Publication date 22-12-2020
Introduction: As pediatric cochlear implant (CI) candidacy expands, children with greater degrees of residual hearing are receiving CIs. These nontraditional candidates have audiometric thresholds that meet adult manufacturer labeling but are better than current pediatric guidelines allow. The purpose of this study was to determine the impact of delayed cochlear implantation on speech perception in nontraditional pediatric CI recipients.
Methods: Pediatric CI recipients with a history of progressive hearing loss and a preoperative 4-frequency pure-tone average of ≤75 dB HL at the time of implantation were considered for this retrospective study. Preoperative serial audiograms and word recognition scores were reviewed, and a method was created to establish a date when each individual ear 1st met nontraditional candidacy. The length of time between the date of candidacy and implantation was calculated and defined as the “delay time.” A multiple linear regression investigated delay time, age at surgery, surgery type (1st vs. 2nd side), and array type as predictive factors of maximum postoperative Consonant-Nucleus-Consonant (CNC) word scores. A one-way ANCOVA was performed comparing the postoperative CNC scores between subjects grouped by delay time.
Results: A significant regression was found (F(4, 38) = 5.167, p = 0.002, R2 = 0.353). Both age at implantation (p = 0.023) and delay time (p = 0.002) predicted CNC word scores. Longer delay time was associated with poorer word recognition scores, while older age at implantation correlated with higher CNC word scores in this progressive hearing loss group. A significant difference was noted between subjects implanted with #x3c;1 year of delay and those with 3 or more years of delay (p = 0.003). All ears implanted within a year of candidacy achieved word recognition abilities that are generally accepted as above average (M = 84.91).
Conclusion: CI candidacy for adults has evolved to allow for greater degrees of residual hearing, while audiometric guidelines for children have not changed since 2000. Our findings suggest that delay of cochlear implantation, even for children with significant levels of residual hearing, leads to poorer outcomes. Modified candidacy guidelines for children should be established to expedite referral to multidisciplinary CI teams and minimize delays in this population. Audiol Neurotol
Publication date 22-12-2020
Introduction: Non-syndromic hereditary hearing loss is characterized by extreme genetic heterogeneity. So far, more than 100 pathogenic or likely pathogenic variants in TMC1 gene have been reported in patients with autosomal recessive hearing loss (HL) DFNB7/11. The prevailing auditory phenotype of individuals with DFNB7/11 is congenital, profound, bilateral HL, but the functional outcome after cochlear implantation (CI) described in the literature is variable. The objective of this work is to evaluate the auditory outcome after CI in pediatric patients with DFNB7/11, born to non-consanguineous parents.
Methods: A retrospective analysis of genetic and audiological data of DFNB7/11 patients followed up in a single Italian otolaryngology clinic was performed. Cases with biallelic pathogenic variants in TMC1 were selected from the cohort of children with non-syndromic hearing loss who had undergone CI and had been molecularly characterized by multigene panel testing. All patients underwent extensive audiological assessment, and the auditory outcome after CI was evaluated.
Results: DFNB7/11 was diagnosed in a total of 3 patients from 2 non-consanguineous families; a novel disease-causing variant in TMC1 was detected c.962G#x3e;A p.(Trp321*). All the affected children showed the typical DFNB7/11 phenotype characterized by prelingual, severe-to-profound HL. The patients showed an excellent functional outcome after CI; speech perception, nonverbal cognition, and speech performance were comparable to those of patients with DFNB1 deafness. Discussion/Conclusion: Our results do not support the variable auditory outcome reported in the literature, which may be affected by several social and environmental factors and by the genetic background. Audiol Neurotol
Publication date 22-12-2020
Introduction: Patients with postlingual deafness usually depend on visual information for communication, and their lipreading ability could influence cochlear implantation (CI) outcomes. However, it is unclear whether preoperative visual dependency in postlingual deafness positively or negatively affects auditory rehabilitation after CI. Herein, we investigated the influence of preoperative audiovisual perception on CI outcomes.
Method: In this retrospective case-comparison study, 118 patients with postlingual deafness who underwent unilateral CI were enrolled. Evaluation of speech perception was performed under both audiovisual (AV) and audio-only (AO) conditions before and after CI. Before CI, the speech perception test was performed under hearing aid (HA)-assisted conditions. After CI, the speech perception test was performed under the CI-only condition. Only patients with a 10% or less preoperative AO speech perception score were included.
