Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study.

CONTEXT: Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined. OBJECTIVE: To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities. METHODS: Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023. RESULTS: There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17-91 years old). 58.6% (n = 51) of patients had carotid body tumours, 25.2% (n = 22) glomus vagal tumours, 6.8% (n = 6) tumours in the middle ear, 2.2% (n = 2) in the parapharyngeal space and 1.1% (n = 1) in the sphenoid sinus. 5.7% (n = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5-6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (n = 36), SDHB in 12.6% (n = 11), SDHC in 2.2% (n = 2) and SDHA in 1.1% (n = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (n = 45) of patients, radiotherapy in 14.9% (n = 13), observation in 28.7% (n = 25), and somatostatin analogue therapy with octreotide in 4.5% (n = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (p = .04), tumour size exceeding 2 cm (p = .03), positive SDHx variants (p = .01), and vagal and jugular tumours (p = .04). CONCLUSION: The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 2: The Translabyrinthine Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 2, we present a codified operative workflow for the translabyrinthine approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Seventeen consultant skull base surgeons (nine neurosurgeons and eight ENT [ear, nose, and throat]) with median of 13.9 years of experience (interquartile range: 18.1 years) of independent practice participated. There was a 100% response rate across both the Delphi rounds. The translabyrinthine approach had the following five phases and 57 unique steps: Phase 1, approach and exposure; Phase 2, mastoidectomy; Phase 3, internal auditory canal and dural opening; Phase 4, tumor debulking and excision; and Phase 5, closure. Conclusion We present Part 2 of a national, multicenter, consensus-derived, codified operative workflow for the translabyrinthine approach to vestibular schwannomas. The five phases contain the operative, steps, instruments, technique errors, and event errors. The codified translabyrinthine approach presented in this manuscript can serve as foundational research for future work, such as the application of artificial intelligence to vestibular schwannoma resection and comparative surgical research.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 1: The Retrosigmoid Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 1, we present a codified operative workflow for the retrosigmoid approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus, was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Eighteen consultant skull base surgeons (10 neurosurgeons and 8 ENT [ear, nose, and throat]) with median 17.9 years of experience (interquartile range: 17.5 years) of independent practice participated. There was a 100% response rate across both Delphi's rounds. The operative workflow for the retrosigmoid approach contained three phases and 40 unique steps as follows: phase 1, approach and exposure; phase 2, tumor debulking and excision; phase 3, closure. For the retrosigmoid approach, technique, and event error for each operative step was also described. Conclusion We present Part 1 of a national, multicenter, consensus-derived, codified operative workflow for the retrosigmoid approach to vestibular schwannomas that encompasses phases, steps, instruments, technique errors, and event errors. The codified retrosigmoid approach presented in this manuscript can serve as foundational research for future work, such as operative workflow analysis or neurosurgical simulation and education.

Patient perceptions of a remote assessment pathway in otology: a qualitative descriptive analysis.

PURPOSE: This study aims to gain in-depth feedback on patient perceptions of remote assessment in otology, to better inform the development of a telemedicine pathway for new otology referrals. METHODS: A qualitative descriptive approach was employed to analyse semi-structured interviews from 14 patients seen in a routine otology clinic. RESULTS: Patients were generally accepting of the proposed telemedicine pathway. Key themes included maintaining quality of care, adequate training for facilitators, reducing waiting times, appropriate actioning of clinic outcomes and anxiety surrounding the use of technology. CONCLUSIONS: Our proposed telemedicine pathway for new otology referrals is acceptable to patients, provided there is no compromise in the standard of their care versus a traditional pathway. These results further our understanding of remote assessment in otology from the patient perspective and may help to inform the development of such pathways outside of our centre.

Cholesteatoma in Children with Sotos Syndrome.

