Evaluation of vestibular function following endolymphatic sac surgery.

PURPOSE: To evaluate objective vestibular function after endolymphatic sac surgery (ELSS) for Menière's disease (MD), using comparative vestibular function tests: videonystagmography (VNG), vestibular evoked myogenic potentials (VEMP) and video head-impulse test (VHIT) METHODS: Patients with definite MD using the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) of 1995 criteria modified in 2015 and treated with ELSS (sac decompression or sac opening) were included. The primary outcome was the preservation of vestibular function, comparing pre- and postoperative vestibular function tests: VNG, VEMP, VHIT. Secondary outcomes were control of episodes of vertigo, hearing outcome using AAO-HNS criteria, and QoL using the Menière's disease outcome questionnaire. RESULTS: 73 patients were included in the study. We found a significant preservation of vestibular function as measured by VNG and VHIT. There was no statistical difference in the presence or absence of cervical and ocular (P13/N23 and N1/P1) waves on VEMP pre- and postoperatively. 67% of patients had good control of episodes of vertigo post-operatively, with significantly better results in the sac opening group (75%). There was no significant change in hearing postoperatively, and QoL scores were significantly improved after surgery (p < 0.0001). CONCLUSION: Endolymphatic sac surgery (ELSS) is a conservative surgical treatment, which does not negatively impact vestibular function. It was associated with improved control of episodes of vertigo, preservation of hearing, and a clear improvement in QoL scores. Despite its pathophysiology not being fully understood, it remains a first-line procedure preserving vestibular function, for MD refractory to medical management.

Cochleovestibular involvement in patients with Fabry disease: data from the multicenter cohort FFABRY.

PURPOSE: Fabry disease (FD) is a lysosomal storage disease responsible for cochleovestibular involvement. Exact prevalence and pathophysiological mechanisms behind ENT affections are still poorly known. Treating FD with enzyme replacement therapy (ERT) does not seem to significantly improve the ENT symptoms, while the impact of migalastat has yet to be determined. METHODS: We carried out a retrospective multi-centre study on 47 patients from the FFABRY cohort who had an ENT consultation in the context of their FD. The information collected were as follows: clinical examination, videonystagmoscopy, pure-tone speech audiometry, videonystagmography or VHIT (Video Head Impulse Test). Severe hearing loss was defined as greater than 70 dB. RESULTS: The median age of our cohort was 52 years with a non-negligible proportion of non-classic variants and female carriers. 72.3% of the patients complained of at least one of the following symptoms: hearing loss, tinnitus or vertigo. Pure-tone audiometry was abnormal in 61.7% of the patients (29/47), while speech audiometry was abnormal for 41.7% of the patients. The age of the patients and hypertrophic cardiomyopathy were significantly associated with the existence of an anomaly in pure-tone audiometry results. Severe hearing loss (> 70 dB) was significantly more common in male patients. DISCUSSION: Hearing loss is particularly frequent in FD and is not limited to classic phenotypes. Close ENT follow-up is essential for Fabry patients to detect those who might benefit from hearing aid. Further studies are needed to define the impact of migalastat on cochleovestibular symptoms.

The Royal College of surgeons multidisciplinary guidelines on elective tracheostomy insertion in COVID-19 ventilated patients.

BACKGROUND: The current COVID-19 pandemic has placed enormous strain on healthcare systems worldwide. Understanding of COVID-19 is rapidly evolving. Pneumonia associated with COVID-19 may lead to respiratory failure requiring mechanical ventilation. The rise in patients requiring mechanical ventilation may lead to an increase in tracheostomies being performed in patients with COVID-19. Performing tracheostomy in patients with active SARS-CoV-2 infection poses a number of challenges. METHODS: These guidelines were written following multidisciplinary agreement between Otolaryngology, Head and Neck Surgery, Respiratory Medicine and the Department of Anaesthetics and Critical Care Medicine in the Royal College of Surgeons in Ireland. A literature review was performed and a guideline for elective tracheostomy insertion in patients with COVID-19 proposed. CONCLUSION: The decision to perform tracheostomy in patients with COVID-19 should be undertaken by senior members of the multidisciplinary team. Steps should be taken to minimise risks to healthcare workers.

