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Vestibular schwannomas containing cystic parts are common, but it is extremely rare for a vestibular schwannoma to contain fluid-fluid levels. In this report, we present two cases of vestibular schwannoma with magnetic resonance imaging findings of a fluid-fluid level, and we discuss the radiological features and possible mechanism of fluid-fluid level formation. [ABSTRACT FROM AUTHOR]

Facial palsy is a rare presenting feature of cholesteatoma. Prompt treatment usually results in a good outcome, but if treatment is delayed the prognosis can be difficult to predict. We retrospectively reviewed 326 consecutive patients who had undergone temporal bone surgery for cholesteatoma. Eleven patients had presented with facial palsy, of whom eight had petrous apex involvement and three had disease confined to the middle-ear cleft. All patients with middle-ear disease were operated upon within two months of presentation, and all showed some recovery in facial function. The diagnosis had been delayed in all eight cases of apical disease, with four cases having had a long-standing total weakness. One case treated after seven months' partial weakness achieved a full recovery. In three cases of long-standing partial weakness, pre-operative facial function was preserved by maintaining the facial nerve in its normal anatomical location.Facial palsy associated with cholesteatoma should be treated as early as possible. Recovery can occur even if treatment is delayed for up to seven months. After this time, recovery is increasingly unlikely, but any residual function can be maintained and further deterioration prevented. [ABSTRACT FROM AUTHOR]

Purpose: Studies have assessed the trauma and change in hearing function from the use of otological drills on the ossicular chain, but not the effects of partial laser ablation of the incus. A study of the effectiveness of a novel middle-ear microphone for a cochlear implant, which required an incus recess for the microphone balltip, provided an opportunity to compare methods and inform a feasibility study of the microphone with patients., Methods: We used laser Doppler vibrometry with an insert earphone and probe microphone in 23 ears from 14 fresh-frozen cadavers to measure the equivalent noise level at the tympanic membrane that would have led to the same stapes velocity as the creation of the incus recess., Results: Drilling on the incus with a diamond burr created peak noise levels equivalent to 125.1-155.0 dB SPL at the tympanic membrane, whilst using the laser generated equivalent noise levels barely above the baseline level. The change in middle ear transfer function following drilling showed greater variability at high frequencies, but the change was not statistically significant in the three frequency bands tested., Conclusions: Whilst drilling resulted in substantially higher equivalent noise, we considered that the recess created by laser ablation was more likely to lead to movement of the microphone balltip, and therefore decrease performance or result in malfunction over time. For patients with greatly reduced residual hearing, the greater consistency from drilling the incus recess may outweigh the potential benefits of hearing preservation with laser ablation., (© 2022. The Author(s).)

Objectives: All commercially available cochlear implant (CI) systems use an external microphone and sound processor; however, external equipment carries lifestyle limitations. Although totally implantable devices using subcutaneous microphones have been developed, these are compromised by problems with soft tissue sound attenuation, feedback, and intrusive body noise. This in vivo pilot study evaluates a middle ear microphone (MEM) that aims to overcome these issues and compares hearing performance with that of an external CI microphone., Design: Six adult participants with an existing CI were implanted with a temporary MEM in the contralateral ear. Signals from the MEM were routed via a percutaneous plug and cable to the CI sound processor. Testing was performed in the CI microphone and MEM conditions using a range of audiometric assessments, which were repeated across four visits., Results: Performance of the MEM did not differ significantly from that of the CI on the assessments of Auditory Speech Sounds Evaluation loudness scaling at either 250 or 1000 Hz, or in the accuracy of repeating keywords presented at 70 dB. However, the MEM had significantly poorer aided sound-field thresholds, particularly at higher frequencies (≥4000 Hz), and significantly poorer performance on Arthur Boothroyd words presented at 55 dB, compared with the CI., Conclusion: In this pilot study, the MEM showed comparable performance to that of an external CI microphone across some audiometric assessments. However, performance with the MEM was poorer than the CI in soft-level speech (55 dB) and at higher frequencies. As such, the benefits of MEM need to be considered against the compromises in hearing performance. However, with future development, MEM is a potentially promising technology., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

Important research by Rosowski et al. [Twenty-Seventh Meeting of the Association for Research in Otolaryngology, 2004, p. 275] has led to a standard practice by the American Society for Testing Materials [West Conshohocken: ASTM International; 2014] to assess normal function of temporal bones used in the development of novel middle ear actuators and sensors. Rosowki et al. [Audiol Neurotol. 2007; 12(4): 265-76] have since suggested that the original criteria are too restrictive and have proposed modified criteria. We show that both the original and modified criteria are inappropriate for assessing individual temporal bones. Moreover, we suggest that both the original and modified Rosowski criteria should be applied with caution when assessing whether mean data from a study are within physiological norms because the multiple comparisons resulting from verification at each frequency will lead to very liberal rejection. The standard practice, however, has led to the collection of more extensive and consistent data. We suggest that it is now opportune to use these data to further modify the Rosowski criteria., (© 2019 S. Karger AG, Basel.)