Results: Multivariable regression analysis showed that age, gender, residual hearing, operation side, education level, and HA usage were not correlated with either postoperative AV (pAV) or AO (pAO) speech perception. However, duration of deafness showed a significant negative correlation with both pAO (p = 0.003) and pAV (p = 0.015) speech perceptions. Notably, the preoperative AV speech perception score was not correlated with pAO speech perception (R2 = 0.00134, p = 0.693) but was positively associated with pAV speech perception (R2 = 0.0731, p = 0.003).
Conclusion: Preoperative dependency on audiovisual information may positively influence pAV speech perception in patients with postlingual deafness. Audiol Neurotol
Publication date 22-12-2020
Objectives: The aim of this study is to perform a histopathologic analysis of temporal bones with an intralabyrinthine schwannoma (ILS) in order to characterize its extension.
Methods: Archival temporal bones with a diagnosis of sporadic schwannoma were identified. Both symptomatic and occult nonoperated ILS were included for further analysis.
Results: A total of 6 ILS were identified, with 4 intracochlear and 2 intravestibular schwannomas. All intracochlear schwannomas involved the osseous spiral lamina, with 2 extending into the modiolus. The intravestibular schwannomas were limited to the vestibule, but growth into the bone next to the crista of the lateral semicircular canal was observed in 1 patient.
Conclusions: Complete removal of an ILS may require partial removal of the modiolus or bone surrounding the crista ampullaris as an ILS may extend into these structures, risking damage of the neuronal structures. Due to the slow growth of the ILS, it remains unclear if a complete resection is required with the risk of destroying neural structures hindering hearing rehabilitation with a cochlear implant. Audiol Neurotol
Publication date 22-12-2020
Background: Genetically determined prelingual hearing loss (HL) may occur in an isolated or syndromic form.
Objective: The aim of the study was to unravel the genetic cause of medical problems in a 21-year-old woman, whose phenotypic presentation extended beyond Stickler syndrome and included enlarged vestibular aqueduct (EVA) and persistent microhematuria.
Methods and Results: After sequencing of clinical exome, a known de novo COL2A1 pathogenic variant (c.1833+1G#x3e;A, p.?) causative for Stickler syndrome and one paternally inherited pathogenic change in COL4A5 (c.1871G#x3e;A, p.
Gly624Asp) causative for X-linked Alport syndrome were found. No pathogenic variants, including those within the SLC26A4 5′ region (Caucasian EVA haplotype), explaining the development of EVA, were identified.
Conclusions: The study reveals a multilocus genomic variation in one individual and provides a molecular diagnosis of two HL syndromes that co-occur in the proband independent of each other. For the third entity, EVA, no etiological factor was identified. Our data emphasize the relevance of detailed clinical phenotyping for accurate genotype interpretation. Focus on broadening the phenotypic spectrum of known genetic syndromes may actually obscure patients with multiple molecular diagnoses. Audiol Neurotol
HG Choi,SK Hong,HJ Lee,J Chang
Publication date 18-12-2020
Objectives: The hearing process involves complex peripheral and central auditory pathways and could be influenced by various situations or medications. To date, there is very little known about the effects of alcohol on the auditory performances. The purpose of the present study was to evaluate how acute alcohol administration affects various aspects of hearing performance in human subjects, from the auditory perceptive threshold to the speech-in-noise task, which is cognitively demanding.
Methods: A total of 43 healthy volunteers were recruited, and each of the participants received calculated amounts of alcohol according to their body weight and sex with a targeted blood alcohol content level of 0.05% using the Widmark formula. Hearing was tested in alcohol-free conditions (no alcohol intake within the previous 24 h) and acute alcohol conditions. A test battery composed of pure-tone audiometry, speech reception threshold (SRT), word recognition score (WRS), distortion product otoacoustic emission (DPOAE), gaps-in-noise (GIN) test, and Korean matrix sentence test (testing speech perception in noise) was performed in the 2 conditions.
Results: Acute alcohol intake elevated pure-tone hearing thresholds and SRT but did not affect WRS. Both otoacoustic emissions recorded with DPOAE and the temporal resolution measured with the GIN test were not influenced by alcohol intake. The hearing performance in a noisy environment in both easy (−2 dB signal-to-noise ratio SNR) and difficult (−8 dB SNR) conditions was decreased by alcohol.
Conclusions: Acute alcohol elevated auditory perceptive thresholds and affected performance in complex and difficult auditory tasks rather than simple tasks. Audiol Neurotol
Publication date 15-12-2020
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D Borsetto,V Corazzi,S Franchella,C Bianchini,S Pelucchi,R Obholzer,AJ Soulby,N Amin,A Ciorba
Publication date 14-12-2020
Aim: To assess the current opinion on the effects of hearing loss treatment by hearing aids (HAs) and the benefits of HA use on imbalance.
