Systematic Review of Auditory Training Outcomes in Adult Cochlear Implant Recipients and Meta-Analysis of Outcomes.

Objective: to review evidence on the efficacy of auditory training in adult cochlear implant recipients. Data Sources: PRISMA guidelines for a systematic review of the literature were followed. PubMed, Scopus, and CINAHL databases were queried on 29 June 2023 for terms involving cochlear implantation and auditory training. Studies were limited to the English language and adult patient populations. Study Selection: Three authors independently reviewed publications for inclusion in the review based on a priori inclusion and exclusion criteria. Inclusion criteria encompassed adult cochlear implant populations, an analysis of clinician- or patient-directed auditory training, and an analysis of one or more measures of speech recognition and/or patient-reported outcome. Exclusion criteria included studies with only pediatric implant populations, music or localization training in isolation, and single-sample case studies. Data Extraction: The data were collected regarding study design, patient population, auditory training modality, auditory training timing, speech outcomes, and data on the durability of outcomes. A quality assessment of the literature was performed using a quality metric adapted from the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) Working Group guidelines. Data Synthesis and Meta-Analysis: Data were qualitatively summarized for 23 studies. All but four studies demonstrated significant improvement in at least one measured or patient-reported outcome measure with training. For 11 studies with sufficient data reporting, pre-intervention and post-intervention pooled means of different outcome measures were compared for 132 patients using meta-analysis. Patient-direct training was associated with significant improvement in vowel-phoneme recognition and speech recognition in noise (p < 0.05 and p < 0.001, respectively), and clinician-directed training showed significant improvement in sentence recognition in noise (p < 0.001). Conclusions: The literature on auditory training for adult cochlear implant recipients is limited and heterogeneous, including a small number of studies with limited levels of evidence and external validity. However, the current evidence suggests that auditory training can improve speech recognition in adult cochlear implant recipients.

Cleft Lip and/or Palate Repair in Children With Hypopituitarism: Analysis of the Kids' Inpatient Database.

OBJECTIVE: Children with hypopituitarism (CwHP) can present with orofacial clefting, frequently in the setting of multiple midline anomalies. Hypopituitarism (HP) can complicate medical and surgical care; the perioperative risk in CwHP during the traditionally lower risk cleft lip and/or palate (CL/P) repair is not well described. The objective of this study is to examine the differences in complications and mortality of CL/P repair in CwHP compared to children without hypopituitarism (CwoHP). DESIGN: A retrospective cross-sectional analysis. SETTING: The 1997 to 2019 Kids' Inpatient Databases (KID). PATIENTS: Children 3 years old and younger who underwent CL/P repair. MAIN OUTCOME MEASURE(S): Complications and mortality. RESULTS: A total of 34 106 weighted cases were analyzed, with 86 having HP. CwHP had a longer length of stay (3.0 days [IQR 2.0-10.0] vs 1.0 day [IQR 1.0-2.0], P < .001) and higher rates of complications and mortality (12.8% vs 2.9%, P < .001) compared to CwoHP. Controlling for demographic factors, CwHP had 6.61 higher odds of complications and mortality than CwoHP (95% CI 3.38-12.94, P < .001). CONCLUSIONS: CwHP can present with a CL/P and other midline defects that can increase the complexity of their care. These data show a significant increase in length of stay, complications, and mortality in CwHP undergoing CL/P repair. Increased multidisciplinary attention and monitoring may be needed for these children peri- and postoperatively, especially if additional comorbidities are present. Further studies on perioperative management in this population are warranted to reduce morbidity and mortality.

Non-tuberculous Mycobacterium Rhinosinusitis in an Immunocompetent Patient.

Non-tuberculous mycobacteria (NTM) are slow-growing opportunistic pathogens that cause a variety of cutaneous, soft tissue, and pulmonary infections. On rare occasions, NTM causes chronic rhinosinusitis, with the majority of cases presenting in immunocompromised individuals. Other potential risk factors include the presence of foreign bodies, previous sinus surgery or chemoradiation, and use of contaminated water in sinus rinses. We report here a rare case of NTM rhinosinusitis in an otherwise immunocompetent 66-year-old female. The patient underwent functional endoscopic sinus surgery where intraoperative acid-fast bacteria cultures grew Mycobacterium abscessus. She received five weeks of broad-spectrum IV antibiotic therapy followed by three months of oral azithromycin, tigecycline, and linezolid. A one-year post-operative visit showed appropriate healing without crusting or visible infection. This case contributes to the small handful of documented presentations of NTM rhinosinusitis in immunocompetent patients. NTM should be considered when patients present with refractory rhinosinusitis as they may require extended courses of antibiotic treatment. Familiarity with risk factors can further expedite making a diagnosis, ensuring prompt initiation of treatment and relief of symptoms for patients.

