Creation of a Prototype Cochlear Training Model.

OBJECTIVE: Creation of a novel 3D-printed physical cochlear model that demonstrated the feasibility of creating the model, and impact of a Graphical User Interface (GUI) system on training insertion metrics. STUDY DESIGN: Feasibility study with a pilot prospective data collection. SETTING: Tertiary academic center. METHODS: The study was IRB exempt. Five resident trainees (PGY1-PGY5) practiced electrode insertions in cadaveric temporal bones before using the simulator. Nine students were educated on how to hold the electrodes and position them, and then allowed to use the simulator. All trainees were instructed that slower insertions were favorable. One cochlear implant (CI) surgeon used the simulator. The GUI captured the real video feed, but also provided distance, trajectory, and velocity measurements. The program is designed to plot the real-time depth of insertion and speed of insertion of the electrode; the user is also provided real-time occurrence of any kinks and back-outs. RESULTS: A total of 14 trainees and 1 CI surgeon inserted the electrode at least 5 times without the use of the GUI (before) and then at least 5 times with the use of the GUI (after). Average Speed before and after (100.84 and 53.23 mm/s); Average minimum speed before and after (59.34 and 9.65 mm/s); and Average maximum speed before and after (416 and 285.81 mm/s). Statistically significant improvements were noted in all the measured speeds of insertion (P < .001). The other variables improved but not to a statistical significance. CONCLUSIONS: Real-time training using the 3D-printed model and GUI for cochlear implantation can help improve surgical resident training and comfort levels with electrode insertion for surgical trainees. The advantage of this model is that surgeons/trainees can use it as many times as they like, as the whole set-up is easy, economical, and reusable. The real time graphical user interface enhances training and retention of the practiced skills.

Variation in cochlear size: A systematic review.

BACKGROUND: Advancements in imaging and implantation technology have invited reexamination of the classic teaching that the human cochlea maintains uniform size across demographics. Yet, studies yield conflicting results and relatively few broad systematic reviews have examined cochlear size variation. PURPOSE: The purpose of this study is to quantify cochlear variability across eight different measurement categories and suggest normative values and ranges for each with consideration of disease state and gender where possible. METHODS: A systematic search was conducted up to October 1, 2022, using the search terms "Cochlea/anatomy and histology"[Mesh]) AND 'size'" with filters "Humans" and "English" across three databases (PubMed, CINAHL, Medline). Further inclusion criteria involved reporting of numerical measurements in any of the eight included categories. RESULTS: Of the 625 articles manually reviewed for relevance by title and abstract, 91 were selected for full-text review and 33 met all eligibility criteria. 5,791 cochleae were included and weighted means and ranges were calculated: "A" value (defined as the distance from the round window, through the modiolus, to the oppsite lateral wall) = 9.23 mm (8.43-10.4 mm, n = 2559); cochlear duct length (CDL) = 33.04 mm (range 28.2-36.4 mm, n = 2252); cochlear height = 5.14 mm (2.8-6.9 mm, n = 2098); the basal turn lumen diameter = 2.09 mm (1.7-2.2 mm, n = 617); "B" value (defined as perpendicular to "A" value and in the same plane) = 6.52 mm (5.73-6.9 mm, n = 908); width of the basal turn = 6.4 mm (6.22-6.86 mm, n = 356); height of the basal turn = 1.96 mm (1.77-2.56 mm, n = 204); length of the basal turn 21.87 mm (21.03-22.5 mm, n = 384). CONCLUSION: A notable size range exists across the eight different cochlear parameters considered and we provide normative values for each measurement. Females tend to have smaller CDL and "A" value than males and the sensorineural hearing loss patients had smaller CDL and "A" value but larger cochlear height than the general population.

Cochlear Implant After Sudden Onset Sensorineural Hearing Loss: A Case Report.

