Superior petrosal sinus causing superior canal dehiscence syndrome.

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.

Post-stapedectomy granuloma: a devastating complication.

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.

Is low-dose intratympanic gentamicin an effective treatment for Ménière's disease: the Birmingham experience.

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.

Intrinsic facial nerve tumours of the temporal bone: a proposed management guideline.

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.

Outcome of facial physiotherapy in patients with prolonged idiopathic facial palsy.

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.

Linear accelerator stereotactic radiosurgery for vestibular schwannomas: a UK series.

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.

Facial nerve outcomes in functional vestibular schwannoma surgery: less than total tumour excision significantly improves results.

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.

The bone-anchored hearing aid in the rehabilitation of single-sided deafness: experience with 58 patients.

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.

A case of paraganglioma of the hypoglossal nerve.

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.

The crucial role of imaging in determining the approach to glomus tympanicum tumours.

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.

Conservative versus primary surgical treatment of acoustic neuromas: a comparison of rates of facial nerve and hearing preservation.

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.

The genetics of paragangliomas: a review.

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.

Vestibular schwannomas with fluid-fluid level.

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.

Facial palsy secondary to cholesteatoma: analysis of outcome following surgery.

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.

A large and uncharacteristically aggressive cholesterol granuloma of the middle ear.

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.

A distributed computation of Interpro Pfam, PROSITE and ProDom for protein annotation.

Interpro is a widely used tool for protein annotation in genome sequencing projects, demanding a large amount of computation and representing a huge time-consuming step. We present a strategy to execute programs using databases Pfam, PROSITE and ProDom of Interpro in a distributed environment using a Java-based messaging system. We developed a two-layer scheduling architecture of the distributed infrastructure. Then, we made experiments and analyzed the results. Our distributed system gave much better results than Interpro Pfam, PROSITE and ProDom running in a centralized platform. This approach seems to be appropriate and promising for highly demanding computational tools used for biological applications.

The influence of the Internet on decision making in acoustic neuroma.

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.