Case Report: Recurrent pediatric cavernous malformation of the trigeminal nerve.

Background: Most cavernous malformations (CM) usually involve the parenchyma and rarely occur in cranial nerves. Recurrence of CM associated with cranial nerves after surgical resection has not been previously reported. Case description: This paper describes the case of an 11-year-old girl who presented with left otalgia and headache because of a left trigeminal cavernous malformation. She underwent radical resection via a left retrosigmoid approach while sparing the trigeminal nerve. Surveillance imaging at 18 months demonstrated recurrence along the length of the trigeminal nerve into Meckel's cave with significant extension into the middle cerebellar peduncle. Subsequent re-operation via an extended middle fossa approach with anterior petrosectomy enabled complete resection with division of the trigeminal nerve. Postoperatively, she had a transient left facial paresis, and right hemiparesis that resolved within 48 h. Conclusion: This case highlights the importance of close postoperative surveillance in CM associated with cranial nerves as recurrence after nerve-sparing resection is possible. Surgical treatment due to the morphology of significant recurrence required the use of a complex skull base approach through a new corridor to achieve optimal clinical outcome.

The potential effect of the updated national criteria on adult cochlear implantation in England and Wales.

OBJECTIVES: Following an update of 'old' TA166 guidance (March 2019), the National Institute for Health and Care Excellence (NICE) predicted a 70% increase in cochlear implantation (CI). We investigated the effect of the 'new' TA566 criteria on adult CI at our regional centre. METHODS: Adult CI assessments between 1st January 2015 and 31st December 2018 (before new criteria) and between 7th March 2019 and 31st August 2019 (after new criteria) were retrospectively examined. Calculated eligibility and uptake rates predicted change in annual implantation under the new guidance. RESULTS: 552 patients were identified in the first study period, with a median of 148 assessments per year. Of 533 with complete assessments, 58% were eligible, of whom 74% were implanted. Retrospective application of the new criteria was possible for 277 patients; eligibility and uptake were 67% and 80%, respectively. In the second study period (n = 60), new criteria eligibility was 62% and uptake was 78%. Increased eligibility under the new criteria predicts an increase of between 13 and 23% in annual adult implantation. CONCLUSIONS: We demonstrate increased implant eligibility under the latest NICE guidance. Assuming consistent referral patterns, our predicted increase in adult implantation is considerably less than that predicted elsewhere.

Phantosmia as the first presentation of a cavernous sinus - clinoidal meningioma.

Cavernous sinus meningiomas (CSM) are complex skull base lesions that, due to their particular anatomical location, render surgical management difficult. Their symptomatology is versatile, and the clinical outcome is difficult to predict. We present the case of a 57-year old female patient who experienced phantosmia - an abnormal, persistent, olfactory sensation of cigarette smell for 18 months. MRI was performed and revealed a left cavernous sinus meningioma, extending into the left temporal fossa, with olfactory and optic nerve distortion. To our knowledge, this is the first reported case of phantosmia as the initial presentation of a lesion in the cavernous sinus.

Giant Juvenile Ectopic Schwannoma of the Temporal Bone.

BACKGROUND: Ectopic intracranial schwannomas (those that do not arise from a named cranial nerve) are rare. They account for <2% of surgically resected central nervous system schwannomas. CASE DESCRIPTION: We report the case of a 14-year-old boy presenting with a left conductive hearing loss and temporal bone deformity. No facial or cranial nerve deficits were present. Cross-sectional imaging demonstrated a large expansile extra-axial temporal bone mass, extending into and distorting the middle cranial fossa. At surgical resection the tumor was functionally and anatomically distinct from the facial nerve or any other identifiable neural structure within the middle ear or temporal bone. Histology confirmed a World Health Organization grade 1 schwannoma. CONCLUSIONS: This is the first reported case of a giant juvenile ectopic schwannoma within the temporal bone.