We present an uncommon case of a pediatric patient with sudden-onset sensorineural hearing loss (SSNHL), a medical condition in which a person experiences a rapid loss of 30 or more decibels within a matter of hours or days. The patient is a nine-year-old female who, two years prior, suddenly lost hearing in her left ear after a 24-hour episode of nausea, vomiting, and left ear pain. She presented to our clinic two years after the episode, long after the window for evidence-based treatment for acute SSNHL, such as corticosteroid therapy or antivirals, had passed. However, she remembered the moment of her hearing loss vividly, an uncommon occurrence in pediatric patients. CT, MRI, family history, and physical exam were unremarkable. The patient had a brief hearing aid trial where she described being able to hear the sound but did not have any clarity in understanding the sound. The patient was ultimately treated with a unilateral cochlear implant and showed excellent subjective and audiogram responses. Continued research on the management of SSNHL in pediatric patients who present outside of the acute therapeutic window is needed.

An Unusual Presentation and Diagnosis of a Type 4 Middle Ear Paraganglioma: A Case Report.

We discuss the unusual presentation and subsequent diagnosis of a patient with a glomus tympanicum tumor, also known as middle ear paraganglioma, which is a rare and benign tumor of the middle ear that usually presents with pulsatile tinnitus, cranial nerve pathology, hearing loss, and dizziness. The patient was a 35-year-old female with no past medical or surgical history who presented with a three-year history of mild left-ear hearing loss. The initial examination was negative for otalgia or otorrhea but was notable for a mass filling her left ear canal. The patient denied pulsatile tinnitus or dizziness. CT imaging was used to examine the soft tissue mass in the middle ear and ear canal and was consistent with a soft tissue heterogeneous mass with a subsequent biopsy confirming a diagnosis of paraganglioma. After the diagnosis, a laser surgical excision was scheduled to remove the tumor. Constant awareness is needed to examine the different presentations of middle ear paragangliomas so that appropriate treatment is promptly provided.

Complications of Cochleostomy Versus Round Window Surgical Approaches: A Systematic Review and Meta-Analysis.

We conducted a systematic review and meta-analysis to compare round window (RW) and cochleostomy (C) surgical approaches for the placement of cochlear implants (CIs). After obtaining the Institutional Review Board (IRB) approval, 213 peer-reviewed articles published between January 1, 2000, and August 1, 2021, comparing RW and C approaches were identified via a search on Google Scholar, Cochrane, and PubMed. The inclusion criteria were articles having an English version and involving only human subjects (cadaveric or alive). Statistical analysis of compiled electrode-to-modiolus distances was performed with two-sample independent t-tests. Live patients were categorized as having complete hearing preservation (<10 dB threshold shift), partial hearing preservation (10-20 dB shift), or minimal hearing preservation (>20 dB shift). Chi-squared testing was used to compare the distribution of hearing preservation categories between surgical approaches. Due to the heterogeneous nature of the data, only summative information was provided on the effects of approaches on trauma, electrical impedance, speech perception, vestibular dysfunction, ease of scala tympani insertion, and scalar shift. A total of 3,797 CI patients were evaluated. The RW approach resulted in a smaller (0.15 mm smaller on average, p<0.05) electrode-to-modiolus distance when compared to the C approach. The RW approach (93.0%) led to statistically better hearing preservation than the C approach (84.3%) (p<0.05). The RW approach was also associated with better outcomes in terms of speech perception, ease of scala tympani insertion, and reduced scalar shift. No difference between approaches was found with regard to trauma, electrical impedance, and vestibular dysfunction. Based on our findings, the RW approach appears to have several benefits compared to the C approach.

Pediatric Thyroid Nodules: Ultrasound Characteristics as Indicators of Malignancy.

Objective: Pediatric thyroid nodules are uncommon but carry a 25% malignancy risk. Adult patients have well-established ultrasound characteristics that are predictive of malignancy, but these are not clearly defined in pediatric patients. We reviewed a case series of pediatric thyroid nodules. Study Design: Retrospective chart review. Setting: Tertiary children's hospital. Method: Institutional review board approval was obtained. This case series with chart review includes all pediatric thyroid nodules with ultrasounds from 2006 to 2016 at 2 pediatric tertiary care centers. Results: An overall 112 pediatric thyroid nodules were analyzed. The mean patient age was 14.3 years; there was a female:male ratio of 4:1; and 94% were Caucasian. Seventeen percent (20/112) of nodules were malignant. In patients with malignant nodules, the average presenting age was 15.5 years, with a female:male ratio of 5.6:1. Seventy percent of malignant nodules had accompanying microcalcifications, 55% had abnormal lymph nodes, and 45% had irregular margins. In the benign nodules, 11% had microcalcifications, 12% had abnormal lymph nodes, and 26% had irregular margins. The presence of microcalcifications (odds ratio, 19.1 [95% CI, 6.0-61.0]; P < .0001), abnormal lymph nodes (odds ratio, 9.0 [95% CI, 3.0-26.6]; P = .0001), and size >3.5 cm (odds ratio, 5.8 [95% CI, 1.5-22.5]; P = .01) was associated with thyroid cancer. Irregular margins were not statistically significant (odds ratio, 2.3 [95% CI, 0.86-6.3]; P = .9). Conclusions: Our data suggest that abnormal lymph nodes, microcalcifications, and size >3.5 cm could be predictors of malignancy in the pediatric population and influence clinical decision making.

