Management of Cholesteatoma: Hearing Rehabilitation.

Surgical treatment of cholesteatoma requires a highly individualized approach, balancing disease eradication with hearing preservation, and necessitates thorough patient counseling on expected auditory outcomes and options for rehabilitation. The choice between canal wall up and canal wall down mastoidectomy can influence hearing outcomes. Hearing rehabilitation options following cholesteatoma surgery include air- and bone-conduction devices and are individualized for the patient. Assistive listening devices such as frequency modulation systems, hearing loops, and Bluetooth technology are critical for enhancing speech understanding in noisy environments for patients who employ various hearing rehabilitation methods and require further advanced solutions.

Clinical Practice Guideline: Age-Related Hearing Loss Executive Summary.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with an explanation of the support in the literature, the evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the Guideline Development Group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life. (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related quality of life at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

Clinical Practice Guideline: Age-Related Hearing Loss.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with explanation of the support in the literature, evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life (QOL). (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related QOL at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

Auditory Outcomes Following Cochlear Implantation in Children with Unilateral Hearing Loss.

OBJECTIVE: Unilateral hearing loss (UHL) in children is associated with speech and language delays. Cochlear implantation (CI) is currently the only rehabilitative option that restores binaural hearing. This study aims to describe auditory outcomes in children who underwent CI for UHL and to determine the association between duration of hearing loss and auditory outcomes. STUDY DESIGN: Retrospective case series. SETTING: Three tertiary-level, academic institutions. PATIENTS: Children <18 years with UHL who underwent CI between 2018 and 2021. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Speech perception and Speech, Spatial and Qualities of Hearing Scale (SSQ) were assessed postimplantation. Scores >50% on speech perception and SSQ scores >8 points were considered satisfactory. Associations between duration of UHL and implantation age and outcomes were assessed using Spearman's rank correlation. RESULTS: Of the 38 children included, mean age at CI was 7.9 ± 3.2 years and mean UHL duration was 5.0 ± 2.8 years. Mean datalogging was 8.1 ± 3.1 hours/day. Mean auditory testing scores were SSQ, 7.9 ± 1.2; BABY BIO, 68.1 ± 30.2%; CNC, 38.4 ± 28.4%; WIPI, 52.5 ± 23.1%. Scores >50% on CNC testing were achieved by 40% of patients. SSQ scores >8 points were reported by 78% (7/9) of patients. There were no significant correlations between UHL duration and auditory outcomes. CONCLUSION: Overall, children with UHL who undergo CI can achieve satisfactory speech perception scores and SSQ scores. There were no associations between duration of hearing loss and age at implantation with auditory outcomes. Multiple variables may impact auditory outcomes, including motivation, family support, access to technology, and consistent isolated auditory training postactivation and should be taken into consideration in addition to age at implantation and duration of UHL in determination of CI candidacy.

Intelligence quotient testing in children with hearing loss: A systematic review.

OBJECTIVE: The objective of the current study is to perform a systematic review of the research literature to evaluate the impact of hearing loss on intelligence quotient (IQ) scores in pediatric patients. DATA SOURCES: Ovid MEDLINE, EMBASE, CINAHL, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from their inception up to December 21st, 2021. REVIEW METHODS: Studies evaluating neurocognitive testing and hearing loss in children aged 21 years old or younger who had not undergone auditory rehabilitation were included in the study. Two independent reviewers evaluated titles, abstracts, and full texts for all included studies. RESULTS: The literature search yielded 3199 studies of which 431 studies underwent full-text screening. 21 studies were ultimately selected for inclusion and contained a total of 1716 pediatric patients assessed through 13 different validated tests of intelligence. Six studies included both hearing impaired (HI) and normal hearing (NH) patients, and IQ testing results. CONCLUSION: The results of this large systematic review demonstrate that hearing impaired children may perform lower than their age-matched normal hearing peers on IQ testing across a battery of IQ testing modalities.

Cochlear Implantation Outcomes in Pediatric Unilateral Hearing Loss: Impact of Device Use and Direct Input.

