Intraoperative assessment of cochlear nerve functionality in various vestibular schwannoma scenarios: Lessons learned.

The use of cochlear implants (CIs) is on the rise for patients with vestibular schwannoma (VS). Besides CI following tumor resection, new scenarios such as implantation in observed and/or irradiated tumors are becoming increasingly common. A significant emerging trend is the need of intraoperative evaluation of the functionality of the cochlear nerve in order to decide if a CI would be placed. The purpose of this paper is to explore the experience of a tertiary center with the application of the Auditory Nerve Test System (ANTS) in various scenarios regarding VS patients. The results are compared to that of the studies that have previously used the ANTS in this condition. Patients with unilateral or bilateral VS (NF2) who were evaluated with the ANTS prior to considering CI in a tertiary center between 2021 and 2023 were analyzed. The presence of a robust wave V was chosen to define a positive electrical auditory brainstem response (EABR). Two patients underwent promontory stimulation (PromStim) EABR previous to ANTS evaluation. Seven patients, 2 NF-2 and 5 with sporadic VS were included. The initial scenario was simultaneous translabyrinthine (TL) tumor resection and CI in 3 cases while a CI placement without tumor resection was planned in 4 cases. The ANTS was positive in 4 cases, negative in 2 cases, and uncertain in one case. Two patients underwent simultaneous TL and CI, 1 patient simultaneous TL and auditory brainstem implant, 3 patients posterior tympanotomy with CI, and 1 patient had no implant placement. In the 5 patients undergoing CI, sound detection was present. There was a good correlation between the PromStim and ANTS EABR. The literature research yielded 35 patients with complete information about EABR response. There was one false negative and one false positive case; that is, the 28 implanted cases with a present wave V following tumor resection had some degree of auditory perception in all but one case. The ANTS is a useful intraoperative tool to asses CI candidacy in VS patients undergoing observation, irradiation or surgery. A positive strongly predicts at least sound detection with the CI.

Clinical practice guideline on the management of vestibular schwannoma.

INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

Variations in the treatment of acute peripheral facial paralysis. A nationwide survey.

BACKGROUND: Acute peripheral facial paralysis may be diagnosed and treated by different specialists. OBJECTIVE: The aim of this study was to explore the variability in the treatment of Bell's palsy (BP) and Ramsay Hunt Syndrome (RHS) among different medical specialties. METHODS: An anonymous nationwide online survey was distributed among the Spanish Societies of Otorhinolaryngology (ORL), Neurology (NRL) and Family and Community Medicine (GP). RESULTS: 1039 responses were obtained. 98% agreed on using corticosteroids, ORL using higher doses than NRL and GP. Among all, only 13% prescribed antivirals in BP routinely, while 31% prescribed them occasionally. The percentage of specialists not using antivirals for RHS was 5% of ORL, 11% of NRL, and 23% of GP (GP vs. NRL p = 0.001; GP vs. ORL p < 0.0001; NRL vs. ORL p = 0,002). 99% recommended eye care. Exercises as chewing gum or blowing balloons were prescribed by 45% of the participants with statistically significant differences among the three specialties (GP vs. NRL p = 0.021; GP vs. ORL p < 0.0001; NRL vs. ORL p = 0.002). CONCLUSION: There is general agreement in the use of corticosteroids and recommending eye care as part of the treatment of acute peripheral facial paralysis. Yet, there are discrepancies in corticosteroids dosage, use of antivirals and recommendation of facial exercises among specialties.

Validation of the Spanish version of the Electronic Facial Palsy Assessment (eFACE).

PURPOSE: The clinician-graded electronic facial paralysis assessment (eFACE) is a relatively new digital tool for assessing facial palsy. The present study aimed to determine the validity and reliability of the Spanish version of the eFACE. METHODS: Forward-backward translation from the original English version was performed. Videos and photographs from 65 adult patients with unilateral facial paralysis (any severity, time course, and etiology) were evaluated twice by five otolaryngologists with varying levels of experience in facial palsy evaluation. Internal consistency was measured using Cronbach's α and the intra- and inter-rater reliability were measured using intraclass correlation coefficient. Concurrent validity was established by calculating Spearman's rho correlation (ρ) between the eFACE and the House-Brackmann scale (H-B) and Pearson's correlation (r) between the eFACE and the Sunnybrook Facial Grading System (SFGS). RESULTS: The Spanish version of the eFACE showed good internal consistency (Cronbach's α > 0.8). The intra-rater reliability was nearly perfect for the total score (intraclass correlation coefficient: 0.95-0.99), static score (0.92-0.96), and dynamic score (0.96-0.99) and important-to-excellent for synkinesis score (0.79-0.96). The inter-rater reliability was excellent for the total score (0.85-0.93), static score (0.80-0.90), and dynamic score (0.90-0.95) and moderate-to-important for the synkinesis score (0.55-0.78). The eFACE had a very strong correlation with the H-B (ρ = - 0.88 and - 0.85 for each evaluation, p < 0.001) and the SFGS (r = 0.92 and 0.91 each evaluation, p < 0.001). CONCLUSION: The Spanish version of the eFACE is a reliable and valid instrument for assessment of facial function in the diagnosis and treatment of patients with facial paralysis.