Secondary autoimmune immune ear disease (AIED): a systematic review and meta-analysis on vestibular manifestations of systemic autoimmune and inflammatory disorders.

Secondary autoimmune inner ear disease (AIED) is often bilateral and asymmetric in patients presenting with audiovestibular symptoms due to a systemic autoimmune disease. This systematic review and meta-analysis are aimed at identifying and highlighting patterns in prevalence of vestibular dysfunction, symptom presentation, and diagnostic methods in extant literature by combining clinical context from case reports with quantitative analyses from cohort studies. Screening of articles by title, abstract, and full text was completed by four reviewers (K.Z., A.L., S.C., and S.J.). In this study, we grouped secondary AIED and systemic autoimmune diseases by pathophysiologic mechanism: (1) connective tissue disease (CTD), (2) vasculitides (VAS), (3) systemic inflammatory disorders (SID), and (4) other immune-mediated disorders (OIMD). The search for AIED disease identified 120 articles (cohorts and case reports) that met the final inclusion criteria. All 120 were included in the qualitative review, and 54 articles were included for meta-analysis. Of these 54 articles, 22 included a control group (CwC). Ninety individual cases or patient presentations from 66 articles were included for analysis in addition to the 54 cohort articles. Secondary AIED does not have a diagnostic algorithm for managing vestibular symptoms. The management of audiovestibular symptoms requires close collaboration between otolaryngologists and rheumatologists to preserve end-organ function of the ear. To improve our ability to understand the impact on the vestibular system, vestibular clinicians need to develop a standardized reporting method. Clinical presentation should frequently be paired with vestibular testing to contextually investigate symptom severity and provide higher quality care.

Impact of auditory environments on language outcomes in children with a cochlear implant.

OBJECTIVES: To analyse the effects of auditory environments on receptive and expressive language outcomes in children with a CI. DESIGN: A single-institution retrospective review was performed. The auditory environments included Speech-Noise, Speech-Quiet, Quiet, Music, and Noise. Hearing Hour Percentage (HHP) and percent total hours were calculated for each environment. Generalised Linear Mixed Models (GLMM) analyses were used to study the effects of auditory environments on PLS Receptive and Expressive scores. STUDY SAMPLE: Thirty-nine children with CI. RESULTS: On GLMM, an increase in Quiet HHP and Quiet percent total hours were positively associated with PLS Receptive scores. Speech-Quiet, Quiet, and Music HHP were positively associated with PLS Expressive scores, with only Quiet being significant for percent total hours. In contrast, percent total hours of Speech-Noise and Noise had a significant negative association with PLS Expressive scores. CONCLUSIONS: This study suggests that more time spent in a quiet auditory environment positively influences PLS Receptive and Expressive scores and that more time listening to speech in quiet and music positively influences PLS Expressive scores. Time spent in environments recognised as Speech-Noise and Noise might negatively impact a child's expressive language outcomes with a CI. Future research is needed to better understand this association.

Patient-Related Factors Do Not Predict Use of Computer-Based Auditory Training by New Adult Cochlear Implant Recipients.

OBJECTIVE: The use of computer-based auditory training (CBAT) after cochlear implantation is associated with improved speech recognition and real-world functional abilities. However, patient-related factors associated with CBAT use remain unknown. This study seeks to identify such factors and recognize those at risk for not implementing CBAT. STUDY DESIGN: Prospective natural experiment. SETTING: Tertiary academic center. PATIENTS: A total of 117 new adult cochlear implant (CI) recipients with bilateral moderate-to-profound hearing loss. INTERVENTIONS/MAIN OUTCOME MEASURES: Patient demographic and lifestyle information, preimplantation aided speech recognition scores, Cochlear Implant Quality of Life (CIQOL) domain and global scores, CIQOL-Expectations scores, and CBAT use in the first 3 months after activation. Patient-related variables included age, sex, race, duration of hearing loss before implantation, hours of CI use per day, hearing-aid use before implantation, living arrangements/marital status, annual household income, employment, technology use, and education. RESULTS: Overall, 33 new CI users (28.2%) used CBAT in the first 3 months after activation. On bivariate analysis of the pre-CI CIQOL scores, CIQOL-Expectations score, aided speech recognition scores, and demographic/lifestyle factors examined, regular use of smartphone, tablet, or computer technology was significantly associated with an increased likelihood of CBAT use (odds ratio, 9.354 [1.198-73.020]), whereas higher CIQOL-Expectations emotional domain scores were associated with a lower likelihood of CBAT use (d = -0.69 [-1.34 to -0.05]). However, using multivariable analysis to control for potential confounding factors revealed no significant associations between CBAT use in the first 3 months after cochlear implantation and any examined factor. CONCLUSIONS: No associations between patient demographic, lifestyle, or pre-CI speech recognition and patient-reported outcome measures and CBAT use were identified. Therefore, discussions with all patients after implantation on the availability of CBAT and its potential benefits are warranted. In addition, given the limited overall use of CBAT and its association with improved CI outcomes, future studies are needed to investigate facilitators and barriers to CBAT use.