Surveillance, Epidemiology, and End Results Database update for pediatric thyroid carcinomas incidence and survival trends 2000-2016.

OBJECTIVE: Review the trends in pediatric thyroid carcinomas using the Surveillance, Epidemiology, and End Results (SEER) Database. METHODS: Institutional review board approval was obtained from Mercy-Bon Secours. The National Cancer Institute's SEER database was used for all cases of pediatric thyroid cancer between the years 2000 and 2016 for patients aged 0-19. Patients were grouped by carcinoma histological subtype, disease specific survival based on treatment modality, and demographic data. Treatment methods were compared using Fifteen-Year Disease Specific Survival Curves. RESULTS: 1175 pediatric patients were identified. The average age-adjusted rate of malignancy was 0.3 per 100,000 patients. Incidence of pediatric thyroid cancer was approximately 1:3.6, male to female. The papillary follicular variant histological subtype was the most common (n = 689, 58.6%), followed by papillary (n = 223, 18.9%), follicular (n = 153, 13.1%), and medullary (n = 110, 9.4%). Overall incidence of thyroid carcinomas increased with age, highest in patients aged 15-19 (69.8%). Incidence of medullary thyroid carcinomas was highest in patients aged 0-9. Patients aged 10-19 treated with surgery alone had the highest disease specific survival fifteen-years past initial diagnoses and treatment in all histologic subtypes (p < 0.05). Patients with metastatic medullary thyroid carcinoma at initial diagnosis who underwent surgery alone showed significantly poorer fifteen-year disease specific survival when compared to other histologic subtypes (p < 0.05). CONCLUSION: There was improved prognosis in pediatric thyroid carcinomas if diagnosed and treated early. All four major histological subtypes exhibit an increase in overall survival rates, (excluding medullary carcinomas).

Unusual Thyroid Mass in an Adolescent Patient.

Hurthle cell tumors are rare follicular-derived thyroid neoplasms. Hurthle cell tumors may be benign or malignant. Workup includes imaging, fine needle aspiration, and treatment usually consists of observation versus thyroidectomy. We describe a case of Hurthle cell adenoma in an adolescent; to the best of our knowledge, this represents only the third case described in the English literature of adolescent Hurthle cell adenoma.

Hearing Loss's Incidence and Impact on Employment in the United States.

OBJECTIVE: To determine the incidence of hearing loss (HL) as well as differences in wages and labor force participation rates between individuals with and without HL. STUDY DESIGN: Retrospective ecological study. PATIENTS: 1% of the US population including individuals with and without HL from the public use micro data sample (PUMS) of the 2011 to 2016 American Community Survey (ACS) was analyzed. MAIN OUTCOME MEASURE: ACS census data on individual age, self-reported hearing loss, labor force participation, and monetary earnings between 2011 and 2016. RESULTS: HL incidence rates were 13.4, 0.4, 3.8, 18.1, and 117.1 per 10,000 people among 0 to 2, 3 to 17, 18 to 44, 45 to 64, and 65+ years old, respectively. HL 18 to 44, 45 to 64, and 65+ years old participated in the labor force at 86, 81, and 61% of the rate of hearing individuals. HL 18 to 44, 45 to 64, and 65+ years old earned 78, 73, and 72% of the wages earned by non-HL individuals. CONCLUSIONS: Calculated HL incidence and labor force participation rates were higher than previously published in literature analyzing 1991 census data. The changes may be due to the methodology used in this study but may also reflect improvements in diagnosis, access to technology, and the implementation of the Americans with Disabilities Act (ADA) of 1990.