A Systematic Review of Interventions for Balance Dysfunction in Patients With Vestibular Schwannoma.

OBJECTIVE: Dizziness is a major contributing factor to poor quality of life for patients with vestibular schwannoma (acoustic neuroma). We wished to review the literature on interventions for balance dysfunction in these patients. DATA SOURCES: A systematic literature review was performed identifying studies that measured balance function before and after treatment for vestibular schwannoma. Data sources include Medline (1950-present), EMBASE (1974-present), Cochrane Library (issue 3, 2008), NHS Centre of reviews and dissemination, Clinical Evidence, Cochrane central register of controlled trial, and CINAHL. STUDY SELECTION: A minimum follow-up of 6 months was required, to estimate long-term balance function. Eight articles were identified, including five studies with surgical intervention, two studies with stereotactic radiotherapy, and one comparing the two. Study design was generally poor with a high risk of bias. These studies all utilized the Dizziness Handicap Inventory (DHI) as a measure of pre- and postintervention balance function. DATA EXTRACTION: Results showed that overall DHI scores are not statistically affected by intervention irrespective of modality (surgery or stereotactic radiotherapy). Patients selected with severe dizziness, who undergo surgery, improved postoperatively. No other studies for severe dizziness were noted for comparison. CONCLUSIONS: Age, sex, and tumor size have no statistically significant effect on DHI outcomes, and no evidence to suggest which treatment modality has better dizziness related outcomes.No specific treatment modality was superior in terms of long-term balance function. Patients with severe dizziness may benefit from surgery, although no comparator studies were identified.

The Cochlear Baha 4 Attract System - design concepts, surgical technique and early clinical results.

Bone conduction implant systems utilize osseointegrated fixtures to transmit sound through the bones of the skull. They allow patients with hearing loss to receive acoustic signals directly to the inner ear, bypassing the outer and middle ear. The new Cochlear™ Baha(®) Attract System (Cochlear Bone Anchored Solutions AB, Mölnlycke, Sweden) has been designed as a non-skin penetration hearing implant. The system uses magnetic coupling to hold the external sound processor in place and transmit acoustic energy. An implantable magnet is anchored to the skull via a single osseointegrated fixture, maximizing the efficiency of energy sound transfer. The interposed soft tissue is protected by a SoftWear pad that evenly distributes pressure in order to minimize the risk of pressure necrosis. This article summarizes the design features and early clinical results of the Baha 4 Attract System and provides context as to its place in the broader hearing aid market.

The role of post-operative imaging in cochlear implant surgery: a review of 220 adult cases.

OBJECTIVES: To determine the incidence of abnormal radiological findings after cochlear implantation and their effect on clinical outcomes. METHODS: Retrospective review of 220 adult cochlear implants. Clinical records and post-operative plain X-rays were reviewed and compared with pre-operative and 6-month post-operative City University of New York (CUNY) speech scores. RESULTS: There were no cases of extra-cochlear array misplacement. Imaging showed 20 cases of incomplete array insertion (9.2%), 3 cases of kinking of the array (1.4%), 2 cases of tip rollover (0.9%), and 1 case of apparent array fracture (0.5%). Patient management was not altered by abnormal imaging. Patients with abnormal radiological findings had slightly minor improvements (median 39 vs. 56%) in City University of New York (CUNY) speech discrimination scores at 6 months (Mann-Whitney U test, P = 0.043). CONCLUSION: All abnormalities on post-operative imaging were minor and did not alter patient management. The future role of post-operative imaging is discussed.

What factors influence patient and parent choice of cochlear implant model for children?

