Cochlear implantation in syndromic patients: difficulties and lessons learnt.

OBJECTIVE: Identify the prevalence of syndromes in a cohort of patients who underwent cochlear implantation, to report on the presence of inner and middle ear malformations and highlight the surgical difficulties encountered. STUDY DESIGN: Observational, retrospective study. SETTING: Tertiary referral children's hospital pediatric cochlear implant program. MATERIAL & METHODS: An IRB-approved retrospective chart review of children undergoing cochlear implantation at a tertiary academic medical center, from 2018 to 2023. Preoperative imaging data of syndromic patients in that cohort with special attention to the presence of inner ear or middle ear malformations were collected. Abnormal intraoperative findings and difficulties reported by the surgeons were also noted. RESULTS: 1024 children were unilaterally implanted for bilateral profound hearing loss. There were 45 cases diagnosed with associated syndromes (4.3%). The commonest syndromes were Jervell and Lange Nielsen (JLN) syndrome followed by Waardenberg syndrome (WS), in a prevalence of 34% and 32% respectively. These syndromes had no associated inner ear malformations (IEM). Less common syndromes included Branchio-oto-renal (BOR) syndrome, CHARGE association and Treacher Collins syndrome, 3 cases each, and keratosis icthyosis deafness syndrome (KID), Usher syndrome and Albino, 2 cases each and an H syndrome case. There were 9 cases (20%) with IEM, with 6 cases of perilymph gusher. Two cases had middle ear anomalies and one case had a facial nerve course abnormality. The outcome of these cases was similar to non-syndromic cases. CONCLUSION: Children with syndromic HL should be dealt with on a case by case scenario to diagnose inner and middle ear malformations. Additional disabilities can affect the rehabilitation procedures. All children with congenital hearing loss should undergo pediatric, cardiologic, ophthalmologic and nephrologic examination in order to exclude the syndromic etiology of hearing loss.

Evaluation of Gustatory Function in Patients with Chronic Rhinosinusitis.

Chronic rhinosinusitis (CRS) is a chronic inflammatory disease of the mucosa of the nasal cavity and nasal sinuses. Many patients with CRS complain of gustatory dysfunction which affects their quality of life. To assess the gustatory function and acuity in patients with chronic rhinosinusitis using taste strips. This is a case control study carried out at our institute from May 2021 to June 2022 on 63 Patients with chronic rhinosinusitis with nasal polyposis and 63 normal controls. All patients of the study and control group will be subjected to full medical history taking including SNOT-22-Questionnaire, sinuscopic examination, computed tomography scan nose and paranasal sinuses using Lund-Mackay score. Taste strips representing five tastes (sweet, sour, salty, bitter and umami) were used to assess gustatory function in CRS patients and controls. 63 patients with CRS and 63 healthy controls were involved in our study, age 42.05 ± 14 years old for cases and 40.9 ± 13.6 years old for controls. There was a highly significant difference between cases and controls as regard SNOT-22-Questionnaire scores and Meltzer-scores with higher mean scores among cases. There was a highly significant difference between cases and controls as regard Sweet, Salt Sour, Bitter, Umami and Total Taste score, with lower mean scores among cases. This is most evident in bitter taste. There was a significant negative Correlation between each of SNOT-22-Questionnaire score, Meltzer-score and Lund-Mackay score and total taste score. Patients with chronic rhinosinusitis exhibited decreased gustatory function compared to healthy controls. The effect was most pronounced for bitter taste.

The Value of Endoscopic Exposure of Round Window in Cochlear Implant via Facial Recess.

Introduction Cochlear implantation has been considered as the best treatment in patients with severe to profound hearing loss unaidable with hearing aids. The main value of endoscope-assisted cochlear implantation is improved visibility of the RW Objective to assess the value of endoscopic assisted CI surgery via facial recess approach without elevating tympanic anulus. Methods This Prospective case series study non-randomized sample was performed on 50 patients with severe to profound hearing loss unaidable with hearing aids undergoing unilateral endoscopic assisted cochlear implant surgery with round window electrode insertion Results There were 23 male and 27 female patients. Most of the cases were children (41 cases). Of those 50 patients, 39 were prelingually hearing impaired. Four cases had various inner ear abnormalities. The standard mastoidectomy and Posterior Tympanotomy approach were used for all cases. Endoscopic identification of the RW through the PT enabled us to perform regular surgery in all cases. The current study concludes the difference between microscopic exposure and endoscopic exposure represented by Saint Tomas classification found that endoscopic exposure of round window classification is better represented by downgrading in the classification of round window exposure as type I 29(58%), type IIa 18(36%) type IIb 3 (6%) Non were type III by endoscopic exposure compared to microscopic exposure of round window is a type I 7(14%), type II 14(28%), type IIb 22(44%) and type III 7 (14%). Conclusion Endoscopy proved a great value in exposure and identification of RW in CI surgery through posterior tympanotomy approach.