Clinical Guidelines in Pediatric Hearing Loss: Systemic Review Using the Appraisal of Guidelines for Research and Evaluation II Instrument.

OBJECTIVES: Despite the importance, impact, and prevalence of pediatric hearing loss (HL), there are very few published clinical practice guidelines (CPG) supporting the evaluation and management of pediatric patients with HL. Our objective was to appraise existing CPGs to ensure safe and effective practices. METHODS: A literature search was conducted in PubMed, Google Scholar, EBSCO, as well as a manual Google search. Three independent assessors using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) instrument evaluated CPGs related to HL in children. Standardized domain scores were calculated for each guideline. RESULTS: A total of four guidelines met the inclusion criteria and were appraised. Scope and purpose achieved a high median score of 83%. Stakeholder involvement, clarity of presentation, and editorial independence achieved intermediate scores of 67%, 54%, and 50%, respectively. The areas that required most improvement and achieved low scores were rigor of development and applicability, with scores of 22% and 38%, respectively. Based on the AGREE II measures, the four guidelines had domain scores less than 60% for each domain, and without modification no guideline could be recommended. CONCLUSIONS: Based on the AGREE II, the qualities of CPGs for pediatric HL have several shortcomings, and the need for a comprehensive CPG remains. Rigor of development and applicability present the greatest opportunities for improvement of these CPGs. Laryngoscope, 130:212-216, 2020.

Systematic Review of Pathogenic GJB2 Variants in the Latino Population.

OBJECTIVES: Define the extent to which GJB2-related hearing loss is responsible for non-syndromic hearing loss (NSHL) in the Latino population. METHODS: Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed. PubMed and MEDLINE were accessed from 1966 to 2019 using permutations of the MeSH terms: "Hearing Loss," "Hearing Impairment," "Deafness," "Latin American," "Latino," "GJB2," and "Genetic." Additionally, countries designated as Latino by the US Office of Management and Bureau were cross-referenced as key terms against the aforementioned search criteria. Exclusion criteria included non-English publications, a non-Latino study population, and literature not investigating GJB2. An allele frequency analysis of pathogenic GJB2 variants in the Latino population was performed and stratified by country of origin and reported ethnicity. RESULTS: One hundred twenty two unique studies were identified of which 64 met our inclusion criteria. Forty three studies were included in the GJB2 systematic review. A total of 38 pathogenic GJB2 variants were identified across 20 countries in the Latino population. The prevalence of pathogenic GJB2 variants varied by country; however, were generally uncommon with the exception of c.35delG (p.Gly12Valfs*) which displayed an allele frequency of 3.1% in the combined Latino population; ranging from 21% in Colombia to 0% in Guatemala. CONCLUSION: Variation in the prevalence of pathogenic GJB2 variants by country likely reflect the heterogeneous nature of ethnic ancestral contributions to the Latino population. Additional research utilizing next generation sequencing might aid in the development of assays for high throughput diagnosis of inherited hearing loss in the multitude of ethnic sub-groups that comprise this and other traditionally marginalized populations.

Systematic Review of Hearing Loss Genes in the African American Population.

OBJECTIVE: Literature review of the genetic etiology of hearing loss (HL) in the African American (AA) population. DATA SOURCES: PubMed, EBSCO, and CINAHL were accessed from 1966 to 2018. REVIEW METHODS: PRISMA guidelines were followed. Search terms included permutations of "hearing loss," "African American," "black," and "genetic"; "African American" was then cross-referenced against documented HL genes. AA subjects included in multiethnic cohorts of genetic HL testing were identified by searching the key terms "hearing loss" and "ethnic cohort" and "genetic." The Q-Genie tool was used in the quality assessment of included studies. An allele frequency meta-analysis of pathogenic GJB2 variants in the AA population was performed and stratified by hearing status. RESULTS: Four hundred seventeen articles were reviewed, and 26 met our inclusion criteria. Ten studies were included in the GJB2 meta-analysis. In the general AA population, pathogenic GJB2 variants are rare, including the 35delG allele, which displayed a carrier frequency of 0.05%. Pathogenic variants were discovered in seven nonsyndromic HL genes (GJB2, MYO3A, TECTA, STRC, OTOF, MYH14, TMC1), eight syndromic HL genes, and one mitochondrial HL gene. Recent comprehensive genetic testing using custom genetic HL testing platforms has yielded only a 26% molecular diagnosis rate for HL etiologies in the AA population. CONCLUSIONS: Investigators should be encouraged to provide an ethnic breakdown of results. Sparse literature and poor diagnosis rates indicate that genes involved in HL in the AA population have yet to be identified. Future explorative investigations using next-generation sequencing technologies, such as whole-exome sequencing, into the AA population are warranted.

