FCOM 2022 Poster Submission

Sjogren syndrome (SS) is an autoimmune inflammatory condition which often affects female patients between 45 and 55 years of age and presents with sicca symptoms.1 However, SS can rarely occur in pediatric populations, namely Juvenile SS (JSS), which often presents recurrent parotitis, positive serology, neurological and renal manifestations.2 Salivary gland ultrasonography (SGUS) with a simplified score assessment of parenchyma dyshomogeneity is a highly specific test for primary SS.3 In this case report, we present the case of a 12 year-old female who was diagnosed with JSS based on the 2016 ACR/EULAR SS diagnostic criteria for adults and monitored for progress via SGUS throughout treatment involving sialendoscopy.

The patient with a history of type I diabetes mellitus presented with recurrent, tender swelling of her parotid glands refractory to antibiotic treatment, xerostomia, and keratoconjunctivitis sicca. Her lab work was negative for SSA and SSB antibodies but showed early markers for JSS with positive salivary protein 1 IgA autoantibody. A labial biopsy was then performed to reveal lymphocytic sialadenitis. On the basis of clinical symptoms, serologic markers, and positive lip biopsy, she was diagnosed with JSS. Pharmacologic treatment included 200mg Plaqenil to manage symptoms. She underwent bilateral parotid duct and gland sialendoscopy with 100mg Kenalog irrigation to each gland. Her SGUS score before sialendoscopy was 16, graded based on the Hocevar’s scoring system, with ultrasound showing multiple hypoechoic lesions.4 In the months following her sialendoscopy, her score reduced to the 10-11 range with resultant homogeneous findings on SGUS, indicating significant improvement of glandular inflammation. Eleven months post-sialendoscopy, she had a recurrent swelling of the right gland which resulted in increasing daily Plaqenil dose from 200mg to 300mg. Twelve months after her sialendoscopy, her SGUS score remained at 11.

Diagnosis of JSS is challenging and must draw from multiple elements including clinical presentation, serology for autoantibodies or cytopenia, and imaging. Since patients’ clinical phenotype can be heterogeneous, patients with negative SSA/SSB are still recommended to undergo a labial biopsy to rule out JSS.5 According to Theander and Mandl, SGUS is an effective method for diagnostic and prognostic purposes in diagnosing SS, as it has a positive predictive value and specificity of 98%.3 Additionally, the imaging can offer clinicians a visual representation of progress over time with treatment. While SGUS monitoring has its benefits, notable drawbacks include missing early disease if disease severity is mild, and US monitoring must be executed with the same technique, standardized scoring system, same machine, and technician if a long term follow-up is planned.

Sialendoscopy is a minimally invasive and effective tool for the diagnosis and treatment of glandular ductal pathology, including inflammation, stenosis, or stricture. In a review of juvenile recurrent parotitis, Schwarz et al note that it is unclear whether the steroid treatment or the procedure itself effectively treats symptoms.6 With that said, according to a study by Schiffer et al, patients with JSS can be diagnosed based upon sialendoscopy and histopathology from labial biopsy, and Faure et al note that sialendoscopy has a greater sensitivity than radiologic ultrasound or MRI.7 8 However, it should be noted that the findings of the sialendoscopy alone were not specific for diagnosis of JSS and other clinical indicators like labial biopsy or antibody titers were necessary for JSS diagnosis. For JSS, no reports to date adequately estimate the effectiveness of sialendoscopy in halting recurrence.

In pediatric populations, JSS will present more commonly with parotid involvement with punctate sialectasis in comparison to adults who can also have submandibular involvement and fatty degeneration.9 With this in mind, SGUS scoring may need to be modified to better suit the clinical distinctions of SS in the pediatric population. Whether sialendoscopy alone can provide a long-term remission of recurrent parotitis in patients with JSS warrants further investigation.