Inverted clip technique for cochlear implant electrode array fixation: how I do it.

BACKGROUND: Electrode migration has been identified as a complication primarily related to straight electrodes. To address this issue, the use of a fixation clip has been suggested as an alternative to stabilize the electrode lead and reduce the risk of migration. METHODS: A modified approach to the standard fixation clip procedure is introduced. Steps and technical considerations are presented in the video attached. CONCLUSIONS: This novel configuration offers enhanced fixation of the electrode array without requiring additional surgical time and without posing any additional risk to the facial nerve.

Validation of the Spanish version of the Sunnybrook facial grading system.

PURPOSE: The Sunnybrook facial grading system (SFGS) is one of the most widely employed tools to assess facial function. The present study aimed to determine the validity and reliability of the Spanish language version of the SFGS. METHODS: Forward-backward translation from the original English version was performed by fluent speakers of English and Spanish. Videos from 65 patients with facial paralysis (FP) were evaluated twice by five otolaryngologists with experience in FP evaluation. Internal consistency and intra- and inter-rater reliability were assessed. The House-Brackmann scale was used to display concurrent validity which was established by Spearman's rho correlation. RESULTS: The Cronbach's α score exceeded 0.70. The intra-rater intraclass correlation coefficient (ICC) was nearly perfect for the composite score (0.96-0.99), voluntary movements (0.97-0.99), and synkinesis (0.91-0.98), and important to almost perfect for symmetry at rest (0.79-0.97). In both evaluations, the inter-rater ICC was higher than 0.90 for the composite score (0.92-0.96) and voluntary movements (0.91-0.96) and slightly lower for symmetry at rest (0.66-0.85) and synkinesis (0.72-0.87). A strong negative correlation was found between the H-B scale and SFGS (Spearman's rho coefficient = - 0.92, p < 0.001) in both evaluations. CONCLUSION: The Spanish version of the SFGS is a reliable and valuable instrument for the assessment of facial function in the diagnosis and treatment of patients with FP.

Contribution and safety of the side-to-end hypoglossal-to-facial transfer in multidisciplinary facial reanimation.

BACKGROUND: This study evaluates facial and tongue function in patients undergoing side-to-end hypoglossal-to-facial transfer (HFT) with additional techniques. METHODS: Thirty-seven patients underwent a side-to-end HFT. Twelve had additional cross-face grafts, and 9 had an additional masseter-to-facial transfer. Facial was assessed with House-Brackmann (HB), Sunnybrook Facial Grading Scale (SFGS), and eFACE. Martins scale and the Oral-Pharyngeal Disability Index (OPDI) were used to assess tongue function. RESULTS: Ninety-four percent of cases reached HB grades III-IV. Mean total SFGS score improved from 16 ± 15 to 59 ± 11, while total eFACE score from 52 ± 13 to 80 ± 5. Dual nerve transfers were a predictor for a better eFACE total score p = 0.034, ß = 2.350 [95% CI, 0.184-4.516]), as well as for a higher SFGS total score (p = 0.036, ß = 5.412 [95% CI, 0.375-10.449]). All patients had Martin's grade I. Mean postoperative OPDI scores were 84 ± 17 (local physical), 69 ± 16 (simple and sensory motor components), 82 ± 14 (complex functions), and 73 ± 22 (psychosocial). CONCLUSIONS: The side-to-end HFT offers predictable facial function outcome and preserves tongue function in nearly all cases. Dual nerve transfers appear to improve the final outcome.

Clinical-radiological Correlation in Temporal Bone Fractures.