Genetic susceptibility to aminoglycoside ototoxicity.

INTRODUCTION: Aminoglycosides are a well-known clinically relevant antibiotic family used to treat bacterial infections in humans and animals and can produce toxic side effects. Aminoglycoside-induced hearing loss (HL) has been shown to have a genetic susceptibility. Mitochondrial DNA mutations have been implicated in inherited and acquired hearing impairment. OBJECTIVE: Literature review of genetic mutations associated with aminoglycoside-induced ototoxicity. METHODS: PubMed was accessed from 1993 to 2017 using the search terms "aminoglycoside, genetic, ototoxicity, hearing loss". Exclusion criteria consisted of a literature in a language other than English, uncompleted or ongoing studies, literature with non-hearing related diseases, literature on ototoxicity due to cisplatin/carboplatin based chemotherapy, literature on ototoxicity from loop diuretics, animal studies, literature studying oto-protective agents, and literature without documented aminoglycoside exposure. RESULTS: 108 articles were originally identified, and 25 articles were included in our review. Mitochondrial 12S rRNA mutations were identified in all 25 studies in a total of 220 patients. Eight studies identified A1555G mutation as primary genetic factor underlying HL in cases of aminoglycoside-induced ototoxicity. The next most common mutation identified was C1494T. DISCUSSION: Mitochondrial 12s rRNA mutation A1555G was present in American, Chinese, Arab-Israeli, Spanish and Mongolian ethnicities. All mutations leading to aminoglycoside ototoxicity were mitochondrial mutations. CONCLUSIONS: Consideration of preexisting genetic defects may be valuable in treatments involving aminoglycosides. In particular populations such as those of Chinese origin, clinicians should continue to consider the increased susceptibility to aminoglycosides.

Cochlear implant provider awareness of vaccination guidelines.

OBJECTIVE: Guidelines for vaccination of cochlear implant (CI) recipients have been promulgated and updated by the Center for Disease Control and Prevention (CDC) to mitigate bacterial meningitis risk. The objective of this study was to survey current CI specialists on: 1) knowledge of current CDC CI recipient immunization recommendations, and 2) impediments to implementation of those guidelines. METHODS: A survey to assess knowledge of the CI recipient vaccination guidelines was administered to the American Academy of Otolaryngology-Head and Neck surgery (AAO-HNS), the American Cochlear Implant Alliance (ACIA), and the American Neurotology Society (ANS). The members of the AAO-HNS and ACIA were invited to participate in the survey via an e-mail. The members of the ANS were polled during a session of their fall meeting. RESULTS: A total of 256 individuals participated: 64 from AAO, 59 from ACIA, and 133 from ANS. Participants reported knowledge of the vaccination guidelines as high among all groups and statistically similar. The survey indicated that the participants noted difficulty in obtaining age-group-specific immunizations. Vaccination status was not consistently reported. CONCLUSION: Cochlear implant providers have high awareness of vaccination guidelines but less detailed knowledge of age-specific recommendations. Obtaining age-specific vaccines is a challenge, as is consistent documentation of vaccination. Future efforts should be focused on improving knowledge of age-specific recommendations, easing access of age-specific vaccines, and improving documentation. LEVEL OF EVIDENCE: NA. Laryngoscope, 128:2145-2152, 2018.

Otitis Media with Effusion and Helicobacter pylori.

