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.

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.

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.

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.

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.

An analysis of diagnostic delay in unilateral facial paralysis.

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.

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.

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.

Cerebellopontine angle arachnoid cysts in adult patients: what is the appropriate management?

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.

Curtailment of higher surgical training in the UK: likely effects in otology.

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.

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.

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.

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.

Hearing preservation in patients undergoing vestibular schwannoma surgery: comparison of middle fossa and retrosigmoid approaches.

OBJECT: The goal of this retrospective study was to evaluate hearing preservation after surgery for vestibular schwannoma in which the middle fossa (MF) or retrosigmoid (RS) approaches were used. Hearing preservation in vestibular schwannoma surgery can be achieved by using either the MR or RS approach. Comparative outcome data between these approaches are lacking, and, as a result, selection has generally been determined by the surgeon's preference. METHODS: The authors have compared removal of small vestibular schwannomas via MF and RS approaches with regard to hearing preservation and facial nerve function. The study group was composed of consecutively treated patients with vestibular schwannoma, 48 of whom underwent operation via an MF approach and 50 of whom underwent the same number of RS operations. Tumors were divided into size-matched groups. Hearing results were recorded according to the American Academy of Otolaryngology-Head and Neck Surgery criteria, and facial nerve outcome was recorded as the House-Brackmann grade. Overall, 26 (52%) of the patients treated via the MF approach achieved a Class B or better hearing result compared with seven (14%) of the RS group. Some hearing was preserved in 32 (64%) of the patients in the MF group and in 17 (34%) of the RS group. The results obtained by using the MF approach were superior for intracanalicular tumors (p=0.009, t-test), and for tumors with a cerebellopontine angle (CPA) component measuring 0.1 to 1 cm (p=0.006, t-test). For tumors in the CPA that were 1.1 to 2 cm in size, our data were inconclusive because of the small sample size. Facial weakness was seen more frequently after MF surgery in the early postoperative period, but results were equal at 1 year. CONCLUSIONS: The results of this study have demonstrated a more favorable hearing outcome for patients with intracanalicular tumors and tumors extending up to 1 cm into the CPA that were removed via the MF when compared with the RS approach.

Molecular genetic investigation of the neurofibromatosis type 2 tumor suppressor gene in sporadic meningioma.

The authors investigated the role of somatic mutations of the neurofibromatosis type 2 (NF2) gene in sporadic meningioma. Neurofibromatosis 2 is a dominantly inherited familial tumor syndrome predisposing affected patients to a variety of central nervous system tumors including vestibular schwannoma and meningioma. Neurofibromatosis type 2 is caused by germline mutations in the NF2 tumor suppressor gene. In addition, the authors and others have reported that somatic NF2 gene mutations occur frequently in nonfamilial vestibular schwannoma. In this study, molecular genetic analysis was performed on 23 nonfamilial meningiomas. Paired DNA samples extracted from the blood and tumors of the patients were analyzed for loss of heterozygosity (LOH) in the region of the NF2 gene on chromosome 22 using closely linked DNA markers. The NF2 gene mutations were sought by single-stranded conformation polymorphism analysis and DNA sequencing. Fourteen (61%) of 23 meningiomas showed LOH in the region of the NF2 gene on chromosome 22. Somatic NF2 gene mutations were detected in eight meningiomas (35%) after screening all 17 exons. All tumors with NF2 gene mutations showed simultaneous chromosome 22 LOH. Review of the histopathological findings of the cases studied did not demonstrate any predominance of genetic abnormalities in a particular histological type of meningioma. These results are compatible with the hypothesis that the NF2 gene acts as a tumor suppressor and that its inactivation is important in the pathogenesis of sporadic meningioma.

Langerhans' cell histiocytosis in childhood: management of head and neck manifestations.

The authors carried out a retrospective analysis of 131 cases of Langerhans' cell histiocytosis treated at two centers over a 30-year period. All those with head and neck manifestations have been reviewed with particular reference to management and outcome. The role of chemotherapy, radiotherapy, and surgery is discussed, and we present our results of the successful introduction of topical nitrogen mustard (Mustine) ear drops for meatal skin involvement. During the period of the study our modus operandi has evolved from an aggressive medical and surgical approach, favored early in the series, to our present more conservative approach, with an attendant reduction in morbidity.

Nervus intermedius function after vestibular schwannoma removal: clinical features and pathophysiological mechanisms.

The results of facial nerve outcome following vestibular schwannoma removal have generally ignored the sensory component of the nerve. This lack of reporting occurs partly because the distress relating to these functions is less obvious to the surgeon, and partly because the facial nerve grading systems currently used do not include the functions of the nervus intermedius. We have estimated the frequency and nature of abnormalities of nervus intermedius function following vestibular schwannoma removal using a retrospective questionnaire. Questionnaires were mailed to 257 patients and correctly completed and returned by 224 (87%) of the patients. Prior to surgery 5 (2%) of the patients complained of crocodile tears, 9 (4%) noted dryness of the eye, and 15 (6%) complained of an abnormality of taste. Postoperative crocodile tears occurred in 98 (44%), an absence or significant reduction in the production of tears was noted in 162 (72%), and a taste abnormality, either a significant reduction or an alteration in character, was noted in 107 (48%). The onset of crocodile tears approximated to a bimodal distribution, and the recovery of nervus intermedius functions was variable. This study has demonstrated that nervus intermedius abnormalities are common following vestibular schwannoma removal. It also documents their natural history and discusses the underlying pathophysiological mechanisms. We suggest that appropriate preoperative counseling be given to all patients undergoing surgery and that the functions of the nervus intermedius be included in the surgical reporting of facial nerve results in cerebellopontine angle surgery.