years. MethodsThe study included 53 patients with bilateral microtia and 53 sex- and age-matched children with normal hearing. Auditory development was evaluated using the Meaningful Auditory Integration Scale (MAIS) and Categories of Auditory Performance (CAP). Speech Intelligibility Rating (SIR) and Meaningful Use of Speech Scale (MUSS) were used to assess speech development. The psychometric properties of these patients were evaluated using Achenbach's Child Behavior Checklist (CBCL), and subjective benefits were measured using the Glasgow Children's Benefit Inventory (GCBI) questionnaire. ResultsThe average unaided and aided hearing thresholds measured using VRA were 73.8 ± 5.1 dB HL and 30.5 ± 6.0 dB HL, respectively. The total MAIS scores of the patients were 89.6 ± 9.6% and 93.0 ± 8.8% of normal hearing children at the last follow-up. The CAP scores of the two groups were 6.5 ± 1.3 and 6.9 ± 0.3, respectively. The mean MUSS score of the patients and the control group were 31.9 ± 7.0 and 34.3 ± 6.0, respectively. The mean SIR score of the two groups were 4.6 ± 0.7 and 4.8 ± 0.4. CBCL found that only two patients could be considered problematic psychosocially. The average benefit score on the GCBI was 32.9 ± 29.3. ConclusionsSoftband BCHD significantly improved auditory development in patients with bilateral microtia, with speech development reaching the level of normal hearing peers. No significant behavioral problems were found in the patients, with subjective evaluations showing that softband BCHD improved patient quality of life.

Long-term quality of life in children with complex needs undergoing cochlear implantation

ObjectivesAn estimated 30–40% of children undergoing cochlear implantation (CI) have additional disabilities and traditional outcome measures are often not reliable or appropriate. Few studies have evaluated long-term quality of life (Qol) in this group. Our aim was to assess long term QoL of children implanted with complex needs against a cohort of children implanted without. We also compared both groups for secondary outcome measures such as parental opinion, complications and hours of device wear. MethodsA retrospective casenote review of all children undergoing assessment between 2005 and 2010 was undertaken. Children with complex needs were identified using the Scottish Complex Needs Group Criteria (ISD 2006). Quality of life was measured using the GCBI (Glasgow Children's Benefit Inventory) which is a validated questionnaire suitable for the retrospective assessment of health-related QoL following paediatric surgical interventions. It consists of a 200-point scale which ranges from −100 (maximal harm) to +100 (maximal benefit) and can be analysed in four QoL subdomains (health, vitality, physical, learning). Parents were also asked to complete a simple questionnaire asking if they would recommend CI to other families and whether they would wish their child to undergo CI if given the option again. Results174 children were implanted and 17 (10%) were found to have complex needs. Follow-up ranged from 4 to 9 years. Total GCBI scores were similarly high between groups but significantly higher scores were found in the emotion (p = 0.05) and learning domains (p = 0.03) in the complex needs group. Linear regression analysis was used to show that total GCBI score was not influenced by the number of categories of impairment (R2 = 0.0123) in the complex needs group. All parents of children with complex needs said that they would proceed with implantation again or recommend to a friend. The complication rate in the complex needs group was higher (29 vs 15%), with device failure being particularly more common. Data-logging was consistent between both groups. A higher rate of development of significant other health issues that could affect outcome was found in the complex needs group (35% vs 8%). ConclusionsOur study suggests that children with complex needs have comparable long-term QoL scores to those without and seem to particularly benefit in the domains of learning and emotion. Complications are higher in this group and there remains an increased risk of further development of medical conditions following early implantation, which may influence progress.

Assessment of quality of life outcomes with the Glasgow Children's Benefit Inventory following cochlear implantation in children.