OBJECTIVE: To investigate the impact of daily cochlear implant (CI) use on speech perception outcomes among children with unilateral hearing loss (UHL). STUDY DESIGN: Multi-institutional retrospective case series of pediatric patients with UHL who underwent CI between 2018 to 2022. SETTING: Three tertiary children's hospitals. METHODS: Demographics were obtained including duration of deafness and age at CI. Best consonant-nucleus-consonant (CNC) word scores and data logs describing hours of CI usage were assessed postimplantation. Use of direct audio input (DAI) during rehabilitation was recorded. RESULTS: Twenty-seven children were included, with a mean age at CI of 7.8 years. Mean datalogging time was 7.8 ± 3.0 hours/day. 40.7% of children utilized daily DAI. The mean CNC score using the best score during the study period was 34.9%. There was no significant correlation between hours of CI usage and CNC score. There was a significant improvement in CNC score associated with whether the child used DAI during rehabilitation (CNC 50.91% [yes] vs 23.81% [no]), which remained significant when adjusting for age at CI, duration of deafness, and data log hours. CONCLUSION: Unlike children with bilateral hearing loss and CI, children with UHL and CI demonstrate no significant correlation between hours of daily CI usage and CNC scores. However, children who used DAI during postoperative rehabilitation achieved significantly higher CNC scores than those who did not. This suggests that rehabilitation focused on isolated listening with the implanted ear maybe critical in optimizing outcomes with CI in UHL patients.

Longitudinal outcomes with intracapsular tonsillectomy, a single surgeon's experience.

PURPOSE: To assess the risk of tonsillar regrowth and post-operative complications associated with intracapsular tonsillectomy (IT) when performed by a single surgeon using a standardized technique. MATERIALS AND METHODS: The current study was conducted as a retrospective chart review of all IT performed by a single surgeon between November 11, 2009 and July 22, 2020 at the Cleveland Clinic and the Cleveland Clinic Beachwood Family Health and Surgery Center . Data collection included patient demographics, surgical data, post operative results, complications, and available long-term follow-up data. RESULTS: There were 221 ITs performed between November 2009 and July 2020. The post operative bleeding rate was 3.5 %. A single adult patient required re-operation for bleeding. Rate of tonsillar regrowth was 3.9 % (n = 7) and 1.1 % (n = 2) required re-operation (total tonsillectomy). No patients in the current study developed post-operative dehydration or had excessive post-operative pain requiring an emergency department visit or hospitalization. CONCLUSIONS: The current study demonstrated similar rates of post operative bleeding after IT when compared to established rates following TT. The current study's regrowth rate was 3.9 % with a low 1.1 % rate of re-operation. This study adds to a growing body of literature supporting the use of IT due to lower complication rates when compared to TT, including post-operative bleeding, dehydration, and pain, with minimal rate of re-operation for bleeding or regrowth.

Attention-deficit/hyperactivity disorder in children with hearing loss.

OBJECTIVE: To evaluate rates of Attention-Deficit/Hyperactivity Disorder (AD/HD) diagnosis among pediatric patients with normal hearing (NH) and hearing loss (HL) with and without comorbidities. STUDY DESIGN: Retrospective cohort study of NH and HL patients following chart review of all pediatric patients with a history of tympanostomy tube placement treated at the Cleveland Clinic Foundation between 2019 and 2022. METHODS: Patient demographic information, hearing status (type of HL, laterality, severity), and comorbidities including prematurity, genetic syndromes, disorders with neurological impairment, and autism spectrum disorder (ASD) were collected. Rate of AD/HD amongst HL and NH cohorts with and without comorbidities were compared using Fisher's exact test. Covariate-adjusted analysis was also completed (sex, current age, age at tube placement, and OSA). The primary outcome of interest was rates of AD/HD among children with NH and HL, and the secondary outcome of interest was the impact of comorbidities on rates of AD/HD diagnosis in these cohorts. RESULTS: Of the 919 screened patients between 2019 and 2022, there were 778 NH patients and 141 HL patients (80 bilateral, 61 unilateral). HL ranged from mild (n = 110) to moderate (n = 21) to severe/profound (n = 9). Overall, the rate of AD/HD was significantly higher in HL children (12.1% HL vs 3.6% NH, p < 0.001). Of the 919 patients, 157 had comorbidities. In children without comorbidities, HL children still had significantly higher rates of AD/HD compared to NH children (8.0% vs 1.9%, p = 0.02), but there was loss of significance after covariate adjustment (p = 0.72). CONCLUSION: The rate of AD/HD among children with HL (12.1%) is higher than the rate of AD/HD in NH children (3.6%), consistent with previous findings. After excluding patients with comorbidities and adjusting for covariates, there are similar rates of AD/HD between HL and NH patients. Given high rates of comorbidities and AD/HD in HL patients and potential for augmented developmental challenges, clinicians should have a low threshold to refer children with HL for neurocognitive testing, particularly those with any of the comorbidities or covariates described in this study.