Objective: To determine signs and symptoms for superior canal dehiscence syndrome caused by the superior petrosal sinus., Methods: A review of the English-language literature on PubMed and Embase databases was conducted, in addition to a multi-centre case series report., Results: The most common symptoms of 17 patients with superior petrosal sinus related superior canal dehiscence syndrome were: hearing loss (53 per cent), aural fullness (47 per cent), pulsatile tinnitus (41 per cent) and pressure-induced vertigo (41 per cent). The diagnosis was made by demonstration of the characteristic bony groove of the superior petrosal sinus and the 'cookie bite' out of the superior semicircular canal on computed tomography imaging., Conclusion: Pulsatile tinnitus, hearing loss, aural fullness and pressure-induced vertigo are the most common symptoms in superior petrosal sinus related superior canal dehiscence syndrome. Compared to superior canal dehiscence syndrome caused by the more common apical location of the dehiscence, pulsatile tinnitus and exercise-induced vertigo are more frequent, while sound-induced vertigo and autophony are less frequent. There is, however, considerable overlap between the two subtypes. The distinction cannot as yet be made on clinical signs and symptoms alone, and requires careful analysis of computed tomography imaging.

Objectives: This paper reports three cases of severe post-stapedectomy granuloma, emphasising the variable presentation of this devastating complication and the challenges of its management., Methods: A retrospective review was conducted of three cases of post-stapedectomy granuloma requiring surgical debulking between 2010 and 2015. Clinical symptoms, serial imaging, histopathology and post-operative outcomes were considered., Results: Intra-operatively, extensive granulation tissue with erosion of the otic capsule was found. There was spread along the VIIth and VIIIth cranial nerves to the cochlear nucleus in one patient. Post-operative clinical improvement was demonstrable, corroborated by diminution of contrast enhancement on serial magnetic resonance imaging. Facial nerve function recovered, tinnitus amelioration was variable and some otalgia persisted. Post-operative complications included grade IV facial weakness and late Pseudomonas aeruginosa meningitis, which all resolved., Conclusion: To the authors' knowledge, this paper reports the only case of post-stapedectomy granuloma tracking to the brainstem. Otalgia was present in all our cases, and may be deemed a red flag symptom of progressive bony destruction and otic capsule involvement. Although granuloma remains rare, it should be considered in any patient with worsening otological symptoms following stapes surgery.

Facial nerve palsy causes disfigurement with cosmetic, functional and psychological repercussions. The facial nerve can be affected anywhere along its course. A comprehensive assessment considering all differential diagnoses is critical to optimal management, as prompt, appropriate therapy leads to better outcomes.

This article discusses conservatively managed tumors, whether larger tumors at presentation are more likely to grow, and whether position at presentation corresponds with growth. A review is presented of more than 900 patients managed at Queen Elizabeth Hospital, Birmingham, between 1997 and 2012. Tumors were arbitrarily divided into 3 groups: intracanalicular (IC), and extracanalicular (EC) tumors measuring 1 to 10 mm or 11 to 20 mm at the cerebellopontine angle. This series shows that larger EC tumors grow faster than IC tumors and that EC tumors overall at presentation are more likely to grow than IC tumors., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Since the development of intratympanic aminoglycoside in the 1950s, otologists have been able to chemically ablate the vestibule. We present the results of using low-dose intratympanic gentamicin to treat Ménière's disease., Method: A retrospective review was performed of all patients who underwent low-dose intratympanic gentamicin therapy over seven years. Data on gender, age, number of procedures, pure tone audiometry and symptom control were analysed., Results: In all, 38 patients underwent low-dose intratympanic gentamicin therapy. These comprised 25 females and 13 males, with an average age of 58.4 years. Hearing was preserved in 87.5 per cent of patients, with no significant difference before and after treatment (p = 0.744). In all, 85.7 per cent of patients had complete or substantial symptom control (classes A and B, respectively)., Conclusion: Low-dose intratympanic gentamicin therapy was effective in controlling the symptoms of Ménière's disease patients, while preserving hearing.

Objective: The aim of this paper was to propose a guideline for the management of intrinsic facial nerve tumours based on our practice and findings in the literature., Method: A retrospective review of intrinsic facial nerve tumours over the last 15 years was performed. Parameters measured included age, presenting symptoms, pre- and post-treatment hearing and House-Brackmann grading, tumour position, treatment and duration of follow up., Results: A total of 15 patients presented with intrinsic facial nerve tumours over the study period. The most common presenting complaint was facial symptoms (93.3 per cent), followed by hearing loss (46.7 per cent). Three patients with stable facial nerve function (House-Brackmann grades II-III) were treated conservatively. Twelve patients underwent surgery to treat progressive or recurrent symptoms. Facial function was maintained or improved in 60.0 per cent of patients and hearing was preserved in 66.7 per cent., Conclusion: We propose that all stable tumours associated with good facial function of grade III or below should be treated conservatively. For symptomatic or progressive lesions, tailored surgery depending on the tumour site and hearing level should be offered to preserve native nerve function and facial musculature. For patients with prolonged paralysis, tumours can be monitored and other forms of facial reanimation and support offered.