Bell's palsy in pregnancy: A scoping review of risk factors, treatment and outcomes.

Objective: There are limited studies reporting on Bell's palsy and pregnancy. Our study aimed to evaluate risk factors, current treatment options and facial function outcomes in women who developed Bell's palsy in pregnancy. To our knowledge this is the first review analyzing these factors. Data sources/review methods: A search of PubMed/MEDLINE, Embase, Web of Sciences and Scopus was carried out. Studies describing risk factors, treatment and/or facial function outcomes of Bell's palsy in pregnancy were included. PRISMA-Scr guidelines were followed. Results: The search yielded 392 abstracts, of which 15 studies were included for analysis. It was not possible to perform a meta-analysis due to small numbers and quality of studies. There were 559 patients included from the 15 studies. The third trimester was the most common time for Bell's palsy to occur (n = 364, 65%). Pre-eclampsia was the most common co-morbidity reported. The most common treatment was corticosteroids and the majority of patients had a complete recovery of their palsy (58%, n = 192). Conclusion: This analysis has evaluated all available data concerning risk factors, treatment and facial function outcomes of BP in pregnancy. The third trimester is the most common time for Bell's palsy to occur in pregnancy. There is currently a lack of high quality evidence into this condition in pregnancy. Level of evidence: 1.

Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis.

Langerhans cell histiocytosis is a rare condition affecting the temporal bone in up to 60% of cases. Symptoms are non-specific and the differential diagnosis includes infection, benign lesions such as cholesteatoma, and malignant lesions of the skull base. Here, we report the case of a 14-yearold child referred with chronic ear discharge, and background of multifocal Langerhans cell histiocytosis 9 years prior. Recurrence of Langerhans cell histiocytosis was initially suspected and systemic treatment was considered. Further imaging workup and surgical exploration of the mastoid showed a secondary acquired cholesteatoma arising from a dehiscent posterior ear canal wall. Surgical removal of the cholesteatoma was performed with a canal wall down procedure. We review the presentation and management of temporal bone Langerhans cell histiocytosis. We recommend that cholesteatoma should be considered in case of recurrence of otological symptoms in patients with a background of Langerhans cell histiocytosis.

Comparative study of vestibular function preservation in manual versus robotic-assisted cochlear implantation.

OBJECTIVE: To compare vestibular outcomes in cochlear implant (CI) surgery, between robotic-assisted insertion of the electrodes versus manual insertion. METHODS: We performed a monocentric retrospective study. From March 2021, the robotic system RobOtol© was used for all CI cases. We compared this robotic-assisted insertion group with a manual insertion group of patients who received a CI between July 2020 and March 2021. Primary objective was vestibular outcome. We used objective vestibular function tests: caloric testing, Vestibular Evoked Myogenic Potential (VEMP), and Video Head Impulse Test (VHIT). Secondary objectives were postoperative complications including patient-reported postoperative vertigo. RESULTS: We found no statistically significant difference between the two groups in terms of caloric testing, VEMP or VHIT outcomes. In patient-reported outcomes, there was significantly more vertigo in the manual insertion group compared with robotic-assisted insertion. CONCLUSION: It is hypothesized that a non-traumatic insertion would cause less vestibular dysfunction postoperatively. Larger prospective studies are required to determine whether robotic-assisted CI insertion has a significant impact on vestibular outcomes in CI surgery.

Pattern of nodal metastasis of cutaneous squamous cell carcinoma involving the temporal bone.