Introduction
Approximately 13% of adults 18 and older had some difficulty hearing even when using a hearing aid in the United States.1 Hearing difficulties increase with age and up to 26.8% of adults 65 and older have some difficulty hearing.1 Hearing loss is associated with a variety of poor health outcomes such as dementia, depression, increased risk of falls, poor physical health, cognitive decline, and increased healthcare costs.2 Because of the large portion of aging adults that are effected by hearing loss, it is a major public health and social concern. To our knowledge, there is limited data that explores the demographic and socioeconomic status of counties that may be disproportionately affected with hearing loss.

Objectives
To compare hearing loss rates in adults in the 67 Florida counties and socioeconomic status of each county.

Methods
Hearing loss rates were collected using Florida Health Charts from 2020. Demographic and socioeconomic data was collected using County Health Rankings and Roadmaps for each Florida county from 2018-2021, depending on the variable. Linear regression was used with a p-value of less than 0.05 set as significant.

Results
The mean percentage of hearing loss for each county for individuals aged 18-64 is 2.4% (95% CI 2.2-2.6) and those older than 64 is 15.2 (95% CI 14.2-16.1). Counties with lower rates of Hispanics and Asians had less rates of adult hearing loss while counties with higher rates of non-Hispanic whites had the highest rates of hearing loss (p-value

Objectives: Clinically, there is a lack of routine speech-in-noise testing due to a variety of barriers, including clinician perceived time constraints, uncertainty in which test material is most appropriate for a specific patient, and lack of materials which clearly quantify degrees of speech-in-noise impairment. The speech-in-noise tests available at present use a variety of target signals, such as digits, sentences, or monosyllabic words. Linguistic cues of speech, such as semantics, syntactics, word familiarity and word frequency, influence speech-in-noise performance; and, thus, may add to the clinician confusion about performance interpretation. Our study aimed to evaluate the performance between three speech-in-noise tests with varying linguistic cues currently available for clinical implementation: the Digits in Noise test (DIN), the Words in Noise test (WIN), and the American English Matrix Test (AEMT). To our knowledge, the AEMT, that is available in 15 languages, has yet to be compared to other clinically available speech-in-noise tests. We hypothesized that performance across tests would be correlated for both listeners with normal hearing and listeners with hearing loss. Understanding correlational relationships on these tests will both allow for standardization of degrees of speech-in-noise hearing loss and determine if these tests can be used interchangeably in clinic.

Design: Participants were 27 listeners with normal hearing and 32 listeners with sensorineural hearing loss. All participants were native English speakers. The outcome of interest was performance on the DIN (most linguistic cues), the AEMT (some linguistic cues), and the WIN (least amount of linguistic cues). The DIN and the WIN were presented in the presence of multi-talker babble and used a descending paradigm to derive the 50% correct recognition point using the Spearman-Karber Equation. The AEMT was presented in an open set in the presence of steady-state speech-spectrum noise and used an automated adaptive procedure to converge to the 50% correct recognition point.

Results: Speech-in-noise performance, reported as the signal to noise ratio (SNR) at which 50% correct word recognition is achieved (SNR-50), for the normal hearing listeners was the following: DIN: -12.6 dB SNR (SD = 1.94), AEMT: -7.8 dB SNR (SD = 4.22), and WIN: 4.8 dB SNR (SD = 1.65). Performance for the listeners with hearing loss was: DIN: -4.9 dB SNR (SD = 2.68), AEMT: -1.5 dB SNR (SD = 4.20), and WIN: 16.6 dB SNR (SD = 5.12). For both groups, performance increased with increasing linguistic cues. Strong positive relationships were found when assessing the materials using a Pearson product moment correlation with r values of: DIN/WIN: r= 0.849*, AEMT/WIN: r= 0.848*, and AEMT/DIN: r= 0.729* (*Indicates a p-value

Background: Reconstruction of large laryngotracheal defects remains a surgical challenge. Rebuilding the airway with rigid, vascularized tissue is optimal to restore function and prevent stenosis. We describe a two-staged reconstruction method for a large laryngotracheal defect using a pre-laminated free flap.