Objective: This study investigated whether patients who remain symptomatic more than a year following idiopathic facial paralysis gain benefit from tailored facial physiotherapy., Methods: A two-year retrospective review was conducted of all symptomatic patients. Data collected included: age, gender, duration of symptoms, Sunnybrook facial grading system scores pre-treatment and at last visit, and duration of treatment., Results: The study comprised 22 patients (with a mean age of 50.5 years (range, 22-75 years)) who had been symptomatic for more than a year following idiopathic facial paralysis. The mean duration of symptoms was 45 months (range, 12-240 months). The mean duration of follow up was 10.4 months (range, 2-36 months). Prior to treatment, the mean Sunnybrook facial grading system score was 59 (standard deviation = 3.5); this had increased to 83 (standard deviation = 2.7) at the last visit, with an average improvement in score of 23 (standard deviation = 2.9). This increase was significant (p < 0.001)., Conclusion: Tailored facial therapy can improve facial grading scores in patients who remain symptomatic for prolonged periods.

Objective: To report a case of cochlear ossification as a result of neurosarcoidosis in a patient with bilateral profound sensorineural hearing loss., Study Design: Case report: Setting University teaching hospital, tertiary referral center., Patient: Forty-year-old man with neurosarcoidosis and bilateral profound sensorineural hearing loss. Intervention Unilateral cochlear implantation., Outcome Measure: Aided thresholds speech perception tests., Results: Marked improvement in hearing following implant., Conclusion: Patients with neurosarcoidosis are at risk of labyrinthitis ossificans. Early imaging of these patients is recommended and if early cochlear ossification is identified they should be offered rapid access to rehabilitation with a cochlear implant.

Aims: To evaluate non-auditory toxicity and local control after linear accelerator stereotactic radiosurgery (SRS) for the treatment of vestibular schwannomas., Materials and Methods: The institutional policy was to use SRS for radiologically progressing vestibular schwannomas. Case notes and plans were retrospectively reviewed for all patients undergoing SRS for vestibular schwannomas between September 2002 and June 2012. All patients were surgically immobilised using a BrainLab stereotactic head frame. The treatment plan was generated using BrainLab software (BrainScan 5.03). The aim was to deliver 12 Gy to the surface of the target with no margin. Patients with a minimum of 12 months of follow-up were included for toxicity and local control assessment. Radiological progression was defined as growth on imaging beyond 2 years of follow-up. Overall local control was defined in line with other series as absence of surgical salvage., Results: Ninety-nine patients were identified. Two patients were lost to follow-up. After a median follow-up interval of 2.4 years, the actuarial radiological progression-free survival at 3 years was 100% and overall local control was also 100%. However, two patients progressed radiologically at 3.3 and 4.5 years, respectively. Twenty-one of 97 (22%) evaluable patients suffered trigeminal toxicity and this was persistent in 8/97 (8%). Two of 97 (2%) suffered long-term facial nerve toxicity (one with associated radiological progression causing hemi-facial spasm alone). One of 97 (1%) required intervention for obstructive hydrocephalus. No statistically significant dosimetric relationship could be shown to cause trigeminal or facial nerve toxicity. However, 7/8 patients with persistent trigeminal nerve toxicity had tumours in contact with the trigeminal nerve., Conclusions: SRS delivering 12 Gy using a linear accelerator leads to high local control rates, but only prospective evaluation will fully establish short-term toxicity. In this study, persistent trigeminal toxicity occurred almost exclusively in patients whose tumour was in contact with the trigeminal nerve., (Crown Copyright © 2014. Published by Elsevier Ltd. All rights reserved.)

Objective: Rehabilitation of hearing is complicated in patients with profound bilateral hearing loss in the presence of sporadic vestibular schwannoma (VS) or neurofibromatosis 2 (NF2), especially if the tumor does not need to be removed. We present the outcome of patients who have had a cochlear implant in the tumor affected ear without removal of the primary tumor., Design: This is a retrospective multicentre study investigating outcomes of cochlear implantation in profoundly deaf patients with vestibular schwannoma in the implanted ear., Materials and Methods: Out of 11 implanted patients, 5 required no treatment for their tumor, whereas 6 had previously undergone radiotherapy. Nine patients experienced NF2, and 2 had unilateral VS in the only hearing ear. Postoperative hearing was assessed with open and closed set speech discrimination, including City University of New York (CUNY) in noise and Bamford, Kowal and Bench (BKB) sentence scores., Results: Patients with untreated lesions experienced marked improvement in their BKB and CUNY scores in the implanted ear and were daily cochlear implant users. The improvement was less consistent in the patients who had radiotherapy where only 1 patient attained open set speech discrimination., Conclusion: Patients with unilateral VS (sporadic or those affected with NF2) whose tumor status was stable, benefited from cochlear implantation in their tumor-affected ear. Patients who had radiotherapy also benefited from CI, but their outcomes were variable.