BACKGROUND: Sotos syndrome is a rare genetic disorder characterized by neurodevelopmental delay and excessive childhood growth including macrocephaly. In this study, we present our experience of children with Sotos syndrome and cholesteatoma. METHODS: Retrospective case note review and cross-referencing with hospital picture archive and communication systems or cases identified from a prospective database of consecutive cholesteatoma surgeries. RESULTS: A total of 400 children underwent surgery for acquired cholesteatoma and 5 (1%) had Sotos syndrome (1 bilaterally). In comparison, 42(11%) had cleft palate which is around 10 times more common than Sotos syndrome, 5 (1%) had Down syndrome, and 3 (1%) had Turner syndrome. The median age at primary surgery was 8 years old (3.5-10.9 years), 124 children with Sotos syndrome were identified in picture archive and communication systems (4% with cholesteatoma) of which temporal bone imaging was available in 86 (70%) at the median age of 9 years (0-17.2), and 33/86 (38%) had normal ears bilaterally on all imaging. Changes consistent with fluid or inflammation were present in 9/30 (30%) computed tomography and 24/72 (33%) magnetic resonance imaging scans. Development of mastoid pneumatization was impaired in 20/30 (67%) computed tomography and 8/72 (11%) magnetic resonance imaging scans. At 5 years, children with Sotos syndrome (33%) had greater recidivism than those with cleft palate (15%) (Kaplan-Meier log-rank analysis, P=.001) CONCLUSION: Children with Sotos syndrome appear to be at increased risk of developing acquired cholesteatoma. Impaired temporal bone pneumatization is a common incidental finding in Sotos syndrome in keeping with this risk. Further study of this previously unreported association may improve the understanding of pathogenetic mechanisms in cholesteatoma.

Esterified Hyaluronic Acid Placed in the Middle Ear Does Not Improve Outcomes in Cholesteatoma Surgery.

BACKGROUND: The aim of this article is to assess the efficacy of esterified hyaluronic acid as a barrier to formation of adhesions and improvement of tympanomastoid ventilation. METHODS: A prospective cohort analysis was performed at a tertiary referral centre. 126 ears were analysed in children with cholesteatoma. Esterified hyaluronic acid was placed on the promontory of 63 ears at primary canal wall intact surgery for cholesteatoma. No esterified hyaluronic acid was used in 63 control ears. Cholesteatoma recurrence, histopathological analysis of scar tissue following second-stage procedure, and middle ear pressure were the main outcome measures. RESULTS: At 5 years, esterified hyaluronic acid (7%) and non-esterified hyaluronic acid (10%) did not differ in cholesteatoma recurrence (Kaplan- Meier log rank analysis, P=.52). Esterified hyaluronic acid (n=11) and non-esterified hyaluronic acid (n=2) ears formed scar at the site of packing material (n=11) (Fisher's exact test, P=.04). Foamy histiocytes/macrophages were found in esterified hyaluronic acid (n=15) and non-esterified hyaluronic acid ears (n=1) (Fisher's exact test, P-125 daPa) in 44% (14/32) esterified hyaluronic acid ears and 42% (15/36) non-esterified hyaluronic acid ears (P=1.0, Fisher's exact test). CONCLUSIONS: We have discontinued the use of esterified hyaluronic acid in cholesteatoma surgery due to lack of detectable benefit. Esterified hyaluronic acid in the middle ear neither reduces cholesteatoma recurrence nor appears to improve the ventilation of the middle ear. Furthermore, esterified hyaluronic acid alters the inflammatory process within the middle ear, the significance of which remains unclear.

Comparing Transcanal Endoscopic Ear Surgery to Post-Auricular Microscope-Guided Surgery in Pediatric Ossiculoplasty: Hearing Outcomes and Post-Operative Pain.