Newborn hearing screening methodology impacts the timing of diagnosis for auditory neuropathy spectrum disorder.

INTRODUCTION: Auditory Neuropathy Spectrum Disorder (ANSD) accounts for 10 % to 15 % of pediatric hearing loss. In most cases, otoacoustic emissions (OAE) are present as the outer hair cell function is normal, and the auditory brainstem response (ABR) is abnormal. Newborn hearing screen (NBHS) is completed using OAE or ABR depending on the institution. Because OAEs are often present in ANSD, NBHS done solely with OAE can miss and delay diagnosis of patients with ANSD. OBJECTIVES: To assess whether NBHS methodology impacts the age of diagnosis of ANSD. METHODS: This is a retrospective study of patients, 0-18 years of age, diagnosed with ANSD at two tertiary pediatric hospitals from 1/01/2010 to 12/31/2018 after referral from NBHS performed in the community. Data recorded included patient demographics, method of NBHS, NICU stay, and age at ANSD diagnosis. RESULTS: 264 patients were diagnosed with ANSD. Of those, 123 (46.6 %) were female, and 141 (53.4 %) were male. Ninety-seven (36.8 %) were admitted to NICU and the mean stay was 6.98 weeks (STD = 10.7; CI = 4.8-9.1). The majority (244, 92.4 %) of patients had NBHS with ABR, and 20 (7.5 %) had NBHS with OAE. Patients screened with ABR were diagnosed with ANSD earlier than those who screened with OAE, with a mean age of 14.1 versus 27.3 weeks (p = 0.0397, CI = 15.2-39.3). Among those screened with ABR, median age at diagnosis was 4 months for NICU infants and 2.5 months for infants with no history of NICU stay over 5 days. In comparison, median diagnosis age was 8 months for non-NICU infants screened with OAEs. CONCLUSION: Patients with ANSD who had NBHS with ABR were diagnosed earlier than those with OAE. Our data suggest that universal screening with ABR may facilitate earlier diagnosis of ANSD and earlier evaluation for aural rehabilitation, especially in high-risk cohorts such as NICU patients. Further research is needed into factors that contribute to earlier diagnosis among patients screened with ABR.

Refractory tympanostomy tube otorrhea: Outcomes with otowick placement.

OBJECTIVE: To evaluate rates of resolution of and antibiotic use in refractory otorrhea (otorrhea ≥2 weeks with no more than 2 days of otorrhea resolution during the episode) with otowick placement in comparison to medical therapy and tube removals with or without replacements. STUDY DESIGN: Retrospective cohort study of pediatric patients with a history of tympanostomy tube placement and refractory otorrhea between 2017 and 2022. SETTING: Cleveland Clinic Foundation (CCF) pediatric otolaryngology group. METHODS: Demographic data (sex, race, and age at tympanostomy tube placement) were collected. Outcome measures included number of oral and topical antibiotic treatments, duration of otorrhea episode, and resolution of otorrhea at 1 and 3 months follow-up. Wilcoxon rank sum and Kruskal-Wallis tests were used to compare number of antibiotics treatments. An ANOVA test and linear regression model using log-transformed data were used to compare duration of otorrhea. Fisher's exact test was used to compare rates of otorrhea recurrence. RESULTS: 70 patients met inclusion criteria: 37 male (52.9%), 33 female (47.1%), with an average age at tube placement of 29.66 months. There was a statistically significant decrease in total duration of otorrhea (in days) with otowicks (17.29 ± 13.13), compared to patients receiving medical therapy (27.09 ± 22.02) and tube removals and replacements (29.63 ± 19.95) (p = 0.025). There was no significant difference between the follow-up rates of resolution at 1 month (p = 1) and 3 months (p = 0.12). CONCLUSION: Otowick use is associated with shorter duration of otorrhea and can be considered as a first-line treatment for refractory otorrhea. Otowicks and medical therapy alone can reduce need for tube removals and the associated risk of anesthesia.