The nephrotoxicity and nephrocarcinogenicity of trichloroethylene (TCE) and tetrachloroethylene (PCE) are believed to be mediated primarily through the cysteine S-conjugate β-lyase-dependent bioactivation of the corresponding cysteine S-conjugate metabolites S-(1,2-dichlorovinyl)-l-cysteine (DCVC) and S-(1,2,2-trichlorovinyl)-l-cysteine (TCVC), respectively. DCVC and TCVC have previously been demonstrated to be mutagenic by the Ames Salmonella mutagenicity assay, and reduction in mutagenicity was observed upon treatment with the β-lyase inhibitor aminooxyacetic acid (AOAA). Because DCVC and TCVC can also be bioactivated through sulfoxidation to yield the potent nephrotoxicants S-(1,2-dichlorovinyl)-l-cysteine sulfoxide (DCVCS) and S-(1,2,2-trichlorovinyl)-l-cysteine sulfoxide (TCVCS), respectively, the mutagenic potential of these two sulfoxides was investigated using the Ames Salmonella typhimurium TA100 mutagenicity assay. The results show both DCVCS and TCVCS were mutagenic, and TCVCS exhibited 3-fold higher mutagenicity than DCVCS. However, DCVCS and TCVCS mutagenic activity was approximately 700-fold and 30-fold lower than DCVC and TCVC, respectively. DCVC and DCVCS appeared to induce toxicity in TA100, as evidenced by increased microcolony formation and decreased mutant frequency above threshold concentrations. TCVC and TCVCS were not toxic in TA100. The toxic effects of DCVC limited the sensitivity of TA100 to DCVC mutagenic effects and rendered it difficult to investigate the effects of AOAA on DCVC mutagenic activity. Collectively, these results suggest that DCVCS and TCVCS exerted a definite but weak mutagenicity in the TA100 strain. Therefore, despite their potent nephrotoxicity, DCVCS and TCVCS are not likely to play a major role in DCVC or TCVC mutagenicity in this strain., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

N-Acetyl-S-(1,2-dichlorovinyl)-L-cysteine (NA-DCVC) has been detected in the urine of humans exposed to trichloroethylene and its related sulfoxide, N-acetyl-S-(1,2-dichlorovinyl)-L-cysteine sulfoxide (NA-DCVCS), has been detected as hemoglobin adducts in blood of rats dosed with S-(1,2-dichlorovinyl)-L-cysteine (DCVC) or S-(1,2-dichlorovinyl)-L-cysteine sulfoxide (DCVCS). Because the in vivo nephrotoxicity of NA-DCVCS was unknown, in this study, male Sprague-Dawley rats were dosed (i.p.) with 230 μmol/kg b.w. NA-DCVCS or its potential precursors, DCVCS or NA-DCVC. At 24 h post treatment, rats given NA-DCVC or NA-DCVCS exhibited kidney lesions and effects on renal function distinct from those caused by DCVCS. NA-DCVC and NA-DCVCS primarily affected the cortico-medullary proximal tubules (S(2)-S(3) segments) while DCVCS primarily affected the outer cortical proximal tubules (S(1)-S(2) segments). When NA-DCVCS or DCVCS was incubated with GSH in phosphate buffer pH 7.4 at 37°C, the corresponding glutathione conjugates were detected, but NA-DCVC was not reactive with GSH. Because NA-DCVCS exhibited a longer half-life than DCVCS and addition of rat liver cytosol enhanced GSH conjugate formation, catalysis of GSH conjugate formation by the liver could explain the lower toxicity of NA-DCVCS in comparison with DCVCS. Collectively, these results provide clear evidence that NA-DCVCS formation could play a significant role in DCVC, NA-DCVC, and trichloroethylene nephrotoxicity. They also suggest a role for hepatic metabolism in the mechanism of NA-DCVC nephrotoxicity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Introduction: Reactive metabolite-mediated toxicity is frequently limited to the organ where the electrophilic metabolites are generated. Some reactive metabolites, however, might have the ability to translocate from their site of formation. This suggests that for these reactive metabolites, investigations into the role of organs other than the one directly affected could be relevant to understanding the mechanism of toxicity., Areas Covered: The authors discuss the physiological and biochemical factors that can enable reactive metabolites to cause toxicity in an organ distal from the site of generation. Furthermore, the authors present a case study which describes studies that demonstrate that S-(1,2-dichlorovinyl)-L-cysteine sulfoxide (DCVCS) and N-acetyl-S-(1,2-dichlorovinyl-L-cysteine sulfoxide (N-AcDCVCS), reactive metabolites of the known trichloroethylene metabolites S-(1,2-dichlorovinyl)-L-cysteine (DCVC), and N-acetyl-S-(1,2-dichlorovinyl)-L-cysteine (N-AcDCVC), are generated in the liver and translocate through the circulation to the kidney to cause nephrotoxicity., Expert Opinion: The ability of reactive metabolites to translocate could be important to consider when investigating mechanisms of toxicity. A mechanistic approach, similar to the one described for DCVCS and N-AcDCVCS, could be useful in determining the role of circulating reactive metabolites in extrahepatic toxicity of drugs and other chemicals. If this is the case, intervention strategies that would not otherwise be feasible might be effective for reducing extrahepatic toxicity.

Objectives: To determine the implications of a functional approach to vestibular schwannoma surgery, with facial nerve function prioritised higher than total tumour excision., Study Design: A case-control study in a tertiary referral neurotology clinic., Patients: A 'functional' surgical group treated after April 2007 (n = 44, mean cerebellopontine angle dimension 27 mm), and an 'excisional' surgical group matched for tumour size, treated from 1997 to April 2007 (n = 115)., Intervention: Change to more functional surgical approach., Primary Outcome: facial nerve status. Secondary outcome: tumour recurrence in less-than-total tumour excision., Results: Facial nerve preservation: 77 per cent House-Brackmann grade I-II in functional group at 12 months, versus 57 per cent grade I-II in excisional group (p = 0.027). Tumour recurrence: 1 per cent in total excision group, 2 per cent in near-total group and 40 per cent in sub-total group., Conclusion: A functional approach to vestibular schwannoma surgery improves facial nerve preservation outcomes and reduces the requirement for facial nerve rehabilitative interventions. Tumour recurrence rates are low in near-totally excised lesions but significant if only sub-total excision is achieved.