OBJECTIVE: This study compares post-operative hearing outcomes and morbidity after pediatric total ossicular replacement prosthesis (TORP) ossiculoplasty with transcanal totally endoscopic ear surgery (TEES) versus a post-auricular microscope-guided (PAM) approach. PATIENTS: Forty-four children who underwent ossiculoplasty with titanium TORP after previous cholesteatoma surgery. INTERVENTION: Ossiculoplasty using TEES or PAM approach. MAIN OUTCOME MEASURES: Hearing outcome after ossiculoplasty was determined by post-operative air-bone gap (ABG) on audiogram nearest to 1 year after surgery. Post-operative morbidity was measured by total number of opiate doses the child received during hospital stay, along with the highest documented post-operative pain score. Comparisons were made with Mann-Whitney U test. RESULTS: Hearing data were available for 41 patients: 21 had undergone TEES (median preoperative ABG 39 dB) and 20 had PAM surgery (median preoperative ABG 39 dB). Post-operatively at 1 year, ABG closed significantly in each group (TEES 21 dB, p = 0.003; PAM 23 dB, p = 0.01), and there was no difference between groups (p = 0.6). 57% who underwent TEES and 50% who underwent PAM surgery experienced serviceable hearing post-operatively, defined as air conduction pure-tone average (PTA) ≤ 30 dB HL. Visual analogue pain scores from 0 (no pain) to 10 (worst pain imaginable) were available for 13 who underwent TEES and 18 who underwent PAM surgery. In children undergoing TEES, only two reported pain above 0, with the highest pain score being 4. Children undergoing PAM surgery had a median pain score of 3 (median difference = 3, p < 0.001). Children undergoing TEES required fewer weight appropriate doses of opiate analgesic (median = 0) than children who underwent PAM surgery (median = 1) (median difference = 1, p = 0.003). Children undergoing TEES had a significantly shorter surgical time (median 135 min) than those who underwent PAM surgery (median 168 min) (median difference = 33 min, p = <0.006). CONCLUSION: Hearing outcomes in TORP ossiculoplasty are similar in TEES and PAM surgery, and TEES may decrease post-operative pain.

A Multicenter Retrospective Case Review of Outcomes and Complications of S53P4 Bioactive Glass.

OBJECTIVE: The objective of this multicenter retrospective case review was to assess the natural clinical course, efficacy, and safety of mastoid obliteration with S53P4 Bioactive Glass (bioactive glass). METHODS: Retrospective case note review in a regional Tertiary Referral Centre and District General Hospital. Patients undergoing mastoid cavity obliteration as part of primary or secondary procedure with bioactive glass between 2012 and 2018. Outcome measures were assessed from a prospectively collated database and case note review. Primary outcomes were the common morbidities of a mastoid cavity; dry or discharging ear (Merchant's scale), vertigo in cold air, and a watertight middle ear. Patients were also assessed for audiological outcomes and recidivism. RESULTS: Ninety patients were included. During the follow-up period, (mean, 22 months; range, 6-59 months) cholesteatoma recidivism was observed in 2% of ears (2 patients). An acceptably dry (Merchant Grade 0-1) ear was achieved in 91% of all ears (95% primary cases, 80% secondary cases). Delayed healing of the graft in the external ear canal retaining the S53P4BAG Bioactive Glass (BonAlive Ò (BonAlive Ò Biomaterials Ltd., Turku, Finland)) within the mastoid occurred in 13% (12 ears). However, in all cases, conservative management resulted in complete healing. CONCLUSIONS: Bioactive glass provides a safe and effective means of mastoid obliteration. Complications including overlay graft failure and slow epithelialization, resulting in prolonged postoperative discharge (up to 2 months) and dehiscence into the external ear canal, do not preclude full recovery and may be successfully managed conservatively.

Prospective Comparison of Pediatric Endoscopic Lateral Graft and Interlay Tympanoplasty.

