Hearing-related quality of life assessment of pediatric cochlear implant users with inner ear malformations.

OBJECTIVES: To assess the quality of life (QoL) in child and adolescent cochlear implant users with inner ear malformations (IEM) and to compare their outcomes to their cochlear implant using peers with normal inner ear structures. METHODS: The present sample consisted of 100 children (45 with IEM, 55 without IEM) and 100 adolescents (46 with IEM, 54 without IEM). The following QoL questionnaires were used to assess the hearing-related QoL: The Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26 for children between 7 and 12 years of age) and HEAR-QL-28 (for adolescents between 13 and 18 years of age). Both questionnaires were based on a 5-points Likert scale from 0 to 4, with higher scores indicating a better perception of QoL. The scores were converted to percentage values (never = 100, almost never = 75, sometimes = 50, often = 25, almost always = 0). RESULTS: For the patients with IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 50.4 (SD = 18.9) and 54.5 (SD = 19.6), respectively. For the patients without IEM, mean scores from the HEAR-QL-26 and HEAR-QL-28 were 72.7 (SD = 18.0) and 65.0 (SD = 19.1), respectively. For both child and adolescent subgroups, statistically significant differences were observed between QoL scores from patients with and without IEM (p < 0.001). There were no statistically significant effects of the malformation type on the QoL findings (p ≥ 0.05). CONCLUSION: Child and adolescent cochlear implant users with IEM had significantly lower scores on validated HEAR-QL versions in comparison to their implanted peers without IEM.

Sound localization with bilateral bone conduction devices.

PURPOSE: To investigate sound localization in patients bilaterally fitted with bone conduction devices (BCDs). Additionally, clinically applicable methods to improve localization accuracy were explored. METHODS: Fifteen adults with bilaterally fitted percutaneous BCDs were included. At baseline, sound localization, (un)aided pure-tone thresholds, device use, speech, spatial and qualities of hearing scale (SSQ) and York hearing-related quality of life (YHRQL) questionnaire were measured. Settings to optimize sound localizing were added to the BCDs. At 1 month, sound localization was assessed again and localization was practiced with a series of sounds with visual feedback. At 3 months¸ localization performance, device use and questionnaire scores were determined again. RESULTS: At baseline, one patient with congenital hearing loss demonstrated near excellent localization performance and four other patients (three with congenital hearing loss) localized sounds (quite) accurately. Seven patients with acquired hearing loss were able to lateralize sounds, i.e. identify whether sounds were coming from the left or right side, but could not localize sounds accurately. Three patients (one with congenital hearing loss) could not even lateralize sounds correctly. SSQ scores were significantly higher at 3 months. Localization performance, device use and YHRQL scores were not significantly different between visits. CONCLUSION: In this study, the majority of experienced bilateral BCD users could lateralize sounds and one third was able to localize sounds (quite) accurately. The localization performance was robust and stable over time. Although SSQ scores were increased at the last visit, optimizing device settings and a short practice session did not improve sound localization.

Systematic review of cochlear implantation in adults with asymmetrical hearing loss.

BACKGROUND: Rapid advances in cochlear implantation has witnessed an expanding spectrum for candidacy worldwide. This includes a subgroup of adults with asymmetrical hearing loss who have a wide range in their hearing capacity between the two ears. As per guidelines they are not included in mainstream candidacy for CI across the world. Evidence is now emerging to support the benefits of CI in AHL. METHODS: This review analyzed literature regarding the outcomes of CI in AHL. Primary outcome measure was to assess audiological benefits and secondary outcome measure was to assess hearing related quality of life. 15 relevant articles, published worldwide between 2009 and 2019 were chosen. CASP checklist for systematic reviews was used to ascertain the quality of literature. The strength of recommendations from each study was analyzed and classified as strong, moderate, weak or none based on GRADE guidelines. RESULTS: Heterogeneity in samples was obvious and samples varied largely between the studies. The levels of evidence ranged from systematic review to expert opinion, but overall they reflected positively on both audiological and QOL benefits. CONCLUSION: CI provides important auditory and QOL benefits in AHL, but there is no high level evidence as yet to strongly support CI for AHL. A long term multi-centric study is necessary to influence a change in practice for a growing population of AHL.Trial registration: ClinicalTrials.gov identifier: NCT03052920.

Cochlear implants in adults with partial deafness: subjective benefits but associated psychological distress.

PURPOSE: The present study investigated adults with partial deafness (PD) and asked them to rate the benefits of their cochlear implant (CI), their general level of satisfaction with it, and their level of psychological distress. Of particular interest was the role of gender. METHODS: The study comprised 71 participants (41 females) with PD who had been provided with a CI. The Nijmegen Cochlear Implant Questionnaire (NCIQ) was used to assess the benefits of their CI. Satisfaction with their CI was measured using a visual analog scale. The severity of mental distress was assessed with the General Health Questionnaire (GHQ-28). RESULTS: On various NCIQ scales, the average benefits of a CI were rated at 66%. Females gave a lower rating than males. The mental distress experienced by the group was significantly higher than in the general population. Females had more severe symptoms of anxiety and insomnia than males. There was a significant relationship between psychological distress and CI benefit, but only in females. Besides general distress, the most affected spheres were related to psychosocial functioning-"self-esteem", "activity limitation", and "social interaction". Contrary to expectations, there was no relationship between mental distress and CI satisfaction. CONCLUSIONS: The perceived benefits of a CI in subjects with PD relate mostly to the level of mental distress, although gender is an important factor. For females, their emotional state affects how beneficial their CI is perceived. Due to the higher levels of mental distress, females tend to need more psychological intervention and support.