S-(1,2-Dichlorovinyl)-L-cysteine sulfoxide (DCVCS) is a reactive and potent nephrotoxic metabolite of the human trichloroethylene metabolite S-(1,2-dichlorovinyl)-L-cysteine (DCVC). Because DCVCS covalent binding to kidney proteins likely plays a role in its nephrotoxicity, in this study biotin-tagged DCVCS, N-biotinyl-DCVCS (NB-DCVCS), was synthesized, and its stability in buffer alone and in the presence of rat blood or plasma was characterized in vitro. In addition, reactivity toward GSH and covalent binding to selected model enzymes and isolated kidney proteins were characterized. The half-lives of NB-DCVCS (39.6 min) and the DCVCS (diastereomer 1, 14.4 min; diastereomer 2, 6 min) in the presence of GSH were comparable. Incubating the model enzymes glutathione reductase and malate dehydrogenase with 10 μM NB-DCVCS for 3 h at 37 °C followed by immunoblotting using antibiotin antibodies demonstrated that glutathione reductase and malate dehydrogenase were extensively modified by NB-DCVCS. When rat kidney cytosol (6 μg/μL) was incubated with NB-DCVCS (312.5 nM to 5 μM) for 3 h at 37 °C followed by immunoblotting, a concentration-dependent increase in signal with multiple proteins with different molecular weights was observed, suggesting that NB-DCVCS binds to multiple kidney proteins with different selectivity. Incubating rat kidney cytosol with DCVCS (10-100 μM) prior to the addition of NB-DCVCS (2.5 μM) reduced the immunoblotting signal, suggesting that NB-DCVCS and DCVCS compete for the same binding sites. A comparison of the stability of NB-DCVCS and DCVCS in rat blood and plasma was determined in vitro, and NB-DCVCS exhibited higher stability than DCVCS in both media. Collectively, these results suggest that NB-DCVCS shows sufficient stability, reactivity, and selectivity to warrant further investigations into its possible use as a tool for future characterization of the role of covalent modification of renal proteins by DCVCS in nephrotoxicity.

We report the case of a 58-year old man who presented with a 4 cm right acoustic neuroma. He underwent a translabyrinthine resection. Two years later he presented with multiple strokes and progressive generalised deterioration. A cerebral angiogram demonstrated an extensive right side cerebellar dural arteriovenous fistula with retrograde flow causing corticovenous reflux. The fistula was treated successfully endovascularly. There is only one published report of a dural arteriovenous fistula occurring following acoustic neuromas surgery. The pathogenesis and management of this unusual complication is discussed.

The standard current treatment of an otogenic brain abscess is drainage via neurosurgical burr hole or complete excision, followed by an otological procedure to eradicate the primary pathology – often at a later date. We describe the drainage of otogenic brain abscess via a transtemporal approach. We present a retrospective study of six cases, five children and one adult. All the children had acute middle ear disease while the adult patient had a petrous apex cholesteatoma. All cases had an otogenic intracranial abscess either in the posterior cranial fossa or in both posterior and middle cranial fossae. Diagnosis was confirmed by computed axial tomography (CT) scan. All the patients were treated by mastoidectomy and needle aspiration to drain the abscesses. In all cases the brain abscess and the ear pathology were successfully treated by a single stage transmastoid approach. The five paediatric patients had an extended cortical mastoidectomy approach to both intracranial pathology and ear disease except one patient who required a burr hole to drain a posteriorly located subdural posterior fossa abscess. The adult patient underwent petrosectomy, followed by transtemporal abscess drainage. There was postoperative pus recollection in one patient who required further aspiration. We conclude that transtemporal drainage of an otogenic brain abscess can successfully treat otological and intracranial pathology in a single operation. It has a low complication rate and avoids the need for a craniotomy or subsequent operations.

Objectives: To assess the efficacy of the bone-anchored hearing aid (BAHA) in the rehabilitation of single-sided deafness (SSD)., Study Design: Retrospective case-control series review., Setting: Tertiary referral unit., Patients: Fifty-eight consecutive patients that had a bone-anchored hearing aid for single-sided deafness completed outcome questionnaires, building upon earlier audiological assessment of 19 patients. Single-sided deafness controls (n = 49) were mainly acoustic neuroma patients., Main Outcome Measurements: speech discrimination testing in directional noise, speech and spatial qualities of hearing questionnaire and the Glasgow Benefit Inventory (GBI)., Results: The mean follow-up time was 28.4 months. Five (13%) of the bone-anchored hearing aid patients were non-users because of lack of benefit. The audiometric testing confirmed that when noise was on the bone-anchored hearing aid side speech perception was reduced but benefited when noise was on the side of the hearing ear. There was no difference between the Speech and Spatial Qualities of Hearing Scores in bone-anchored hearing aid users and controls. In particular there was no difference in the spatial subscores. In the bone-anchored hearing aid users the median Glasgow Benefit Inventory score was 11. If the non-users are included then 13 (22%) patients had no or detrimental (negative) Benefit scores. No or negative benefit scores were more frequent in those deaf for <10 years. In open-field questions, patients felt the bone-anchored hearing aid was most useful in small groups or in 'one-to-one' conversation., Conclusions: Bone-anchored hearing aid rehabilitation for single-sided deafness is less successful than for other indications, reflected here by relatively low median Glasgow Benefit Inventory scores. There was also no significant difference between controls and bone-anchored hearing aid users in the Speech and Spatial Qualities of Hearing Questionnaire. Patients with a longer duration of deafness report greater subjective benefit than those more recently deafened, perhaps due to differing expectations.