OBJECTIVE: To compare outcomes and complications of endoscopic lateral graft (LGT) and interlay (IT) tympanoplasty. STUDY DESIGN: Prospective observational cohort study of totally endoscopic ear surgery (TEES) tympanoplasty. SETTING: Pediatric tertiary referral center. PATIENTS: One hundred fourteen surgeries for children with tympanic membrane perforation without cholesteatoma. INTERVENTION: Porcine-derived collagen graft tympanoplasty using either LGT or IT. MAIN OUTCOME MEASURES: Closure rates of perforation, hearing outcomes (four-tone average air conduction [AC] and air bone gap), and complications that required further surgery were assessed 12 months postoperatively. RESULTS: Perforation closure rates did not differ between LGT (52/59 (88%)) and IT (45/51 (88%)), (Fisher's exact p = 1.00) (four ears lost to follow-up). AC thresholds (p = 0.32) and air bone gap (p = 0.88) improved similarly after surgery with LGT (median 8.8 dB) and IT (median 7.5 dB). The proportion of ears with serviceably normal hearing (AC ≤ 30 dB HL) postoperatively was similar (LGT 40/49 (82%), IT 36/46 (78%), Fisher's exact p = 0.80). Following IT, three (6%) ears developed inclusion cholesteatoma requiring revision surgery. One (2%) LGT ear developed blunting after myringitis. CONCLUSIONS: IT and LGT provide similar perforation closure rates and hearing outcomes. When choosing between these two techniques to repair pediatric tympanic membrane perforations, LGT would seem to be preferable than IT due to the risk of inclusion cholesteatoma after IT. There is a small risk of anterior blunting after LGT.

The Effect of the Status of the Ossicular Chain and Choice of Graft Material on Hearing Outcomes in Pediatric Cholesteatoma Surgery.

OBJECTIVE: Compare hearing benefit of incus preservation in primary cholesteatoma surgery versus cartilage-myringostapediopexy. METHODS: Prospective cohort study in a tertiary referral center. Tympanoplasty utilizing cartilage or other grafts, with or without intact incus was performed in 195 ears (187 children) with intact stapes. Outcome measures were pre and post-operative four-tone air conduction (AC) threshold (0.5, 1, 2, 4 kHz) and proportion with normal hearing (AC ≤ 30 dB HL) at 12 months. RESULTS: Ears with intact ossicles had better post-operative AC thresholds than those with incus eroded or removed (median 20 dB HL vs. 30 dB HL, Mann-Whitney P < .001). The normal hearing rate was 81/106 (74%) with intact incus and 46/89 (52%) without (Fisher's exact P = .001). Ears without intact incus and a cartilage-myringostapediopexy had better post-operative thresholds than those with a non-cartilage graft (28.8 dB HL vs. 36.3 dB HL, Mann-Whitney P = .005). Of ears without intact incus, 37/59 (63%) with a cartilage-myringostapediopexy and 9/30 (30%) with a non-cartilage graft had normal hearing post-operatively (Fisher's exact P = .007). By preserving the incus in 12 ears, 1 more ear would have normal hearing than with incus removal plus cartilage-myringostapediopexy (NNT = 12 (CI 3.6-); Fisher's exact = 0.1). CONCLUSION: Preserving an intact ossicular chain conveys a small but significant hearing benefit in cholesteatoma surgery, the magnitude of which should be considered before deciding to remove the intact incus. Cartilage-myringostapediopexy provides a significant gain in hearing when the incus is absent, even without a partial ossicular replacement prosthesis.

Temporal bone dysplasia in Coffin-Siris syndrome.

We report a child, diagnosed with Coffin-Siris syndrome (CSS), with chronic right otorrhoea. CT and DR-MRI were performed to further investigate, diagnose and determine relevant surgical anatomy. CT temporal bones assessment was performed, and the measurements compared with previously published data for normal temporal bone anatomy. These comparisons highlighted various differences which were not initially expected; it showed that there were multiple inner ear abnormalities in addition to middle ear disease. This case highlights the importance of considering temporal bone abnormalities in all children with CSS or any dysmorphia, when they may require mastoid procedures. Reviewing the management of this case provides relevant learning opportunities for both primary, secondary and tertiary care institutions.

Design, prototype development and pre-clinical validation of a novel instrument with a compliant steerable tip to facilitate endoscopic ear surgery.