The Value of Endoscopic Exposure of Round Window in Cochlear Implant via Facial Recess

Abstract Introduction Cochlear implantation has been considered as the best treatment in patients with severe to profound hearing loss unaidable with hearing aids. The main value of endoscope-assisted cochlear implantation is improved visibility of the RW Objective to assess the value of endoscopic assisted CI surgery via facial recess approach without elevating tympanic anulus. Methods This Prospective case series study non-randomized sample was performed on 50 patients with severe to profound hearing loss unaidable with hearing aids undergoing unilateral endoscopic assisted cochlear implant surgery with round window electrode insertion Results There were 23 male and 27 female patients. Most of the cases were children (41 cases). Of those 50 patients, 39 were prelingually hearing impaired. Fourcases had various inner ear abnormalities. The standard mastoidectomy and Posterior Tympanotomy approach were used for all cases. Endoscopic identification of the RW through the PT enabled us to perform regular surgery in all cases. The current study concludes the difference between microscopic exposure and endoscopic exposure represented by Saint Tomas classification found that endoscopic exposure of round window classification is better represented by downgrading in the classification of round window exposure as type I 29(58%), type IIa 18(36%) type IIb 3 (6%) Non were type III by endoscopic exposure compared to microscopic exposure of round window is a type I 7 (14%), type II 14(28%), type IIb 22(44%) and type III 7 (14%). Conclusion Endoscopy proved a great value in exposure and identification of RW in CI surgery through posterior tympanotomy approach,

Comparison of the efficacy of cochlear implantation and stapes surgery in far advanced otosclerosis: a meta-analysis study.

OBJECTIVE: This study is to compare the hearing outcomes and complications of stapes surgery and cochlear implantation (CI) in patients with far-advanced otosclerosis (FAO). DATA SOURCES: A comprehensive electronic search of PubMed/MEDLINE, Scopus, Web of science and Cochrane Library was conducted in June 2021 for articles in the literature till this year. STUDY SELECTION: Studies are published in English language, conducted on human subjects, concerned with comparison of CI and stapes surgery in the management of FAO, not Laboratory study and not Opinion study. The current review followed the guidelines of preferred reporting items for systematic reviews and meta-analysis statement 2009 (PRISMA). DATA EXTRACTION: Twenty-six studies were included with 334 patients in CI group and 241 patients in stapes surgery group. Comparison between both groups was done in terms of postoperative complications, audiological outcomes, rete of revision surgery and patients' satisfaction rate. RESULTS: Postoperative complications rate was significantly lower in CI (13.6%) than stapes surgery (18.6%). CI had a significantly lower rate of revision surgery (8.1%) than stapes surgery (16.4%). CI had a better mean for pure tone average (29.1 dB) than stapedectomy (52.3 dB) while stapes surgery had a higher mean for recognition of monosyllables and disyllables than CI. CI had significantly higher satisfaction rate than stapes surgery. CONCLUSION: Both Stapes surgery and CI are reliable treatment options for FAO with close success rates. Statistics of CI are greater than stapes surgery and CI has a consistent improvement in audiometric outcomes in comparison to stapes surgery.