Association of computed tomography scores to psychophysical measures of olfaction: A systematic review and meta-analysis.

BACKGROUND: While various sinus computed tomography (CT) scoring systems have been proposed and used in the literature, no single system has been identified as superior. The strength of associations between CT scoring systems and measures of olfaction also remains unclear. METHODS: A systematic review of PubMed, CINAHL, Scopus, and the Cochrane Library was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies that reported both CT scores and measures of olfaction in a cross-sectional manner were included. RESULTS: A total of 37 studies were eligible for meta-analysis. Of 8035 patients with chronic rhinosinusitis, 55.6% were male patients and 53.2% had chronic rhinosinusitis with nasal polyps. Analysis by meta-regression was performed of Lund-Mackay (LM) versus Smell Identification Test-40 (SIT-40; 12 studies), Brief Smell Identification Test (BSIT; 10 studies), Sniffin' Sticks (SS; 10 studies), and Toyota & Takagi (T&T) olfactometry (four studies). A significant moderate association was found between LM and SIT-40 (R2  = 0.612, p < 0.001) and LM and SS (R2  = 0.612, p < 0.001). An association between LM and BSIT approached significance (R2  = 0.461, p = 0.054). No significant associations were noted between LM and T&T olfactometry and between LM and SS when stratified by nasal polyp status. CONCLUSION: There is a significant moderate association of current CT scoring systems to SIT-40 and SS. Further research should focus on associations of objective measures of olfaction to CT scores of the nasal cavity, sinuses, and olfactory cleft, as well as other disease markers.

Association of Sinonasal Computed Tomography Scores to Patient-Reported Outcome Measures: A Systematic Review and Meta-analysis.

OBJECTIVE: To perform a systematic review of proposed sinus computed tomography (CT) scoring systems and determine their association with patient-reported outcome measures (PROMs). DATA SOURCES: PubMed, CINAHL, Scopus, and Cochrane Library. REVIEW METHODS: A systematic search was conducted following the PRISMA guidelines (Preferred Reporting Items for Systematic Reviews and Meta-analyses) for studies describing CT scores and PROMs in patients with chronic rhinosinusitis. RESULTS: A total of 144 studies were included. Out of 20,741 patients, 53.6% were male and 55.5% had nasal polyposis. A meta-analysis of correlations revealed a moderate correlation between Lund-McKay (LM) and the 22-item Sinonasal Outcome Test (SNOT-22; r = 0.434, P < .001) and a weaker correlation between LM and the 20-item Sinonasal Outcome Test (SNOT-20; r = 0.257, P = .039). Meta-regression also revealed a weak association between LM and SNOT-20 (n = 25 studies) but no significant associations between Zinreich score and SNOT-22 or LM scores and PROMs, including SNOT-22 (n = 94 studies), Rhinosinusitis Disability Index (n = 25), nasal obstruction visual analog scale (n = 15), Chronic Sinusitis Survey (n = 12), Total Nasal Symptom Score (n = 4), Total Symptom Score (n = 3), and 12-Item Short Form Health Survey (n = 3). CONCLUSION: There is essentially little association between radiologic grade and PROMs. CT grading systems with improved clinical utility are needed.

Understanding Patient Expectations Before Implantation Using the Cochlear Implant Quality of Life-Expectations Instrument.