This study investigated health-related quality of life outcomes for children with cochlear implants in India using the Glasgow Children's Benefit Inventory questionnaire. Cochlear implantation is associated with improved language outcomes. Some studies show this gives higher quality of life after implantation. Previous research demonstrates that India presents unique circumstances that impact perspectives regarding cochlear implantation. Children (aged under 18 years) who had undergone cochlear implantation were recruited from Vani Pradan Kendra, an organisation for individuals with hearing loss based in Bangalore, India. Demographic data including age, sex, duration of hearing loss and age at implant were collected, and the children's parents or caregivers completed the Glasgow Children's Benefit Inventory questionnaire. Sixty-nine children (mean age: 8.0 ± 3.89 years) were recruited, and all reported improved quality of life after cochlear implantation. There was no effect of age, gender or education on reported benefits. However, a younger age at implant and longer experience with an implant were associated with greater quality of life improvements. Cochlear implantation leads to improved quality of life, with greater improvements associated with earlier implantation. This supports early intervention in children with profound hearing loss.

Measuring otoplasty outcome: Expanding the validity to caregivers' perspective and to Portuguese-speaking children.

The GBI (Glasgow Benefit Inventory) and the GCBI (Glasgow Children's Benefit Inventory) are used to evaluate patient-reported Health-Related Quality of Life (HRQoL) following otoplasty for prominent ears. The aim of this study was to validate them through patients' and caregivers' perspectives and in Portuguese language versions. After translation to Portuguese, the GBI and the GCBI were applied to patients (n = 255) who underwent otoplasty throughout a five-year span and to their caregivers, respectively. A second set of questionnaires (retest) was applied to assess reliability. Caregivers were subsequently contacted to evaluate aesthetic satisfaction, recommendation to others, postoperative complications and main motivation for surgery. Paediatric Surgery Department of a tertiary hospital in Porto, Portugal. 134 patients (aged 10.9 ± 3.6 years, 61.2% males) and their caregivers (52.5% responders). Impact on HRQoL using the Portuguese GBI and GCBI (global score, -100 to 100). Validity assessed by principal component factor analysis, Cronbach's α and Intraclass Correlation Coefficient (ICC). The GBI was 30.1 ± 25.7 and the GCBI was 22.6 ± 23.0. For the GBI, three subscales were identified, explaining 63.3% of variance. For the GCBI, two subscales were identified, accounting for 66.8% of variance. Both the GBI and the GCBI showed a high internal consistency (α = 0.92 and 0.94, respectively). 79% were satisfied and 92% recommended the surgery. Statistically significant higher scores were found in satisfied patients, in those who underwent surgery due to teasing, and in females. The Portuguese GBI and GCBI proved to be valid and reliable to evaluate HRQoL following otoplasty in Portuguese-speaking children. The outcome was very favourable; almost all patients would recommend the surgery.

A functional and anatomical comparison between two passive transcutaneous bone conduction implants in children

ObjectiveTo compare anatomical and functional outcomes of two passive transcutaneous bone conduction implant systems: Sophono™ and BAHA Attract™. Materials and methodsTwenty patients, affected by bilateral conductive hearing loss, underwent unilateral transcutaneous bone conduction implant surgery. Ten children received a Sophono™ implant (6 males, 4 females, mean age 11 years, mean unaided Pure Tone Average (PTA) 0.25-4kHz = 69.70dB HL) and 10 a BAHA Attract™ system (7 males, 3 females, mean age 19 years, mean unaided PTA0.25-4kHz = 66.40dB HL). The following outcomes were considered: incidence of local complications, hearing aid benefit, hearing aid gain and changes in quality of life (QOL), as measured by the Glasgow Children's Benefit Inventory (GCBI). ResultsOne patient in the Sophono group experienced magnet-related skin decubitus, while two patients (one per group) had skin hyperemia in the area overlying the magnet. The mean BAHA-aided threshold was 23.70dB, whereas the mean Sophono-aided threshold was 31.60dB. The mean gain was significantly different for lower frequencies, the BAHA having better functional outcomes. All patients reported an improvement in their QOL. ConclusionGiven the lower thickness of the internal magnet, the Sophono™ system might be more suitable for younger children, whereas BAHA offered better functional results. Both systems can be considered valid and safe options for the functional rehabilitation of conductive hearing loss in children, provided that precautions are observed, such as a gradual use of the device and use of the least powerful magnets in the first months after the activation.