Introduction: Paragangliomas are rare tumours arising from the paraganglia of the autonomic nervous system., Case Report: We present a case of a paraganglioma arising from the hypoglossal nerve and producing an unusual clinical picture at presentation., Discussion: We supply radiological evidence of a paraganglioma originating from the hypoglossal nerve, and thus extend the evidence base for this rare site of origin. Our patient presented as an emergency with long tract neurological symptoms and progressive brainstem involvement. This presentation is not characteristic of paragangliomas in general, which usually have an indolent growth pattern and often demonstrate benign symptoms for a number of years prior to diagnosis. The location of a hypoglossal paraganglioma differs significantly from more common paragangliomas described in the neck and skull base, and this should inform the surgical approach undertaken.

Glomus tumours are rare vascular tumours arising within the lateral skull base, infratemporal fossa and upper neck. When they are confined to the middle ear cleft, they are termed glomus tympanicum tumours. Traditionally tympanicum tumours have been treated surgically and their removal has generally been regarded as quite straightforward. Our experience with these lesions however, is that they fall into two distinct categories, those with and those without hypotympanic involvement. This distinction can be determined by high resolution CT scanning in coronal and axial planes. Mesotympanic tumours are indeed typically straightforward in their removal and can be approached by a transcanal route. Hypotympanic extension requires additional evaluation by MRI, and a more extensive surgical approach. In this paper, we present our management approach based on a series of nine cases. We conclude that determining the tumour extension preoperatively by using modern imaging techniques is essential in planning the surgery.

Objectives: To determine whether patients with small to medium sized acoustic neuromas managed conservatively suffer reduced rates of facial nerve and hearing preservation when compared with patients undergoing primary surgery., Design: An intention-to-treat comparison between patients managed conservatively at first presentation and those managed with primary surgery., Setting: Tertiary referral neurotological centre in Birmingham, UK., Participants: Data were drawn from our database of 487 sporadic acoustic neuromas managed from 1997 to present day., Main Outcome Measures: Facial nerve status (assessed using the House-Brackmann system) was collated for all conservatively managed patients (n = 167) and compared with that expected when calculated from primarily surgically managed tumours of equivalent size (n = 121). A chi-square test was employed to test the statistical significance of any difference. Hearing preservation (maintenance of AAO-HNS Class) in patients presenting with Class A or B hearing was compared between conservatively managed patients and those with primary surgical management., Results: Observed facial nerve preservation in conservatively managed patients was significantly better (P < 0.001) than expected when calculated from rates of facial nerve preservation in surgically managed patients. Hearing preservation was also significantly more successful in conservatively managed patients (Pearson chi-square: P < 0.000)., Conclusions: An initial period of conservative management is a safe and reasonable management policy in all acoustic neuromas up to 2 cm in size at the cerebello-pontine angle. Given that there are no failsafe ways of deciding potential for growth in acoustic neuromas, initial conservative management of these tumours offers superior hearing and facial nerve preservation when compared with primary surgical treatment.

Familial head and neck paragangliomas account for approximately 10% of all head and neck paragangliomas. There are three known genes associated with genetic susceptibility to head and neck paragangliomas: succinate dehydrogenase complex subunit 'D', 'B' and 'C' (SDHD, SDHB and SDHC). The genes most frequently implicated: SDHD and SDHB, also predispose to phaeochromocytoma. SDHD shows a complex inheritance pattern - tumours do not develop if the mutation is inherited from the mother. SDHB mutations are associated with malignant phaeochromocytoma. Patients who present with a family history of paraganglioma or phaeochromocytoma, with multiple tumours, or early onset tumours (<50 years), should be referred for genetic investigation.

Cholesterol granulomas of the middle ear tend to be benign in nature, frequently seen by otologists during cholesteatoma or chronic otitis media surgery and easily dealt with by evacuation. In contrast, cholesterol granulomas of the petrous apex appear to have a more aggressive nature and present significant pathological threats. We present a case of a large destructive cholesterol granuloma of the middle ear eroding the middle and posterior cranial fossa bone and dura to exert a mass effect upon the temporal lobe. We emphasize the destructive potential of this extremely rare middle-ear cholesterol granuloma, and draw attention to the distinction between this variant and the commonplace and benign form of the lesion more usually found at this site. We also present the current theories of the aetiology of cholesterol granuloma formation with possible explanations for the rare aggressive behaviour.