This work presents the design of a novel compliant steerable tip (CST) instrument to facilitate transcanal (or totally) endoscopic ear surgery (TEES). The evolution of the instrument's design is shown, where prototypes were evaluated by surgeons and their feedback was used to inform the design changes for the next prototype iteration. The final prototype enables the surgeon to articulate the compliant tip to achieve the desired bending curvature while automatically locking in place and providing dissection and suction capabilities. Pre-clinical validation testing was performed in goat and human cadaver models by two surgeons who successfully removed an allograft from the middle ear. Time and the number of blockages while suctioning saline in both cadaver models were measured and compared with current instruments used during TEES. The CST took significantly less time to suction saline within a flooded middle ear compared to the Panetti suction dissector (PSD) for atticus and underwent less blockages than the PSD for atticus, ear drum and sinus tympani instruments, Wilcoxon Method p < .05. Our study demonstrates the development and successful clinical evaluation of a minimally invasive surgical instrument designed to facilitate endoscopic approaches to the ear.

Measurement Properties of the Hearing Environments and Reflection of Quality of Life (HEAR-QL) 28-item Questionnaire in Cholesteatoma.

OBJECTIVE: We sought to assess the reliability and construct validity of the Hearing Environments and Reflection on Quality of Life Adolescent (HEAR-QL 28) quality of life measure (QoL) in cholesteatoma. STUDY DESIGN: Observational. SETTING: Tertiary referral center. PATIENTS: One hundred seventeen patients with a diagnosis or history of cholesteatoma completed HEAR-QL 28. In addition to patients within the age range recommended for HEAR-QL 28 (13-18 yr), patients under 13 years old who were able to complete HEAR-QL 28 without parental assistance were included. INTERVENTION S: Completion of HEAR-QL 28 QoL measure. MAIN OUTCOME MEASURE S: HEAR-QL 28 score, four tone average pure tone audiogram hearing threshold and categorical classification of hearing loss as mild, moderate, and severe. RESULTS: HEAR-QL 28 did not demonstrate discriminative ability on the basis of audiometric threshold, but did discriminate between participants hearing normally (four tone average pure tone audiogram <30 dB HL) (HEAR-QL 86/100) after cholesteatoma surgery from those with unilateral hearing loss (HEAR-QL 73/100) (p < 0.001). Those with unilateral loss could in turn be differentiated from those with bilateral loss (HEAR-QL 60/100) (p < 0.006). CONCLUSION: HEAR-QL 28 is valid measure of QoL in cholesteatoma with no evidence of redundancy and excellent internal consistency. The importance of considering QoL impact of cholesteatoma is highlighted by 17% of participants reporting the normality or abnormality of their hearing differently from their audiometric threshold. The HEAR-QL 28 provides insight into the ability to cope with their hearing environment in a specific environment.

Management of squamous cell carcinomas of the skull-base.

PURPOSE: The purpose of this review is to assess the recent evidence regarding the management of squamous cell carcinoma of the skull-base and to discuss the implications of these findings on clinical practice. METHOD: Free text Medline and MeSH term search of publications relating to Squamous Cell Carcinoma & Skull-base and Skull base, Neoplasm respectively. Multidisciplinary clinical guidelines were also reviewed. RESULTS: The primary search yielded a total of 271 papers which following initial review was reduced to 28. Secondary search yielded 56 papers. There were no randomised controlled trials relating to squamous cell carcinoma of the skull-base and as such this review is based on cohort studies, case series and expert opinion. CONCLUSION: Squamous cell carcinoma (SCC) is the most common cancer occurring in the Head and Neck. Squamous cell carcinoma is also the most common cancer arising within the nose and sinuses of which skull-base squamous cell carcinoma is a rare subgroup. Evidence relating to the management and survival of skull-base SCC is based on expert opinion and. retrospective analyses Clinical examination and biopsy, imaging and a broad multidisciplinary team are key to the management of skull-base SCC. The information gathered should be used to guide informed discussion by suitably trained experts with patients regarding surgical approach, post-operative recovery and adjuvant or neoadjuvant treatments. The standard of care is currently to perform skull base resection with or without additional craniotomy, pedicled or free flap reconstruction in multiple layers and post-operative radiation (usually photons or protons). Open approaches have traditionally been the mainstay, however in certain cases endoscopic approaches can yield equivalent results and offer many advantages. Despite advances in care survival remains poor with a nearly one in five risk of nodal recurrence within two years.