Methods: A 58 year-old male with a history of prolonged intubation and complete tracheal stenosis just below the vocal cords secondary to cricotracheal separation, presented to our clinic with the hope of regaining his natural voice. The first stage of reconstruction consisted of harvesting rib cartilage and implantation of the graft into the right anterolateral thigh. Approximately 2 months later, the patient underwent cricotracheal resection and right sided anterolateral thigh free flap with placement of the pre-laminated rib cartilage into the cricotracheal defect. A t-tube was placed as a stent inferior to the vocal cords to facilitate rigid structure and patency.

Results: Approximately 6 weeks following reconstruction, the t-tube stent was removed, and the patient’s tracheostomy tube was replaced with a t-tube. The patient was immediately able to phonate.

Conclusion: Pre-laminated free flaps using rib cartilage are an effective option for functional restoration of cricotracheal defects. This two-stage approach allows for complex tracheal defects to be replaced with vascular and rigid tissue that withstands airway pressure and prevents recurrent stenosis.

Background: There is a growing need to develop prophylactic and therapeutic interventions to prevent loss of residual hearing post-cochlear implantation. During cochlear implantation, the initiation of the electrode insertion trauma (EIT) triggers the activation of oxidative stress, apoptosis and inflammatory pathways that can damage sensory cells and consequently lead to the loss of residual hearing. Preserving these sensory cells by blocking the activation of these host pathways can improve hearing preservation and allows implanted individuals to benefit from better hearing outcomes. The aim of this study was to investigate the effect of Taurodeoxycholic acid (TDCA) on the preservation of the residual hearing in a preclinical animal model of cochlear implantation.

Method: Animals were divided into various groups. In first group, animals were implanted unilaterally. In second group, TDCA was applied on the round window membrane before cochlear implantation followed by insertion of the electrode. The animals in third group served as vehicle control whereas naïve animals that were not subjected to treatment with TDCA and cochlear implantation served as the control group. Contralateral ear from each group also served as the control group. Hearing thresholds of animals in each group were determined by auditory brainstem recordings (ABRs). Cochleae harvested from animals in each group was subjected to histopathological examination to determine pathological manifestations. The organ of Corti was dissected and stained with FITC phalloidin to visualize and count the number of hair cells.

Results: Hearing thresholds were significantly lower after TDCA application than in the EIT group. The organ of Corti harvested from cochlea of implanted and TDCA treated animals subject showed a significantly higher number of hair cells after immunostaining compared to implanted animal alone. The molecular mechanisms behind otoprotection involved abrogation of activation of oxidative stress and caspase pathways.

Conclusion: The result of the present study suggests that TDCA provides otoprotection against cochlear implant trauma and can be explored for developing effective interventions. The availability of new interventions to prevent electrode insertion trauma holds great potential to promote hearing preservation and expanding indications of cochlear implantation.

Background: Gaps embedded in noise (GIN) have been used as acoustic stimulus to evaluate the auditory temporal processing of different study populations such as in subjects with central auditory processing disorders (CAPD). Since GIN are very short as miliseconds (ms), they have been used to evaluate temporal resolution which is defined as the ability to detect small changes in sound over time, or slight discontinuities in ongoing stimuli. Poor temporal resolution has been shown to correlate with speech recognition difficulties. The gap detection threshold (GDT) is defined as the shortest gap that can be perceived in an otherwise continuous background stimulus. Electrophysiological assessment of GIN has focused mainly on Late Latency Responses requiring awake alert subjects (humans or animals). As rat models are commonly employed to understand the molecular mechanisms underlying auditory and neurological disorders, there is a need to develop techniques to determine central auditory processing (CAP) in experimental animal models.

Methods: We developed a method to determine the Objective Gap Detection Threshold (OGDT) in rats. QSSR elicited by noise modulated by 40Hz gaps of different durations were analyzed in time and frequency domains using wild-type (WT) and rat model of autism. The detection was performed in frequency domain, by applying the Hotelling’s T2 test to the 40Hz complex fundamental frequency component. The OGDT is estimated by analyzing the confidence ellipses of the 40Hz spectral component.