Objectives: Following translabyrinthine craniotomy the temporal bone defect is commonly obliterated using a free autologous fat graft. In this series the dura was put back in place but not closed primarily. As the fat graft remains in direct contact with the cerebro spinal fluid (CSF) there is potential for dispersal of fat within the CSF space. This paper aims to determine the frequency of such CSF fat dissemination and its clinical significance., Design: A retrospective review of translabyrinthine acoustic neuroma removal with free fat autograft obliteration of the temporal bone defect between the years 1997 and 2000., Setting: Tertiary referral oto-neurosurgical centre. Postoperative magnetic resonance (MR) imaging., Participants: All translabyrinthine patients who had postoperative MR imaging were included. Twenty-six cases were identified. Age range was 13-70 years. Fourteen were male patients., Main Outcome Measures: Evidence of CSF fat dissemination on MR and patients' clinical findings., Results: Twenty-two of the 26 scans (85%) demonstrated evidence of fat dissemination into the subarachnoid CSF spaces in the form of microemboli. The cerebellopontine angle was the most common site involved. No evidence of ventricular dilation or any other abnormality was noted. There was no relationship between the presence or extent of fat microembolization and the patients' clinical course., Conclusions: This study suggests that free fat placed in temporal bone defects commonly migrate into the subarachnoid space and subsequently move around in these spaces. This is not associated with any complications such as hydrocephalus, meningitis or prolonged postoperative headache.

Objective: To determine the access to and the use of the Internet by acoustic neuroma patients as a medical information source, and the influence of the Internet on decision-making, as well as the patients' views on the quality of particular sites., Materials and Methods: A retrospective 27-item questionnaire was sent to 138 consecutive acoustic neuroma patients diagnosed between 1997 and 2002. The study included patients treated by conservative, radiotherapeutic and surgical modalities., Main Outcome Measures: Patient demographics, the extent of Internet use as well as quality and quantity of available information., Results: There were 86.95 per cent questionnaires available for analysis. Twenty-four per cent accessed the Internet to obtain information about acoustic neuromas. Ninety-seven per cent of patients said that the information provided in the clinic matched with the information obtained from the websites, 49 per cent said that the information obtained from the Internet did not influence decision making regarding acceptance of treatment of their tumour. Some patients said the Internet information was depressing, devastating, and they would never look up the Internet again for medical information., Conclusion: A significant proportion of our patients accessed the Internet for information. Caution should be exercised when advising patients regarding websites as the information on the Internet is often not controlled or checked for accuracy. Acoustic neuroma specialists and ENT surgeons in general should familiarize themselves with related websites so that patients can then be directed to high-quality sites.

Higher surgical training in the UK faces a cut of two years. We conducted a questionnaire survey to assess the operative experience of current higher surgical trainees in otological surgery and the likely effect of the proposed reduction from six to four years. 91 (65%) of the 142 higher surgical trainees responded with details of major otological procedures performed (independently or assisting) over one year. In the present six-year scheme a typical trainee performs 72 myringoplasties, 79 mastoidectomies, 7 skull base procedures and 28 other procedures. In the first four years, however, his or her experience is only 39 myringoplasties, 44 mastoidectomies, 4 skull base procedures and 7 others. The large shortfall in experience that might result from shortening of the training programme would need to be met by intensification of the training or institution of accredited otology fellowships. Very similar dilemmas are faced by other surgical specialties.

Arachnoid cysts can occur at different intracranial sites, including the cerebellopontine angle (CPA). The incidence of arachnoid cysts is 1 per cent of all intracranial lesions. Recent advances in MRI (magnetic resonance imaging) scan techniques have led to CPA arachnoid cysts being more frequently diagnosed and with a higher degree of certainty. The need for further understanding of their natural history as well as for the development of a management rationale has been highlighted with this increased rate of diagnosis. We present a series of five adult patients with different clinical presentations attributed to CPA arachnoid cysts. These lesions have a characteristic location in the posterior-inferior aspect of the CPA below the facial and vestibulocochlear nerves. These cysts did not show change in size on repeated MRI scan and the patients' symptoms did not progress over the period of follow up. Our findings would support a conservative management approach to the majority of these cysts.

Bell's palsy or idiopathic facial palsy is the commonest cause of unilateral lower motor neuron facial palsy. Misdiagnosis of facial nerve palsy as Bell's palsy is still seen in clinical practice. The clinician should always consider the possibility of a potentially serious underlying pathology before making the diagnosis of Bell's palsy. We present a series of 13 patients referred to our ENT department with an initial diagnosis of Bell's palsy. Further clinical examination and investigation revealed the underlying cause. Many had additional symptoms and signs related to the ear. In all patients with unilateral facial palsy a detailed history should be taken and thorough clinical examination carried out. Where no recovery occurs within the expected time period further radiological investigations such as computerized axial tomography (CT) and magnetic resonance imaging (MRI) should be performed. Current scanning techniques provide good quality images, which can show occult lesions of the temporal bone, internal acoustic canal and/or cerebellopontine angle. Radiologists with a special interest and experience in otoneurological radiology should ideally report these images, and a close co-operation between ENT surgeon and radiologist is essential in arriving at a proper diagnosis.