OBJECTIVES: The West of England Cochlear Implant Programme purchases two makes of cochlear implant (CI) for paediatric use (MED-EL and Cochlear). If the CI team has no preference, the decision regarding which implant to use is made by the patient and family. Families are provided with information about the devices and allowed time to handle dummy implants and ask questions. The aim of this study is to establish how patients make this choice and which factors are considered most important in the decision-making process. METHOD: Patients who received a CI within the past 4 years were sent a postal survey, with reminders issued when patients attended for checkups. Patients were asked to rate certain factors from 0 to 10 depending on their importance in the decision-making process. RESULTS: Sixty-four patients replied (response rate 74%). In most cases (83%), the parents and/or children were involved in the decision regarding the choice of implant. Eighty-nine percent of patients received information about the choices of CI from the CI team. Patients also accessed information directly from the manufacturer, from other CI users, and from websites. The most important factor in choosing CI model was robustness and reliability (mean score 9.6), followed by comfort (9.4), size/shape (9.2), and control system/ease of use (8.9). All patients were happy with the choices they made. DISCUSSION: In this study, most patients undergoing cochlear implantation were offered a choice of model. Robustness, reliability, comfort, and size/shape of CI are considered the most important factors in this decision.

Systematic review of intralesional cidofovir dosing regimens in the treatment of recurrent respiratory papillomatosis.

OBJECTIVE: Surgical management of recurrent respiratory papillomatosis (RRP) usually involves resection via microlaryngoscopy. Intralesional injection of cidofovir has been shown to be an effective adjuvant treatment, but remains unlicensed. United Kingdom General Medical Council guidelines recommend the prescribing doctor should "be satisfied that there is a sufficient evidence base and/or experience of using the medicine to demonstrate its safety and efficacy". This study reviews the published dosing regimens of intralesional cidofovir in the treatment of RRP in order to provide a precedent for those that wish to prescribe it. METHODS: A systematic review of the literature was undertaken using Medline, EMBASE and CINAHL. Articles describing the use of intralesional cidofovir for RRP were reviewed. Information regarding cidofovir concentration, volume, total dose, number of treatments, interval between treatments, overall treatment period and follow up was extracted. RESULTS: Fifty-one articles were identified. Concentration of cidofovir injected ranged from 0.0001mg/ml to 37.5mg/ml, with 5 to 7.5mg/ml being the most common. The volume of cidofovir solution injection ranged from 0.1 to 20ml. The total dose per injection ranged from 0.15 to 105mg. There was wide variation in dosing regimens with different intervals between endoscopies, number of injections and total doses delivered. CONCLUSIONS: Based on this published literature, the precedent for prescribing intralesional cidofovir supports a concentration of 5 to 7.5mg/ml. Volumes up to 5ml per injection are routinely used. Total dose and frequency of cidofovir administration is highly variable. The need for repeat doses of cidofovir should be judged on an individual basis.

How private is your consultation? Acoustic and audiological measures of speech privacy in the otolaryngology clinic.

The right to confidentiality is a central tenet of the doctor-patient relationship. In the United Kingdom this right to confidentiality is recognised in published GMC guidance. In USA the Healthcare Insurance Portability and Accountability Act of 1996 (HIPAA) strengthened the legal requirement to protect patient information in all forms and failure to do so now constitutes a federal offence. The aims of this study are to assess the acoustic privacy of an otolaryngology outpatient consultation room. Acoustic privacy was measured using the articulation index (AI) and Bamford-Kowal-Bench (BKB) speech discrimination tests. BKB speech tests were calibrated to normal conversational volume (50 dB SPL). Both AI and BKB were calculated in four positions around the ENT clinic: within the consultation room, outside the consulting room door, in the nearest waiting area chair and in the farthest waiting area chair. Tests were undertaken with the clinic room door closed and open to assess the effect on privacy. With the clinic room door closed, mean BKB scores in nearest and farthest waiting area chairs were 51 and 41% respectively. AI scores in the waiting area chairs were 0.03 and 0.02. With the clinic room door open, privacy was lost in both AI and BKB testing, with almost 100% of word discernable at normal talking levels. The results of this study highlight the poor level of speech privacy within a standard ENT outpatient department. AI is a poor predictor or privacy.