Results: When the confidence ellipses (p=0.05) contain the origin of the complex plane, we observed that the subject is not significantly detecting the noise gap. We observed that the rats were detecting noise gaps of 12, 10 and 8ms and not detecting the 6ms gap. We also observed the “vanishing” of response into the background noise when a 4ms noise gap is applied. The 5ms OGDT result was verified by the statistical T2 test and is consistent with the previous animal studies with awake rats. The OGDT values were significantly increased in our rat model of autism in agreement with findings observed in humans having neurological disorders.

Conclusions: We have developed an electrophysiological method to determine GDT as a measure of CAPD in an anesthetized rat model. This technique eliminates the need for being alert and awake during the test bringing a new dimension especially in animal experiments regarding central auditory evaluation. The availability of novel objective techniques to determine CAPD will help in its early detection in affected individuals leading to early intervention and hence better clinical outcomes.

Background: Olfactory disturbances including anosmia and parosmia are unique features of SARS-CoV-2 infection with high positive predictive value. Change in olfaction appears to be the consequence of multiple parallel processes including conductive and obstructive anosmia, destruction of nasal cilia and olfactory sensory neurons, and retrograde destruction of higher-order olfactory neural processes. The global pooled estimate for olfactory disturbance among patients with a positive RT-PCR is approximately 40%. To date, there has been no investigation of regional differences in the prevalence of olfactory disturbance in SARS-CoV-2 infection, and no analysis of this sequelae in the southern US.

Methods: A retrospective cohort analysis was performed using the Informatics for Integrating Biology and the Bedside data tool at University of Florida Shands Hospital (i2b2). Outcomes of interest were “SARS coronavirus 2 RNA” and “R43.0 anosmia” OR “R43.1 parosmia”. Covariates were gender (female, male, not recorded, unknown), race (American Indian, Black Hispanic, Black or African American, Hispanic, Indian, Native Hawaiian, White, White Hispanic, Multiracial, and other), and age (0-9, 10-17, 18-34, 35-44, 45-54, 55-64, 65-74, 75-84, 85+).

Results: 8572 patients with COVID-19 were identified, of which 229 (2.8%) had olfactory disturbance. Among patients with olfactory disturbance, 157 (68.9%) were female and the mean age was 31.7 years (95% CI: 17.8- 45.6). Less than 3 patients were American Indian, Black Hispanic, Hispanic, Indian, Native Hawaiian, White Hispanic, or Multiracial. 62 (27.1%) were Black or African American, 141 (61.6%) were White, and 16 (7.0%) were other. Less than 3 patients were 0-9 years, 12 (5.2%) were 10-17 years, 119 (52%) were 18-34 years, 31 (13.5%) were 35-44 years, 26 (11.4%) were 45-54 years, 18 (7.9%) were 55-64 years, 11 (4.8%) were 65-74 years, 5 (2.2%) were 75-84 years, and less than 3 were 85+ years. Prevalence of anosmia and parosmia was more than double in female patients compared to male patients. R² for age group vs. olfactory disturbance diagnosis was .2382.

Conclusion: In this study we found the prevalence of anosmia and parosmia among patients with a positive RT-PCR at University of Florida Shands was much lower than global pooled estimates. Olfactory disturbance in this study reflected age-dependent anosmia, with patients 75-84 years reflecting the lowest prevalence. Given these findings we conclude that while anosmia is regarded as an early marker of COVID-19 infection, it may not be as reliable of a predictor in elderly populations in the southern United States.

Background. Sialorrhea, also known as excessive salivation, is considered abnormal beyond four years of age. Sialorrhea severity is best measured using a combination of quantitative and qualitative metrics to guide clinical decision making. Severe cases warrant intervention to improve patient quality of life and reduce caretaker burden.

Objective. The primary aim of this study is to review the five major forms of sialorrhea management ranging from least-to-most invasive: oral-motor therapy, anticholinergic medications, botulinum neurotoxin, sialendoscopy sclerotherapy, and surgical intervention.