Cochlear Implantation in Children with Usher's Syndrome: A South Asian Experience.

Usher's syndrome is an autosomal recessive disorder characterized by dual sensory impairment involving both the ears and eyes. Cochlear implantation paves a way to restore hearing loss in such individuals but poor vision among these patients poses additional challenges for the habilitationists. This study aimed to compare the habilitation outcomes and hearing-related quality of life scores of cochlear implantees having Usher syndrome with age-matched cochlear implantees with no such syndromic association. 27 patients aged 1-6 years with Usher syndrome underwent cochlear Implantation over a period of 10 years from 2006 to 2016 and were included in this study along with an age-matched cohort of 30 implantees with no additional disabilities. Category of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were compared at 3, 6, 9 and 12 months respectively. Glasgow Benefit Inventory and Health Utility Index (HUI 3.0) questionnaires were used to assess the hearing-related quality of life in both groups at 1 year post implantation. There was significant difference in CAP and SIR scores between children with Usher's syndrome and the control group (p < 0.05). The overall scores in terms of quality of life as well were statistically different (p < 0.05). Though there was improvement in speech and language acquisition after cochlear Implantation this was found to be of lesser extent than the normative cohort. These children with additional visual disabilities required intensive, individualized therapy catering to their complex needs. Their family's perception of expected benefit from cochlear Implantation was guarded and needed to be appraised in detail prior to surgery. This experience helped prepare an institutional protocol for counseling such implantees in future.

Prospective evaluation of disease-specific quality of life measurements after stapes surgery using Nitinol Head Prostheses.

OBJECTIVES: To analyze health-related quality of life (HRQoL) and audiological results after stapes surgery using a Nitinol Head Prosthesis (NHP). METHODS: Study-Design Prospective longitudinal study. Patients Patients undergoing stapes surgery for otosclerosis and stapes reconstruction with a NHP between January 2018 and December 2018 (n = 48). Intervention Audiological measurements preoperatively and at 6 months follow-up as well as two questionnaires assessing HRQoL, i.e., the Stapes Plasty Outcome Test 25 (SPOT-25) and the Glasgow Benefit Inventory (GBI). Main outcome measures Correlation of pure tone average (4PTA 0.5-3 kHz) with questionnaires assessing HrQOL preoperatively and at follow-up after stapes surgery. RESULTS: Patients showed a significantly reduced air-bone-gap (ABG, 0.5, 1, 2, 3 kHz) at the follow up visit (median: 11.3 dB, IQR 5.0-14.4) compared to the preoperative measurements (median 27.5 dB, IQR 21.9-36.3). Disease-specific HRQoL improved significantly in all scales of the SPOT-25 apart from the subscore "tinnitus". Postoperatively, the subscore "hearing function" correlated well only with the ABG (r = 0.59, p = 0.001). The SPOT-25 and GBI total scores showed a moderate negative correlation (r = - 0.59, p = 0.008). CONCLUSIONS: Stapes surgery leads to significant improvements of hearing and the HRQoL. However, correlations between HRQoL questionnaire scores and the audiometric outcomes were inconclusive, indicating that the use of HRQoL measures for the assessment of stapedotomy outcomes to complement objective outcomes should be encouraged.

Trends in hearing rehabilitation use among vestibular schwannoma patients.

OBJECTIVE: Most patients with vestibular schwannoma (VS) have significant hearing loss in the affected ear as a result of either their tumor or treatment. There is a paucity of data on which hearing rehabilitation options, if any, are preferred by patients with VS. Our study analyzed the use of hearing rehabilitation devices among VS patients. METHODS: Patients with unilateral VS treated at our institution from January 2008 to August 2018 were identified. Those with bilateral VS, profound hearing loss in the contralateral ear, neurofibromatosis type 2, or inadequate follow-up were excluded. Patients who met inclusion criteria were given two online questionnaires: an 8-item instrument that assesses use of hearing rehabilitation devices and the Abbreviated Profile of Hearing Aid Benefit. RESULTS: Of 212 eligible patients, 61 completed both surveys. The majority (88%) were able to hear "poorly" or "not at all" in the affected ear, but less than one-third had ever used a hearing rehabilitation device. Most had used conventional hearing aids (30%) or contralateral routing of signal/bilateral contralateral routing of signal (30%), whereas only a handful had used bone-anchored hearing devices or cochlear implant. Patients who did not pursue hearing rehabilitation were either not bothered by their deficit or were unaware of their options. Most had good functionality despite their deficit. CONCLUSION: Whereas 88% of patients treated for VS had a significant hearing loss, the majority did not pursue hearing rehabilitation but maintained good functional performance. Many did not remember being counseled about hearing rehabilitation options, indicating that regular and repeated counseling may be needed to increase patient utilization of hearing rehabilitation after VS treatment. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1558-1564, 2020.