Swallowing Problems after Thyroidectomy

Abstract Introduction Thyroidectomy is a common procedure. Certain swallowing problems could happen after this surgery and affect the quality of life of the patient. Objective To evaluate swallowing after thyroidectomy in the early and late postoperative periods and to correlate subjective and objective parameters. Methods A prospective study with100 patients who underwent total thyroidectomy at our institution from April 2018 to September 2019. Each patient was assessed by the Arabic version of the Eating Assessment Tool (EAT-10) questionnaire and the fiberoptic endoscopic evaluation of swallowing (FEES) preoperatively, and in the early postoperative (EPO) and late postoperative (LPO) periods. Results The rate of dysphagia was of 82% in the EPO period, and of 36% in the LPO period. Two groups were compared regarding vocal fold mobility using the FEES. Group I included 89 patients with normal vocal fold mobility, 42% of whom had early dysphagia, and only 22% had late dysphagia. Regarding swallowing, we found that in the EPO period, the rates of delayed triggering, aspiration, penetration and residue were of 12.4%, 0%, 0%, and 42.7% respectively. Group II (unilateral immobile vocal fold) included 11 patients in the EPO evaluation, and all of them had early dysphagia. Conclusion Swallowing problems can occur in patients after thyroidectomy regardless of alterations in larynx mobility, and they are characterized by delayed triggering and stasis of food, which are also noticed in the LPO period, though more frequently in the EPO period. Moreover, there is a highly significant correlation between the subjective and objective parameters of swallowing in both EPO and LPO periods.

Swallowing Problems after Thyroidectomy.

Introduction Thyroidectomy is a common procedure. Certain swallowing problems could happen after this surgery and affect the quality of life of the patient. Objective To evaluate swallowing after thyroidectomy in the early and late postoperative periods and to correlate subjective and objective parameters. Methods A prospective study with100 patients who underwent total thyroidectomy at our institution from April 2018 to September 2019. Each patient was assessed by the Arabic version of the Eating Assessment Tool (EAT-10) questionnaire and the fiberoptic endoscopic evaluation of swallowing (FEES) preoperatively, and in the early postoperative (EPO) and late postoperative (LPO) periods. Results The rate of dysphagia was of 82% in the EPO period, and of 36% in the LPO period. Two groups were compared regarding vocal fold mobility using the FEES. Group I included 89 patients with normal vocal fold mobility, 42% of whom had early dysphagia, and only 22% had late dysphagia. Regarding swallowing, we found that in the EPO period, the rates of delayed triggering, aspiration, penetration and residue were of 12.4%, 0%, 0%, and 42.7% respectively. Group II (unilateral immobile vocal fold) included 11 patients in the EPO evaluation, and all of them had early dysphagia. Conclusion Swallowing problems can occur in patients after thyroidectomy regardless of alterations in larynx mobility, and they are characterized by delayed triggering and stasis of food, which are also noticed in the LPO period, though more frequently in the EPO period. Moreover, there is a highly significant correlation between the subjective and objective parameters of swallowing in both EPO and LPO periods.

Landmarks for Proper Round Window Electrode Insertion in Cochlear Implantation.

BACKGROUND: This study aimed to evaluate the role of landmarks for proper round window electrode insertion in cochlear implantation surgery. METHODS: This is a case series study. We included 150 patients undergoing cochlear implantation in a tertiary medical center during the period from January to December 2019. Patients with inner ear malformations or ossification or revision surgery were excluded. Three surgeons partici- pated in the study. During surgery, the round window electrode insertion was marked using 5 surgical landmarks: oval window, pyramid, fustis, round window membrane, and arborization of intracochlear blood vessels. Each surgeon reported on the identification of each landmark and its reliability for round window electrode insertion. RESULTS: Oval window and round window membrane were clearly seen by the 3 surgeons in all cases. Pyramid was seen in 94% of cases, fustis in 85%, and intracochlear wall in 90% of cases. The postoperative transorbital x-ray confirmed the intracochlear position of electrodes in all cases. CONCLUSION: Round window electrode insertion can be precisely performed using these 5 surgical landmarks in straight forwards cases as well as in difficult cases. These landmarks can also assist in teaching young surgeons, in a step-wise manner, how to properly do round window electrode insertion in cochlear implantation surgery.

Patterns and clinical outcomes of olfactory and gustatory disorders in six months: Prospective study of 1031 COVID-19 patients.