Updated Centers for Disease Control and Prevention Guidelines on Opioid Prescribing: What Should Surgeons Know?

The Centers for Disease Control and Prevention (CDC) recently published a 2022 guideline on opioid prescribing for acute, subacute, and chronic pain. This information is relevant to surgeons because many patients receive their first opioid prescription after surgery. When prescribing opioids, surgeons walk the line between benefit and harm. Many of the CDC recommendations mirror the AAO-HNS Clinical Practice Guideline: Opioid Prescribing for Analgesia After Common Otolaryngology Operations. For example, opioids are not recommended as first-line therapy for acute pain from otolaryngology-head, and neck surgery procedures. New insights include safeguards and strategies to mitigate the risk of complications in patients with chronic pain undergoing surgical procedures. Consultation with a pain specialist should be considered for patients transitioning from acute to chronic pain, cognizant of the risks of abrupt discontinuation of opioids in patients with opioid use disorder. This article summarizes key considerations for providing individualized, evidence-based perioperative pain management.

Intraoperative Neural Response Telemetry and Auditory Outcomes in Pediatric Cochlear Implantation.

OBJECTIVE: To evaluate the relationship between intraoperative neural response telemetry (NRT) and postoperative auditory testing outcomes in children. STUDY DESIGN: Retrospective study. SETTING: Tertiary-care academic center. METHODS: Children who underwent cochlear implantation using the Cochlear Corporation device between 2010 and 2019 were included. Associations of average NRT and the slope of amplitude with postoperative auditory outcomes including functional auditory measure Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and speech perception testing (consonant-nucleus-consonant [CNC], Pediatric AzBio [BABY BIO], Hearing In Noise Test [HINT], and Northwestern University Children's Perception of Speech [NU-CHIPS]), measured between 6 and 57 months after implantation, were assessed using Spearman's rank correlation (ρ). RESULTS: Thirty-eight patients (19 female, 19 male) and 54 ears were included. The median age of implantation was 20.6 months (range 9.6 months to 10.6 years). Eight (21%) children had neurologic disorders such as stroke, epilepsy, cerebral palsy, and other causes. Thirteen (34%) children had connexin mutations. Average NRT was not significantly correlated with postoperative auditory outcomes (IT-MAIS [ρ = -0.08, p = .74], CNC [ρ = 0.19, p = .32], BABY BIO [ρ = 0.21, p = .29], HINT [ρ = 0.05, p = .83]) and NU-CHIPS (ρ = 0.21, p = .28). The average slopes of amplitude and comfort level were not strongly correlated with any auditory outcomes (p > .05). CONCLUSIONS: Intraoperative NRT was not correlated with any postoperative functional auditory outcomes. Patient counseling should include discussions that a subpar intraoperative cochlear response does not preclude favorable speech and auditory outcomes.

Auditory Outcomes in Children Who Undergo Cochlear Implantation Before 12 Months of Age: A Systematic Review.

OBJECTIVE: To systematically review the literature to determine auditory outcomes of cochlear implantation in children ≤12 months old. DATA SOURCE: PubMed, EMBASE, Medline, CINAHL, Cochrane, Scopus, and Web of Science databases were searched from inception to 9/1/2021 using PRISMA guidelines. REVIEW METHODS: Studies analyzing auditory outcomes after cochlear implantation (CI) in children ≤12 months of age were included. Non-English studies and case reports were excluded. Outcome measures included functional and objective auditory results. Two independent reviewers evaluated each abstract and article. Heterogeneity and bias across studies were evaluated. RESULTS: Of 305 articles identified, 17 met inclusion criteria. There were 642 children ages 2 to 12 months at CI. The most common etiologies of hearing loss were congenital CMV, meningitis, idiopathic hearing loss, and GJB2 mutations and other genetic causes. All studies concluded that early CI was safe. Overall, outcomes improved following early CI: IT-MAIS (9 studies), LittlEARS (4 studies), PTA (3 studies), CAP (3 studies), GASP (3 studies), and LNT (3 studies). Nine studies compared outcomes to an older implantation group (>12 months); of these (n = 450 early CI, n = 1189 late CI), 8 studies showed earlier CI achieved comparable or better auditory outcomes than later implantation, whereas 1 study (n = 120) concluded no differences in speech perception improvement. CONCLUSION: Auditory outcomes were overall improved in children ≤12 months old undergoing CI. Studies that compared early to late CI demonstrated similar or better auditory outcomes in early implantation group. Given the comparable safety profile and critical time period of speech and language acquisition, earlier CI should be considered for infants with hearing loss.