INTRODUCTION: The diagnosis of temporal bone fractures (TBF) is based on radiological imaging. The most widely used classification divides fractures into longitudinal, transverse, and mixed. In recent years, other classifications have emerged to better predict clinical manifestations. OBJECTIVES: To review computed tomography (CT) images of TBF, define their radiological pattern, and study the concordance of the observed findings with those described in the radiological report. To analyse the association between fracture types and clinical findings. To study the impact of mastoid pneumatization on fracture characteristics. METHODS: Retrospective study of 110 TBF diagnosed with CT between January 2016 and May 2019. RESULTS: Fifty-two transverse (47%), 34 longitudinal (30%), and 19 mixed fractures (17%) were identified with good interobserver agreement (k = .637). Longitudinal fractures were associated with conductive hearing loss (P < .001) and transverse fractures with sensorineural hearing loss (P = .005). Of the fractures, 8.2% showed involvement of the otic capsule, and were associated with sensorineural hearing loss (P < .001), facial paralysis (P = .019) and vertigo (P= .035). Fractures were more frequent in cases of greater pneumatization, and the involvement of the otic capsule in cases of very good pneumatization (P = .024). CONCLUSIONS: The traditional classification of TBF is reproducible. Its association with clinical findings improves when the involvement of the otic capsule is also analysed. Mastoid pneumatization is not a protective factor for TBF or for involvement of the otic capsule.

Quality of Life Following Cochlear Implantation in Patients With Menière's Disease.

Background: Menière's disease (MD) is a disorder characterized by auditory and vestibular dysfunction that significantly deteriorates patients' quality of life (QoL). In addition to the management of vestibular symptoms, some patients with bilateral hearing loss meet criteria for cochlear implantation (CI). Objectives: (1) To assess hearing results and QoL outcomes following CI in patients with MD. (2) To compare these results to a matched control group of patients who had undergone CI. (3) To analyse differences in MD patients who have undergone simultaneous or sequential labyrinthectomy or previous neurectomy. Methods: A retrospective analysis of a study group of 18 implanted patients with MD and a matched control group of 18 implanted patients without MD, who had CI at a tertiary referral center. Hearing and speech understanding were assessed via pure-tone audiometry (PTA) and disyllabic perception tests in quiet. QoL was assessed via the Nijmegen Cochlear Implant Questionnaire (NCIQ), the Glasgow Benefit Inventory (GBI), the Speech, Spatial and Qualities of Hearing Scale (SSQ12), and the Hearing Implant Sound Quality Index (HISQUI19). The impact of MD ablative surgeries was analyzed in the study group (MD group). Results: Mean pre-operative PTA thresholds were significantly lower in the MD group (103 vs. 121 dB). A significant improvement in hearing outcomes was observed following CI in both groups (p < 0.001), with a maximum Speech Discrimination Score of 64 and 65% disyllables at 65 dB for the MD and control group, respectively. Subjective outcomes, as measured by the NCIQ, GBI, SSQ12, and HISQUI19 did not significantly differ between groups. In the MD group, despite achieving similar hearing results, QoL outcomes were worse in patients who underwent simultaneous CI and labyrinthectomy compared to the rest of the MD group. Post-operative NCIQ results were significantly better in patients who had undergone a previous retrosigmoid neurectomy when compared to those who had undergone only CI surgery in the subdomains "basic sound perception" (p = 0.038), "speech" (p = 0.005), "activity" (p = 0.038), and "social interactions" (p = 0.038). Conclusion: Patients with MD and severe hearing loss obtain hearing results and QoL benefits similar to other CI candidates. Delayed CI after labyrinthectomy or vestibular neurectomy can be performed with similar or better results, respectively, to those of other cochlear implanted patients. Patients who undergo simultaneous CI and labyrinthectomy may achieve similar hearing results but careful pre-operative counseling is needed.

The Experience of a Facial Nerve Unit in the Treatment of Patients With Facial Paralysis Following Skull Base Surgery.