Previous studies reported the presence of Helicobacter pylori in middle ear fluid and raised a possible causal role of this bacterium in otitis media with effusion (OME). We investigated 48 children with OME (age, 7 months to 12 years) seen in New Orleans, Louisiana, and found no evidence of H pylori presence in any middle ear fluid specimen by amplification of the 16S rRNA gene. One child had H pylori detected in a stool specimen, in accordance with the low prevalence in our population. While H pylori may be significant in other countries, these findings do not support a role for H pylori in OME in our pediatric population in the United States.

Tracheostomy for Severe Pediatric Obstructive Sleep Apnea: Indications and Outcomes.

Objectives (1) To describe characteristics of pediatric patients undergoing tracheostomy for obstructive sleep apnea (OSA). (2) To highlight perioperative events and outcomes of the procedure. Study Design Case series with chart review. Setting Four tertiary care academic children's hospitals. Subjects and Methods Twenty-nine children aged <18 years from January 1, 2010, to December 31, 2015, who underwent tracheostomy for severe OSA, defined as an apnea-hypopnea index (AHI) >10, were included in the study. Data on patient characteristics, polysomnographic findings, comorbidities, and perioperative events and outcomes were collected and analyzed. Results Twenty-nine patients were included. Mean age at tracheostomy was 2.0 years (95% CI, -2.2 to 6.2). Mean body mass index z score was -1.2 (95% CI, -4.9 to -2.5). Mean preoperative AHI was 60.2 (95% CI, -15.7 to 136.1). Mean postoperative intensive care unit stay was 23.2 days (95% CI, 1.44-45.0). One procedure was complicated by bronchospasm. Thirteen patients had craniofacial abnormalities; 10 had a neurologic disorder resulting in hypotonia; and 5 had a diagnosis of laryngomalacia. Mean follow-up was 30.6 months (95% CI, -10.4 to 71.6). Six patients were decannulated, with a mean time to decannulation of 40.8 months (95% CI, 7.9-73.7). Five patients underwent capped sleep study prior to decannulation with a mean AHI of 6.6 (95% CI, -9.9 to 23.1) and a mean oxygen nadir of 90.0% (95% CI, 80%-100%). Conclusion OSA is an uncommon indication for tracheostomy in children. Patients who require the procedure usually have an associated syndromic diagnosis resulting in upper airway obstruction. The majority of children who undergo tracheostomy for OSA will remain dependent at 24 months.

Pediatric thyroid nodules: A single center experience.

OBJECTIVES: 1. Analyze our center's experience with thyroid nodules. 2. Evaluate the efficacy of fine needle biopsy. 3. Compare our experience with the 2015 ATA guidelines. METHODS: IRB approved retrospective chart review from July 1993-July 2014 at a tertiary pediatric institution. Patients under age 21 with documented thyroid nodules who underwent fine needle aspiration, and/or thyroidectomy were included. RESULTS: 126 patients were identified. 84.1% (n = 106) were female. Age range was 12 months-20 years. The average age was 13.3 ± 4.1 years. The nodules ranged from 0.5 cm to 6 cm 53.9% (n = 68) had a fine needle biopsy done. 42.6% (n = 29) fine needle biopsies were benign, 26.5% (n = 18) were non-diagnostic, 13.2% (n = 9) were classified as "atypia", 0.09% (n = 6) were consistent with thyroiditis, and 0.09% (n = 6) were suspicious for papillary carcinoma. 78.6% (n = 99) underwent surgery. 7 patients with "benign" needle biopsies underwent surgery: all had follicular adenoma. 7 patients with "atypia" needle biopsies underwent surgery: one patient had papillary thyroid cancer. 8 patients with non-diagnostic needle biopsies underwent surgery: one patient had papillary thyroid cancer. All the patients with needle biopsy suspicious for papillary carcinoma had papillary carcinoma on final pathology. 99 patients underwent surgery: 14.1% (n = 14) had papillary carcinoma and 2% (n = 2) had medullary carcinoma. CONCLUSION: Our review of pediatric thyroid nodules at our institution supports data previously reported. 84% of identified patients were female, supporting previous data that nodules are more prevalent in the female population. Of the 126 patients identified, 12.7% had thyroid carcinoma, supporting the 7-25% range described in previous literature. Our institution showed a high positive predictive value of FNA for papillary thyroid carcinoma.