Health-Related Quality of Life in Children With Unilateral Sensorineural Hearing Loss Following Cochlear Implantation.

OBJECTIVE: Evaluate health-related quality of life (HR-QOL) benefits with cochlear implantation (CI) in children with unilateral sensorineural hearing loss (USNHL) versus bilateral sensorineural hearing loss (BSNHL). STUDY DESIGN: A cross-sectional survey of parents of children who underwent CI for USNHL and BSNHL. SETTING: Tertiary care academic centers. METHODS: The "Children with cochlear implants: parental perspectives" survey was administered. Parents rated responses on a 5-point Likert scale. Scores greater than 3.0 were considered favorable. Responses were recorded within 8 domains and groups were compared with respect to domain scores. Analysis of covariance models was used to compare groups while adjusting for age at implantation and duration of implant use. RESULTS: There were 31 patients with USNHL and 27 patients with BSNHL. The average age of implantation in BSNHL patients was 1.9 and 6.7 years for USNHL. Parents of all children answered favorably in all domains. When adjusted for age at implantation and duration of implant use, parents of BSNHL children responded significantly more favorably only in 2 domains. When comparing patients with older age or prolonged duration of hearing loss in the USNHL cohort, there were favorable responses in all domains with no significant differences between groups. CONCLUSION: There are HR-QOL benefits of CI in USNHL children; less pronounced favorable results were noted only in 2 domains when compared to BSNHL children. Benefits were noted with CI in USNHL children at an older age at implantation or prolonged duration of hearing loss. Therefore, these factors should not be absolute contraindications for CI in USNHL.

Voice outcomes following surgical treatment for pediatric vocal fold nodules: A systematic review and meta-analysis.

OBJECTIVES: This study aimed to systematically review the literature to determine outcomes following surgical treatment of pediatric vocal fold nodules. METHODS: Studies with patients ≤18 years with nodules who underwent surgery were reviewed for dysphonia improvement and recurrence in PubMed, EMBASE, Medline, CINAHL, Cochrane, Scopus, and Web of Science databases, searched from inception to November 1, 2022 using PRISMA guidelines. Non-English studies and case reports were excluded. Two evaluators independently reviewed each abstract and article. Heterogeneity and bias across studies were evaluated and meta-analysis was performed. RESULTS: The literature search yielded 655 articles; 145 underwent full-text screening and eight were selected for systematic review and meta-analysis. There were 311 children with nodules, aged 2-18 years, with male-to-female ratio of 3.6:1. There were no surgical complications. Voice therapy was inconsistently reported. Follow-up time ranged from 1 month to 10 years. One study concluded that neither surgery nor voice therapy was effective, while five studies concluded that dysphonia improved with surgery. Voice grading by GRBAS, objective voice measures, and lesion size were improved following surgery, when reported. Meta-analysis of six studies demonstrated improvement in dysphonia in 90% of children post-operatively (95% CI: 74-99%). Meta-analysis of four studies showed that recurrence occurred in 19% of children (95% CI: 13-23%). CONCLUSION: This systematic review suggests possible post-operative improvement in dysphonia for pediatric patients with vocal fold nodules; however, study measures, methods, and surgery utilized were heterogeneous and results should be interpreted cautiously. In order to better understand surgical outcomes, future studies should include standardized definition of nodules and objective measures of voice.

Association of Ear Anomalies and Hearing Loss Among Children With 22q11.2 Deletion Syndrome.