OBJECTIVE: This study aims to comprehensively evaluate olfactory and gustatory dysfunctions during the COVID-19 pandemic regarding onset, course, associated symptoms, prognosis and relation to patients' demographics, treatment received and other symptoms. PATIENTS& METHODS: This is a prospective study conducted on patients proven to be infected with COVID-19 and with olfactory/gustatory dysfunction symptoms. Detailed history was taken from each patient about the onset of this dysfunction, associated symptoms. Then follow-up survey was done after 6 months to evaluate the prognosis. RESULTS: 1031 patients were included in the study, aged 18 to 69 years old, with 31.8% were male. Olfactory/gustatory dysfunctions occurred after other COVID-19 symptoms in 43.5% of cases, occurred suddenly in 80.4% and gradually in 19.6%. These dysfunctions were anosmia & ageusia in 50.2%, hyposmia & hypogeusia in 23.3%, anosmia alone in 17.7%, phantosmia in 18%, Parosmia in 28.4%. In terms of recovery 6-month follow up, 680 patients (66%) recovered completely, 22.1% recovered partially while 11.9% did not recover. Most improvement occurred in the first two weeks. Headache, malaise, nasal obstruction and rhinorrhea were the commonest COVID-19 symptoms associated. CONCLUSION: Most recovery of olfactory/gustatory dysfunction in COVID-19 infection occurs at the first two weeks and is unrelated to patient demographics, treatment or olfactory training. Parosmia is an independent predictor for complete recovery, while phantosmia is significantly associated with lower probability of complete recovery.

Maternal COVID-19 and neonatal hearing loss: a multicentric survey.

PURPOSE: Gestational SARS-Cov-2 infection can impact maternal and neonatal health. The virus has also been reported of causing sensorineural hearing loss. The objective of this study was to determine the possible effect of maternal SARS-COV-2 infection on neonatal hearing as identified during universal hearing screening. METHODS: Retrospective cohort study in two tertiary referral centers including all neonates born from November 2020 through April 1st, 2021 and undergoing the universal hearing screening program. Maternal Covid-19 infection was recorded (timing and severity) and the results of hearing screening of their neonates compared to the incidence of neonatal hearing loss results of the national universal screening program during the same period. RESULTS: A total of 984 neonates were included (508 males and 476 females). Sixty-three neonates were excluded due to comorbidities which could cause hearing loss. The incidence of failed responses in the community at large was 2.3%. Twenty-seven failed both steps of screening (2.9%; p < 0.2). There were 34 Covid-19 positive mothers (17 in the first trimester, 8 in the second and 9 in the third). Twenty-nine neonates failed the first screening (p < 0.00001) but on further testing only one neonate failed (2.9%). CONCLUSION: In this study, neonates born to Covid-19 positive mothers do not seem to have an increased risk of hearing loss. However longer follow-up of these neonates is mandatory to detect any possible delayed effects of the virus.

Case Report of Laryngeal Paraganglioma.

Paragangliomas are rare tumors in head and neck region. Laryngeal paragangliomas are very rare subtype of these tumors. We here present a case of laryngeal paraganglioma which was totally excised laryngoscopically. Good collaboration with the pathologist is essential to confirm the diagnosis. Precise diagnosis is important for ideal management.

Association of Pharyngocutaneous Fistula With Cancer Outcomes in Patients After Laryngectomy: A Multicenter Collaborative Cohort Study.

Importance: Pharyngocutaneous fistula (PCF) results in an inflammatory reaction, but its association with the rate of locoregional and distant control, disease-free survival, and overall survival in laryngeal cancer remains uncertain. Objective: To determine if pharyngocutaneous fistula after salvage laryngectomy is associated with locoregional and distant control, disease-free survival, and/or overall survival. Design, Setting, and Participants: A multicenter collaborative retrospective cohort study conducted at 5 centers in Canada and the US of 550 patients who underwent salvage laryngectomy for recurrent laryngeal cancer from January 1, 2000, to December 31, 2014. The median follow-up time was 5.7 years (range, 0-18 years). Main Outcomes and Measures: Outcomes examined included locoregional and distant control, disease-free survival, and overall survival. Fine and Gray competing risk regression and Cox-proportional hazard regression models were used for outcomes. Competing risks and the Kaplan-Meier methods were used to estimate outcomes at 3 years and 5 years. Results: In all, 550 patients (mean [SD] age, 64 [10.4] years; men, 465 [85%]) met inclusion criteria. Pharyngocutaneous fistula occurred in 127 patients (23%). The difference in locoregional control between the group of patients with PCF (75%) and the non-PCF (72%) group was 3% (95% CI, -6% to 12%). The difference in overall survival between the group with PCF (44%) and the non-PCF group (52%) was 8% (95% CI, -2% to 20%). The difference in disease-free survival between PCF and non-PCF groups was 6% (95% CI, -4% to 16%). In the multivariable model, patients with PCF were at a 2-fold higher rate of distant metastases (hazard ratio, 2.00; 95% CI, 1.22 to 3.27). Distant control was reduced in those with PCF, a 13% (95% CI, 3% to 21%) difference in 5-year distant control. Conclusions and Relevance: This multicenter retrospective cohort study found that development of PCF after salvage laryngectomy is associated with an increased risk for the development of distant metastases.