Data Sources and Review Methods. An electronic literature review identified articles through a comprehensive search of sialorrhea management in pediatric patients in Cambridge Scientific Abstracts, EMBASE, PubMed, and Web of Science.

Conclusions and Implications for Practice. Sialorrhea management starts with more conservative approaches. Viable candidates begin with oral-motor therapy. Anticholinergic medications can decrease saliva production, but adverse side effects may outweigh benefits. Botulinum neurotoxin injection of the salivary glands decreases salivary flow rate; however, relief is transient and thus requires multiple treatments. Sialendoscopy sclerotherapy is the latest treatment option that shows promising results. Surgical intervention is reserved as a treatment option of last resort for patients who suffer from severe sialorrhea due to potential adverse consequences, which include airway swelling/obstruction, facial nerve paralysis, postoperative hemorrhage, and xerostomia with its downstream effects. In conclusion, this review found that the underlying cause and severity of sialorrhea dictate the approach to management.

Stricture formation is a serious complication following pharyngeal reconstruction. These strictures can be life-threatening and can severely impact quality of life. We have reviewed the existing literature on surgical risk factors linked to neopharyngeal stricture formation. Intraoperative preventative measures reconstructive surgeons should consider are also discussed. Finally, we will describe the evaluation and management of pharyngoesophageal strictures, including the challenges and options when dealing with refractory strictures.

Background:
Lingual tonsil hypertrophy (LTH) is known as a possible cause of persistent child obstructive sleep apnea after adenotonsillectomy, especially in patients with trisomy 21, but its role in chronic cough and exercise-induced shortness of breath in non-syndromic patients is less well-established. As such, lingual tonsillectomy is rarely performed. This study is a preliminary exploration of the role of lingual tonsils and subsequent lingual tonsillectomy in pediatric patients with chronic respiratory-related symptoms refractory to more traditional therapies.

Research question: In pediatric patients, does lingual tonsil hypertrophy result in upper airway obstruction and respiratory-related symptoms that can then be treated with lingual tonsillectomy?

Methods:
Ten children were referred by a pediatric pulmonologist with chronic upper airway symptoms (cough, throat-clearing, exercise-induced shortness of breath, and in some cases, a sensation of food sticking in the back of the throat when eating) unresponsive to traditional treatments, and for whom the only abnormal finding on flexible bronchoscopy was LTH. Endoscopic lingual tonsillectomy was subsequently performed, and the post-operative response was assessed.

Results:
The patients had chronic respiratory-related symptoms including for an average of 21.9 months. After surgery, the patients were subsequently followed for an average of 24.2 months. Nine of ten patients saw preoperative symptom resolution after surgery. One patient experienced symptom recurrence and LTH again after 1 year but has seen symptom resolution 14 months after surgical revision. One patient has been subsequently scheduled for palatine tonsillectomy due to symptom persistence 2 years later. There were no intraoperative complications and only one patient experienced mild and self-limited pharyngeal bleeding 8 days postoperatively.

Conclusion:
Lingual tonsillectomy proved to significantly resolve chronic respiratory-related symptoms when no other treatment had in this case series.

Discussion:
The resolution of respiratory-related symptoms after lingual tonsillectomy implicates LTH in difficult-to-treat symptoms, including chronic cough, throat-clearing, and exercise-induced shortness of breath. Other symptoms that were also frequently present were snoring, globus sensation, and a sensation of food sticking in the back of the throat when swallowing. Each patient was previously treated, unsuccessfully, for differential diagnoses such as laryngopharyngeal reflux, allergic rhinitis, vocal cord dysfunction and reactive airway disease. LTH is difficult to diagnose and may be overlooked, contributing to the commonplace chronicity of these respiratory symptoms. Though sample size is a limitation, this study suggests that the role of the lingual tonsils should not be overlooked in patients with these types of symptoms. Further studies with a larger sample size are required to further support the use of lingual tonsillectomy as treatment for these chronic upper airway symptoms.