OBJECTIVE: To identify inner and middle ear anomalies in children with 22q11.2 deletion syndrome (22q11DS) and determine associations with hearing thresholds. STUDY DESIGN: Retrospective study. SETTING: Two tertiary care academic centers. METHODS: Children presenting with 22q11DS between 2010 and 2020 were included. Temporal bone imaging with computed tomography or magnetic resonance imaging was reviewed by 2 neuroradiologists. RESULTS: Twenty-two patients (12 female, 10 male) were identified. Forty-four ears were evaluated on imaging. There were 15 (34%) ears with abnormal semicircular canals, 14 (32%) with abnormal vestibules, 8 (18%) with abnormal ossicles, 6 (14%) with enlarged vestibular aqueducts, 4 (9.1%) with abnormal facial nerve canals, and 4 (9.1%) with cochlear anomalies. There were 25 ears with imaging and audiometric data. The median pure tone average (PTA) for ears with any structural abnormality was 41.0 dB, as compared with 28.5 dB for ears without any structural abnormality (P = .21). Of 23 ears with normal imaging, 6 (26%) had hearing loss in comparison with 13 (62%) of 21 ears with abnormalities (P = .02). Total number of anomalies per ear was positively correlated with PTA (Pearson correlation coefficient, R = 0.479, P = .01). PTA was significantly higher in patients with facial nerve canal anomalies (P = .002), vestibular aqueduct anomalies (P = .05), and vestibule anomalies (P = .02). CONCLUSIONS: Semicircular canal, ossicular, vestibular aqueduct, and vestibular anomalies were detected in children with 22q11DS, especially in the setting of hearing loss. Careful evaluation of anatomic anomalies is needed prior to surgical intervention in these patients.

Speech and Language outcomes in Auditory Neuropathy Spectrum Disorder (ANSD) children managed with amplification.

OBJECTIVE: To evaluate speech and language outcomes in children with Auditory Neuropathy Spectrum Disorder (ANSD) without significant comorbidities who received hearing rehabilitation in the form of hearing aids and/or cochlear implantation. METHODS: Retrospective chart review of pediatric ANSD patients at a large academic tertiary care institution from 2010 to 2019. Patients were included if they received a diagnosis of bilateral ANSD, had minimal to no comorbidities, and had speech and language testing (SLT) on at least two occasions. RESULTS: 51 patients were reviewed and 7 met inclusion criteria. Average age at ANSD diagnosis was 1 year and 11 months, and average age of hearing aid fitting was 3 years and 3 months. Hearing loss ranged from mild to profound, with four of the children wearing behind (BTE) hearing aids and three eventually receiving cochlear implants. Four of five patients who received hearing aids prior to their first speech and language evaluation demonstrated a delay at their initial evaluation, and all five patients continued to demonstrate a delay at their most recent evaluation, despite appropriate audiologic management and speech and language therapy. There were two patients who were unaided at the time of their initial and latest evaluations; one patient showed a delay at both timepoints, and one patient showed no speech delay at either timepoint. CONCLUSIONS: Pediatric ANSD patients, who are otherwise typically developing and received hearing rehabilitation and speech and language therapy, continue to show a speech and language delay (SLD). This outcome underscores the importance of close monitoring of speech and language development, providing early amplification and/or cochlear implantation, and promoting additional education and psychosocial support.

Utility of intraoperative neural response telemetry in pediatric cochlear implants.

OBJECTIVE: The objective of this study is to evaluate the relationship between intraoperative neural response telemetry (NRT) and postoperative Threshold (T) and Comfort (C) levels and their correlation over time after cochlear implantation (CI). METHODS: A retrospective chart review was conducted of patients less than 18 years of age who had CI with a Cochlear™ device and NRT at an academic center from 2010 to 2019. Data collected included demographics, extrapolated NRT threshold (tNRT) and slope of amplitude for electrodes 1, 6/7, 11/12, 16, and 22, and postoperative T and C levels at initial activation and 1 month, 3 months, and 1 year post-activation. Associations between T and C levels and slope of amplitude or tNRT were assessed using Spearman's rank correlation. RESULTS: 39 patients (65 CIs) were included. Intraoperative tNRT correlated strongly with T and C levels at 1 month, 3 months, and 1 year post-activation on nearly all electrodes. Electrodes 6/7 and 11/12 at 3 months and electrodes 6/7 at 1 year did not correlate with T and C levels. There was no significant relationship between the slope of amplitude for nearly all electrodes and the T or C levels post-activation. CONCLUSION: NRT is invaluable in children, with significant correlation found between tNRT and T and C levels over time on nearly all electrodes. There are changes in T and C levels in electrodes 6/7 and 11/12 over time, and close surveillance is beneficial to tailor programming as needed.