: The management of facial paralysis following skull base surgery is complex and requires multidisciplinary intervention. This review shows the experience of a facial nerve (FN) unit in a tertiary university referral center. A multidisciplinary approach has led to the breaking of some old treatment paradigms. An overview of five FN scenarios is presented. For each setting a contemporary approach is proposed in contrast to the established approach. 1) For patients with an anatomically preserved FN with no electrical response at the end of surgery for vestibular schwannoma, watchful waiting is usually advocated. In these cases, reinforcement with an interposed nerve graft is recommended. 2) In cases of epineural FN repair, with or without grafting, and a poor expected prognosis, an additional masseter-to-facial transfer is recommended. 3) FN transfer, mainly hypoglossal-to-facial and masseter-to facial, are usually chosen based on the surgeons' preference. The choice should be based on clinical factors. A combination of techniques improves the outcome in selected patients. 4) FN reconstruction following malignant tumors requires a combination of parotid and temporal bone surgery, involving different specialists. This collaboration is not always consistent. Exposure of the mastoid FN is recommended for lesions involving the stylomastoid foramen, as well as intraoperative FN reconstruction. 5) In patients with incomplete facial paralysis and a skull base tumor requiring additional surgery, consider an alternative reinnervation procedure, "take the FN out of the equation" before tumor resection. In summary, to achieve the best results in complex cases of facial paralysis, a multidisciplinary approach is recommended.

Facial paralysis: Clinical practice guideline of the Spanish Society of Otolaryngology. / Parálisis facial: guía de práctica clínica de la Sociedad Española de ORL.

Bell's palsy is the most common diagnosis associated with facial nerve weakness or paralysis. However, not all patients with facial paresis/paralysis have Bell's palsy. Other common causes include treatment of vestibular schwannoma, head and neck tumours, iatrogenic injuries, Herpes zoster, or trauma. The approach to each of these conditions varies widely. The purpose of this guideline is to provide clinicians with guidance on the treatment and monitoring of patients with different causes of facial paralysis. We intend to draft a practical guideline, focusing on operationalised recommendations deemed to be useful in the daily management of patients. This guideline was promoted by the Spanish Society of Otolaryngology and developed by a group of physicians with an interest in facial nerve disorders, including at least one physician from each Autonomous Community. In a question and answer format, it includes 56 relevant topics related to the facial nerve.

Biomarkers in Vestibular Schwannoma-Associated Hearing Loss.

Vestibular schwannomas (VSs) are benign tumors composed of differentiated neoplastic Schwann cells. They can be classified into two groups: sporadic VS and those associated with neurofibromatosis type 2 (NF2). VSs usually grow slowly, initially causing unilateral sensorineural hearing loss (HL) and tinnitus. These tumors cause HL both due to compression of the auditory nerve or the labyrinthine artery and due to the secretion of different substances potentially toxic to the inner ear or the cochlear nerve. As more and more patients are diagnosed and need to be managed, we are more than ever in need of searching for biomarkers associated with these tumors. Owing to an unknown toxic substance generated by the tumor, HL in VS may be linked to a high protein amount of perilymph. Previous studies have identified perilymph proteins correlated with tumor-associated HL, including µ-Crystallin (CRYM), low density lipoprotein receptor-related protein 2 (LRP2), immunoglobulin (Ig) γ-4 chain C region, Ig κ-chain C region, complement C3, and immunoglobulin heavy constant γ 3. Besides, the presence of specific subtypes of heat shock protein 70 has been suggested to be associated with preservation of residual hearing. It has been recently demonstrated that chemokine receptor-4 (CXCR4) is overexpressed in sporadic VS as well as in NF2 tumors and that hearing disability and CXCR4 expression may be correlated. Further, the genetic profile of VS and its relationship with poor hearing has also been studied, including DNA methylation, deregulated genes, growth factors, and NF2 gene mutations. The knowledge of biomarkers associated with VS would be of significant value to maximize outcomes of hearing preservation in these patients.

Basaloid squamous cell carcinoma: report of five cases.

OBJECTIVES: To document the clinical and histopathological characteristics of basaloid squamous cell carcinoma (BSCC). To add five new cases to the literature and compare them with another published series. CASES: retrospective review of five cases with the diagnosis of BSCC of the larynx. RESULTS: all the patients were male. They were heavy smokers and drinkers. Most of the tumours were supraglottic. Three patients presented with stage-IV disease and the other two with stage-I disease. Surgery supplemented with radiation was used in three patients, partial surgery was used in another case and radiation and associated chemotherapy in the other one. Eight neck dissections were performed, six of them were functional and the other two radical dissections. Two cases were found to have metastatic lymph nodes. There were not any distant metastases but two patients had tumoral relapse. CONCLUSIONS: BSCC has well defined histological features. Central comedonecrosis within the cells nests, cell with nuclear palisading and high-grade dysplasia in overlaying mucosa are the main characteristics.