The management of patients with congenital malformations of the external and middle ear

Rehabilitation of the patients suffering from with congenital malformations of external and middle ear is a specific area of medicine requiring special knowledge and skills of the audiologists and ear surgeons. This article highlights the issues of epidemiology, classification and methods for the rehabilitation of patients presenting with this pathology that reflect our own experience and the methodology based on it for all phases of both functional and aesthetic surgical inerventions. Special attention is given to the assessment of the effectiveness of the treatment, as part of the evaluation of the quality of medical services. The results were demonstrated in 56 patients aged from 5 to 17 years. 32 of them underwent reconstructive, hearing improving surgery (meatotympanoplasty), 24 patients were managed by means of the implantation of the bone-anchored hearing systems. The assessment of the effectiveness of the treatment was conducted according to the algorithm developed by the authors, including free field pure tone audiometry and validated questionnaires for the assessment of the quality of life (the Glasgow Children's Benefit Inventory).

Aesthetic and hearing rehabilitation in patients with bilateral microtia-atresia

ObjectivesTo evaluate the safety and efficacy of auricle reconstruction and active transcutaneous bone-conduction implantation in patients with bilateral microtia-atresia. DesignPatients were chosen prospectively, with each being his/her own control. SettingThe setting was a tertiary referral center. ParticipantsTwelve patients, aged 6–18 years, with bilateral microtia-atresia suffering from bilateral conductive hearing loss. All had an upper bone conduction threshold limit of 45 dB HL at frequencies of 0.5–4 kHz. Main outcome measuresPatient satisfaction with the reconstructed auricle was rated as highly satisfactory, basically satisfactory, or unsatisfactory. Mean pure-tone thresholds and speech audiometry test results were compared among patients unaided, with a soft-band Bonebridge, and with an implanted Bonebridge. Subjective satisfaction was analyzed using three questionnaires: the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Glasgow children's benefit inventory (GCBI), and the International Outcome Inventory for Hearing Aids (IOI-HA). ResultsAll patients who underwent auricle reconstruction expressed satisfaction with their appearance. The mean pure-tone thresholds of unaided patients and those with soft-band and implanted Bonebridge were 55.25 ± 3.43 dBHL, 31.37 ± 3.03 dBHL, and 21.25 ± 2.16 dBHL, respectively. The mean speech discrimination scores measured in a sound field with a presentation level of 65 dB SPL under these three conditions were 46.0 ± 0.11%, 80.0 ± 0.09%, and 94.0 ± 0.02%, respectively. Questionnaires demonstrated patients' benefits and satisfaction with this surgery. ConclusionsThe surgical procedure involving auricle reconstruction and Bonebridge implantation was safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems.

Sacral nerve stimulation for constipation and fecal incontinence in children: Long-term outcomes, patient benefit, and parent satisfaction.

To evaluate the long-term efficacy of sacral nerve stimulation (SNS) in children with constipation and describe patient benefit and parent satisfaction. Using a prospective patient registry, we identified patients <21years old with constipation treated with SNS for >2years. We compared symptoms, medical treatment, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), and Fecal Incontinence Severity Index (FISI) before SNS and at follow-up. We contacted parents to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire. We included 25 children (52% male, median age 10years): 16 had functional constipation, six anorectal malformation, two tethered spinal cord, and one Hirschsprung's disease. Defecation frequency did not change after SNS but patients reporting fecal incontinence decreased from 72% to 20% (P<.01) and urinary incontinence decreased from 56% to 28% (P=.04). Patients using laxatives decreased from 64% to 44% (ns) and patients using antegrade enemas decreased from 48% to 20% (P=.03). GSS, most FIQL domains, and FISI were improved at follow-up. Six (24%) patients had complications requiring further surgery. Of the 16 parents contacted, 15 (94%) parents indicated positive health-related benefit and all would recommend SNS to other families. Sacral nerve stimulation is a promising and durable treatment for children with refractory constipation, and appears particularly effective in decreasing fecal incontinence. Although a quarter of patients experienced complications requiring additional surgery, nearly all parents reported health-related benefit. Future studies to identify predictors of treatment response and complications are needed.