Objective: The objective of this study was to explore the pattern of lymph-node spread of SCCs involving the temporal bone. Methods: We retrospectively reviewed all cutaneous SCCs involving the temporal bone over a 20-year time-period. Forty-one patients were eligible. Results: Mean age was 72.8 years. The diagnosis was cutaneous SCC in all cases.All patients underwent a temporal bone resection, 70.7% had a neck-dissection and 78.0% a parotidectomy.Level 2 was the most common area of neck metastasis, and occurred in 12.2%. The parotid had disease in 34.1%. 51.2% of patients underwent free-flap reconstruction.Mean overall survival of the cohort was 4.2 years. Conclusions: Overall, the rate of cervical nodal metastasis was 22.0% and 13.5% in the occult setting. The parotid was involved in 34.1% and 10.0% in the occult setting. Results from the present study support consideration for performing a parotidectomy at the time of temporal bone resection, while a neck dissection can be performed for adequate staging of the nodal basin. Level of Evidence: 3.

Contemporary management of paragangliomas of the head and neck.

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors typically arising from nonsecretory head and neck parasympathetic ganglia. Historically thought of as aggressive tumors that warranted equally aggressive surgical intervention, evidence has emerged demonstrating that the vast majority of HNPGLs are slow growing and indolent. It is also now recognized that a large proportion of HNPGLs are hereditary with succinate dehydrogenase gene mutations typically implicated. These recent advances have led to significant changes in the way in which clinicians investigate and treat HNPGLs with most now opting for more conservative treatment strategies. However, a proportion of patients present with more aggressive disease and still require nonconservative treatment strategies. Recent studies have sought to determine in which groups of patients the morbidity associated with treatment is justified. We summarize the recent advances in the understanding and management of these tumors and we provide our recommendations regarding the management of HNPGLs.

Persistent Stapedial Artery Encountered during Cochlear Implantation.

OBJECTIVES: Persistent stapedial artery (PSA) is a rare congenital anomaly that can complicate middle ear surgery. METHODS: We present the case of a 25-year-old male who underwent right-sided cochlear implantation. A PSA was encountered lying over the middle promontory intraoperatively. RESULTS: The PSA was carefully lifted off the middle ear promontory using a Hughes elevator to divide adhesions and delineate the artery. The implant electrode was placed through the round window niche in the usual fashion. Tragal cartilage and fibrin glue were used to control the trajectory of the electrode. CONCLUSION: Cochlear implantation can be performed safely in patients with PSA.

National study of hearing preservation rates and outcomes after cochlear implantation in Ireland.

OBJECTIVE: To study the rate of hearing preservation and outcomes of hearing preservation candidates in a national cochlear implant centre. The HEARRING criteria was used. METHODS: All cochlear implant candidates with preserved low frequency pure tone average (PTA) were included. All patients underwent cochlear implantation using a standard 'soft-surgery' technique. PTA was assessed at switch-on, 3, 6, 9 and 12 months postoperatively. The primary outcome was hearing preservation at 12 months. RESULTS: Sixty six patients were included in the study between 2015 and 2020. Seventy one ears were implanted including 33 adults and 33 children with 5 bilateral implantations. Mean preoperative PTA was 74.8 dB (range 52.3-92 dB), mean postoperative PTA was 95.3 dB corresponding to a mean shift of 20.5 dB. In the adult population, HP rates were as follows: complete HP in 13%, partial HP in 39.1%, minimal HP in 30.4%, loss of hearing in 17.4%. In the paediatric population: complete HP in 20.7%, partial HP in 51.7%, minimal HP in 13.8% and loss of hearing in 13.8%. After the initial postoperative shift, there was no significant worsening of residual hearing during follow-up between 3 and 12 months. There were no significant prognostic factors for hearing preservation. CONCLUSION: Hearing preservation rates using the HEARRING criteria are described. This study will help counselling and decision making in patients eligible for cochlear implantation with hearing preservation. Further studies are required to assess the performances and outcomes of electronatural and electroacoustic stimulation.

Cerebrospinal Fluid in the Middle Ear in a Child.

A 25-month-old child presented with unilateral hearing loss; examination found unilateral right middle ear effusion of cerebrospinal fluid. What is your diagnosis?