Methods: PRISMA-compliant systematic review was done, including observational studies in patients affected by mild to severe sensorineural hearing loss with HAs, investigating the benefits of HAs on balance. Electronic searches were performed through Medline, Cochrane, Embase, Web of Science, and Scopus.
Results: A total of 200 patients in 8 studies were included in this systematic review. Four studies were cross-sectional, 3 cross-sectional controlled and 1 prospective nonrandomized study. Static and dynamic balance in the aided condition improved in patients assessed using clinical investigations including Romberg test and Functional Ambulation Performance/mini-BESTest, respectively. Variable outcomes were found measuring static and dynamic balance during the aided condition with objective tests (computerized posturography, Mobility Lab device). Improved quality of life outcomes and self-confidence were noted, while subjective measurements of balance had conflicting results.
Conclusion: Although an improvement in balance in patients with HAs has been shown in certain conditions, the overall benefit is still unclear and it is only possible to speculate that HAs may also improve static, dynamic, or subjective perception of balance function in adults affected by hearing loss. Audiol Neurotol
E Magen,I Blum,DA Waitman,N Kahan,B Forer
Publication date 11-12-2020
Objectives: Autoimmune inner ear disease (AIED) is a distinct clinical entity from sudden sensorineural hearing loss. The purpose of this study was to investigate the clinical characteristics of AIED in patients with selective IgA deficiency (s IgAD).
Materials and Methods: This retrospective observational study was based on data from the Leumit Healthcare Services database in Israel. We searched all subjects aged ≥12 years who had undergone serum total IgA measurements during 2004–2014 for any reason. The s IgAD patients included all subjects with serum IgA of ≤7 mg/dL (0.07 g/L). A control group was randomly sampled from the full study population (n ≈ 730,000) with a case-control ratio of 10 controls for each case (1:10).
Results: Among 347 subjects with s IgAD, we identified 9 patients with concomitant AIED (s IgAD + AIED group). This group was characterized by a higher prevalence of allergic diseases (8 patients; 88.9%) than s IgAD patients without AEID (s IgAD + AIED group; 153 patients; 45.2%; p = 0.014). Both systemic diseases (3 patients; 33.3%) and organ-specific autoimmune diseases (7 patients; 77.8%) were more prevalent in the s IgAD + AIED group (s IgAD + AIED group: 19 patients 5.5%, p = 0.015; s IgAD − AEID group: 76 patients, 21.9%, p #x3c; 0.001), with an OR of 8.39 (1.94–36.19; p = 0.004). s IgAD patients with and without AIED were characterized by a higher prevalence of documented episodes of acute otitis media, allergic diseases, and autoimmune diseases than the control group.
Conclusion: The study exposes a significant association between AIED and s IgAD. We believe that s IgAD has to be excluded in AIED patients. Audiol Neurotol
I Strübing,M Gröschel,S Schwitzer,A Ernst,F Fröhlich,D Jiang,P Boyle,D Basta
Publication date 25-11-2020
Introduction: The preservation of residual hearing has become an important consideration in cochlear implant (CI) recipients in recent years. It was the aim of the present animal experimental study to investigate the influence of a pretreatment with near-infrared (NIR) light on preservation of sensory hair cells and residual hearing after cochlear implantation.
Methods: NIR was applied unilaterally (15 min, 808 nm, 120 mW) to 8 guinea pigs, immediately before a bilateral scala tympani CI electrode insertion was performed. The nonirradiated (contralateral) side served as control. Twenty-eight days postoperatively, auditory brainstem responses (ABRs) were registered from both ears to screen for hearing loss. Thereafter, the animals were sacrificed and inner hair cells (IHCs) and outer hair cells (OHCs) were counted and compared between NIR-pretreated and control (contralateral) cochleae.
Results: There was no IHC loss upon cochlear implantation. OHC loss was most prominent on both sides at the apical part of the cochlea. NIR pretreatment led to a statistically significant reduction in OHC loss (by 39.8%). ABR recordings (across the frequencies 4–32 k Hz) showed a statistically significant difference between the 2 groups and corresponds well with the apical structural damage. Hearing loss was reduced by about 20 dB on average for the NIR-pretreated group (p ≤ 0.05). Discussion/Conclusion: A single NIR pretreatment in this animal model of CI surgery appears to be neuroprotective for residual hearing. This is in line with other studies where several NIR posttreatments have protected cochlear and other neural tissues. NIR pretreatment is an inexpensive, effective, and noninvasive approach that can complement other ways of preserving residual hearing and, hence, should deserve further clinical evaluation in CI patients. Audiol Neurotol