Importance: Previous research suggests that clinicians view realistic patient expectations as the most important nonaudiological factor in the decision to proceed with a cochlear implant (CI). However, clinicians have few data to determine whether patients' outcome expectations are realistic. Objective: To address this unmet clinical need through the development and psychometric analysis of a new patient-reported outcome measure, the CI Quality of Life (CIQOL) Expectations. Design, Setting, and Participants: This cross-sectional study was conducted at a tertiary CI center from February 26, 2020, to August 31, 2021. First, a team comprising 2 CI audiologists, a CI surgeon, a hearing scientist, and 2 psychometricians with experience in instrument development converted all items from the CIQOL-35 Profile instrument into statements reflecting expected outcomes. Then, cognitive interviews with 20 potential CI users assessed the clarity and comprehensiveness of the new instrument. Next, responses to the CIQOL-Expectations instrument for 131 potential adult CI candidates were psychometrically analyzed using confirmatory factor analysis and item response theory. Finally, degree to which patient expectations changed from before to after and their CI evaluation appointments was measured. Intervention: The CIQOL-Expectations instrument. Results: Of 178 participants, 85 (47.8%) were female, and there was 1 (0.6%) Asian, 26 (14.6%) Black or African American, 1 (0.6%) Latinx, and 150 (84.3%) White individuals. No major content or grammar changes were identified during the cognitive interviews. Overall, all CIQOL domains demonstrated adequate to strong psychometric properties. Several domains did not meet all a priori established indicators of model fit or ability to separate CI users based on response patterns, but all met most indicators. Potential CI users demonstrated the highest mean (SD) expectation scores for the environment (70.2 [20.8]) and social (68.4 [18.0]) domains. In addition, the entertainment (20 [15.3%]) and environment (31 [24.4%]) domains had the highest percentage of patients with expectation scores of 100. Yet, normative CIQOL-35 Profile data from experienced CI users suggested few patients obtain this high degree of functional benefit after implant. Conclusions and Relevance: The results of this cross-sectional study suggest that the CIQOL-Expectations instrument may provide an opportunity to assess potential CI users' expected outcomes using modification of an established CIQOL instrument and a patient-centered framework. The included items and domains reflect real-world functional abilities valued by CI users and may provide opportunities for an evidence-based shared decision-making approach to the CI evaluation process. With this instrument, clinicians can compare individual patients' pre-CI outcome expectations with established normative data and provide appropriate counseling.

Normative Cochlear Implant Quality of Life (CIQOL)-35 Profile and CIQOL-10 Global Scores for Experienced Cochlear Implant Users from a Multi-Institutional Study.

OBJECTIVE: Although adult cochlear implant (CI) outcomes have primarily focused on speech recognition scores, the rigorous development of a CI-specific patient-reported outcome measure provides an opportunity for a more comprehensive and ecologically valid approach to measure the real-world functional abilities of adult CI users. Here, we report for the first time normative Cochlear Implant Quality of Life (CIQOL)-35 Profile and global scores and variance for a large, multi-institutional sample of adult CI users. STUDY DESIGN: Cross-sectional study design. SETTING: CI centers in the United States. PATIENTS: Seven hundred five adults with bilateral moderate to profound hearing loss with at least 1 year of CI use. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: CIQOL-35 Profile and CIQOL-10 Global scores. RESULTS: During the development of the CIQOL instruments, 1,000 CI users from all regions of the United States were invited to participate in studies. Of these, 705 (70.5%) completed all portions of the study, and their data are reported here. Mean CIQOL domain scores were highest (indicating better function) for the emotional and social domains and lowest for listening effort. The entertainment and social domains demonstrated the widest distribution of scores and largest standard deviations, indicating greatest variability in function. Overall, there were minimal ceiling and floor effects for all domains. CONCLUSION: Normative scores from a large sample of experienced adult CI users are consistent with clinical observations, showing large differences in functional abilities and large variability. Normative CIQOL data for adult CI users have the potential to enhance preoperative discussions with CI candidates, improve post-CI activation monitoring, and establish standards for CI centers.

Craniosynostosis Surgery for Increased Intracranial Pressure.

INTRODUCTION: Craniosynostosis is the premature fusion of 1 or more cranial sutures. The presentation may vary, and there are differing opinions regarding surgical indications and timing. Though increased intracranial pressure (ICP) is a well-established risk of craniosynostosis, its response to treatment is not uniform. This study aims to identify the signs and symptoms indicative of increased ICP that are most likely to improve after craniosynostosis surgery. METHODS: Pre- and post-operative data were retrospectively collected from patients at our institution with syndromic and non-syndromic craniosynostosis from January 2009 to June 2020. Demographics, symptoms (headache, nausea, emesis, and lethargy), signs (visual disturbances and papilledema), and imaging characteristics (copper beaten changes), if available, were analyzed. RESULTS: One hundred fifty-three children with craniosynostosis were identified, and 56 with preoperative symptoms met inclusion criteria. Older age was significantly correlated with the number of symptoms improved postoperatively ( P = 0.015). Papilledema, headache, nausea, and irritability (if present preoperatively) were the features most likely to improve after craniosynostosis repair. Optic nerve or disc anomalies, feeding difficulties, seizures, and need for glasses were least likely to improve. CONCLUSIONS: Older children undergoing craniosynostosis surgery had a greater number of improved symptoms. Classical features of increased ICP were more likely to improve than developmental difficulties and multifactorial causes. These findings may be useful when considering craniosynostosis repair in a symptomatic child.