Outcomes of combined use of topical and intravenous tranexamic acid on surgical field quality during functional endoscopic sinus surgery: Randomized controlled trial.

BACKGROUND: Intraoperative bleeding during functional endoscopic sinus surgery (FESS) poses a challenge to both surgeon and anesthetist. The primary aim of this study was to evaluate the effectiveness of local, intravenous and combined use of tranexamic acid (TA) in improving the surgical field quality during FESS. METHODS: We conducted a randomized controlled double-blinded prospective trial on 120 patients scheduled for elective FESS. After induction of general anesthesia, patients were randomly and evenly assigned to one of four groups; IV TA, local TA, both IV and local TA, and placebo. Surgical field was assessed using five-point Boezaart scale. Total fentanyl and esmolol consumption, operative time, recovery time, and postoperative complications were recorded. RESULTS: Surgical field quality score was the best in IV and local TA group compared to others (p < 0.001). Mean operative time was found significantly shorter in IV and local TA group than placebo one. Total fentanyl consumption was significantly lower in IV and local TA group comparing to others (p = 0.025). Mean recovery time was significantly shorter for IV and local TA group compared to others. Total fentanyl consumption was significantly lower in IV and local TA group comparing to others. No significant differences were found of mean arterial pressure and heart rate decline in four groups. None of the patients in four groups required esmolol administration. No statistically significant differences were found in change of hemoglobin, hematocrit, prothrombin time, and partial thromboplastin time over time in all groups. CONCLUSION: The combined use of topical and intravenous TA provided the best surgical field in FESS, less fentanyl consumption, and less recovery time without causing significant side effects.

Minimally Invasive Technique for Correction of Prominent Ear.

OBJECTIVES: Prominent ear is not an uncommon deformity with 5% prevalence in population worldwide. Although there is no physiological handicap in this deformity, it affects the psychology and social integration, especially in children. Many surgical techniques are performed to correct this deformity. In this study, we illustrate a minimally invasive technique in cases of prominent ear and evaluate its efficacy. MATERIALS AND METHODS: A total of 16 patients were operated by incisionless otoplasty in both ears simultaneously. Therefore, 32 ears were included in the study. Postoperative follow-up was carried out for 6 months to determine the efficacy of this technique, complications, and recurrence of the abnormal shape. RESULTS: Two ears (2/32) were presented with slight protrusion three months postoperatively Three ears had postoperatively exposed sutures that needed to be embedded again under local anesthesia. The satisfaction rate was found to be 88% by the visual analog scale. No perichondritis or other complications occurred postoperatively. The technique results in correction of the deformity without any visible evidence of surgery. CONCLUSION: This technique is effective and safe for correction of prominent ear with negligible rate of complications and rapid recovery time.

Arterial Blood Supply of the Nose: An Angiographic Study.

OBJECTIVE: To define anatomical variations associated with arterial blood supply of the nose which has clinical implications on the management of different disorders, especially intractable posterior epistaxis. STUDY DESIGN: Case series. METHODS: Selective angiography of external and internal carotid arteries of 100 patients scheduled for routine angiography was done. RESULTS: Different anatomical variations were documented. The ophthalmic artery can arise from the middle meningeal artery in 1% while ethmoidal arteries can be absent in 5%. The maxillary artery courses as 2 loops in the pterygopalatine fossa in 64% of cases where the descending palatine artery originates before the first loop or on its top so that caution is needed in controlling epistaxis. The sphenopalatine artery has different patterns of branching and may have more than 2 branches in 18% of cases. In 19% of cases, there is cross-circulation between both sides through the nasal blood supply. CONCLUSION: Angiographic study of the nose is a very helpful tool for accurate knowledge of anatomical variations of the arteries with a tremendous effect on our surgical approaches and techniques for the management of different diseases in the nasal region, especially intractable posterior epistaxis.