A prospective analysis of patients referred by Otolaryngologists from a tertiary hospital for detailed assessment of the posterior cranial fossa was undertaken. The objectives were to evaluate radiological characteristics of the anterior inferior cerebellar artery (AICA) within the cerebellopontine angle (CPA) and the internal auditory canal (IAC), and to correlate these characteristics with ipsilateral auditory symptoms. Three hundred and thirty-two consecutive adult patients who presented with unilateral auditory symptoms were studied. All patients were referred by the Department of Otolaryngology at City Hospital, Birmingham, from October 1999 to October 2001. Magnetic resonance imaging (MRI) with three-dimensional Fourier Transformation Constructive Interference in Steady State (3D FT-CISS) was the imaging strategy used to investigate each patient. Six hundred and sixty-four sides were studied and the AICA loop was identified in all patients. Using a simple anatomical classification to type the loops: there were 412 type I loops (within the CPA), 202 type II loops (at the porus acousticus, extending up to 50% of IAC) and 50 type III loops (extending beyond 50% of IAC). There was a statistically significant association with unilateral hearing loss and type II and III AICA loops (P = 0.016 and P = 0.006 respectively). An association between the presence of a large diameter vascular loop in the CPA and unilateral auditory symptoms was not found to be significant (P > 0.05).

In pharyngeal pouch surgery, the relatively new technique of endoscopic stapling diverticulotomy has a number of advantages over more traditional surgical treatments, such as Dohlman's procedure and open pouch excision, and now seems to be the procedure of choice. However, a number of iatrogenic perforations and deaths have been reported with this procedure. We present three cases of iatrogenic perforations occurring during endoscopic stapling of a pharyngeal pouch by different surgeons in our unit, and review the management, causes and prevention of this potentially life-threatening complication.

The current incidence of missile injury to the temporal bone (MITB) is very low in the United Kingdom. However, the increasing frequency of firearm violence in Britain suggests a greater risk of occurrence. This, along with the devastating potential sequelae of MITB (facial palsy, dead ear, intracranial damage, major vascular injury and cosmetic disfigurement), requires otolaryngologists to be conversant with all aspects of their management. The risk of major complications is much higher with MITB than with temporal bone injury following blunt trauma, and surgical management is, therefore, much more common. We present one such case, and review the literature outlining the pathogenesis, clinical features, and recommended management.

Dermoid cysts are rare benign tumours, they represent the simplest form of teratoma. Approximately seven per cent affect the head and neck region, within this region they are frequently encountered in the area of the lateral eyebrow, the orbit and the nose. A case of a 17-year-old girl who developed a rapidly growing facial swelling due to an infratemporal fossa dermoid cyst is presented. A review of the literature using Medline has not revealed any previous reports of similar cases. The lesion was completely excised using a lateral approach to the infratemporal fossa.

Objective: Intracranial epidermoids (cholesteatomas) mimic arachnoid cysts in their radiologic characteristics, especially in the cerebellopontine angle. It is essential to differentiate the two conditions because they warrant different therapeutic interventions. The objective of this study is to elucidate the different radiologic characteristics of the conditions., Study Design and Setting: This was a retrospective study of 4 patients referred for a differential diagnosis and management of intracranial cystic lesions to the Departments of Neurotology/Neurosurgery and Neuroradiology in a tertiary referral university hospital., Patients: Four patients of different age groups with cystic intracranial lesions, diagnosed epidermoid or arachnoid cysts, were chosen. A retrospective analysis of their case charts, radiologic and surgical interventions, and follow-up records was undertaken., Methods: The imaging techniques used included computerized tomographic scans, magnetic resonance imaging (MRI) with T1, T2, proton-density, and gadolinium-enhanced T1 images. In addition, special MRI sequences were used that included fluid-attenuated inversion recovery and echo planar diffusion scanning. All the patients underwent an audiovestibular evaluation., Results: Both lesions are characteristically well demarcated and have a homogeneous low density, similar to cerebrospinal fluid on computerized tomographic scan, showing no contrast enhancement. On MRI, epidermoids and arachnoid cysts usually appear hypointense on T1-weighted images and hyperintense on T2-weighted images. On fluid-attenuated inversion recovery, an arachnoid cyst tends to follow cerebrospinal fluid intensity, whereas an epidermoid becomes hyperintense. There are occasions when an epidermoid may appear as a low-intensity lesion on fluid-attenuated inversion recovery. This dilemma is resolved with the use of echo planar diffusion scanning, on which an epidermoid remains bright., Conclusion: The authors recommend the use of fluid-attenuated inversion recovery and diffusion sequence MRI when definitive radiologic diagnosis of cystic intracranial lesions becomes difficult with routine computerized tomographic scanning and MRI.

The purpose of this questionnaire study was to evaluate the existing knowledge of binaural hearing and the attitudes and practices of prescribing bilateral hearing aids amongst otolaryngologists in the United Kingdom. Of the 950 questionnaires sent to the current members of the British Association of Otolaryngologists and Head and Neck Surgeons (BAO-HNS), there were 591 respondents (62 per cent). The true response rate with completed questionnaires was 59 per cent. Eighty-one per cent of the respondents were aware of the importance of binaural hearing and had a positive attitude towards binaural fitting. The practice of bilateral hearing aid prescriptions was found to be poor amongst all grades on the NHS (less than 10 per cent of all hearing aid prescriptions). This practice in the private sector was variable, dependent largely on patient preference and affordability. The practice of binaural prescription was higher for patients in the paediatric age group than amongst adults. Two common indications for hearing aid prescriptions for unilateral deafness were otitis media with effusion in children (23 per cent of respondents) and for tinnitus masking in adults (12 per cent of respondents). Many otolaryngologists believed that there was not enough evidence to support bilateral bone-anchored hearing aid implantation and bilateral cochlear implantation. Ninety-four per cent of the respondents believed that binaural hearing was as important as binocular vision.