Cochlear implantation in auditory neuropathy spectrum disorders: role of transtympanic electrically evoked auditory brainstem responses and serial neural response telemetry.

OBJECTIVE: To evaluate the utility of pre-operative transtympanic electrically evoked auditory brainstem responses and post-operative neural response telemetry in auditory neuropathy spectrum disorder patients. METHODS: Four auditory neuropathy spectrum disorder patients who had undergone cochlear implantation and used it for more than one year were studied. All four patients underwent pre-operative transtympanic electrically evoked auditory brainstem response testing, intra-operative and post-operative (at 3, 6 and 12 months after switch-on) neural response telemetry, and out-patient cochlear implant electrically evoked auditory brainstem response testing (at 12 months). RESULTS: Patients with better waveforms on transtympanic electrically evoked auditory brainstem response testing showed superior performance after one year of implant use. Neural response telemetry and electrically evoked auditory brainstem response measures improved in all patients. CONCLUSION: Inferences related to cochlear implantation outcomes can be based on the waveform of transtympanic electrically evoked auditory brainstem responses. Robust transtympanic electrically evoked auditory brainstem responses suggest better performance. Improvements in electrically evoked auditory brainstem responses and neural response telemetry over time indicate that electrical stimulation is favourable in auditory neuropathy spectrum disorder patients. These measures provide an objective way to monitor changes and progress in auditory pathways following cochlear implantation.

An unusual case of ear mould impression material as a foreign body in the middle ear: case report.

BACKGROUND: The occurrence of retained ear mould impression material is rare and can lead to complications. The current case report describes one such complication, where the silicone impression material used to take the impression of the ear canal flowed into the middle ear through the pre-existing tympanic membrane perforation. Five days later, the patient presented with worsened hearing and blood-tinged discharge from the ear. Ear microscopy revealed a greenish foreign body in the middle ear. CASE REPORT: The foreign body was removed by tympanotomy and the perforation repaired using a temporalis fascia graft. A hearing aid was prescribed after ensuring that the perforation had healed. CONCLUSION: It is essential that the audiologist perform a basic otological examination before prescribing a hearing aid and preparing an ear mould. A clinical approach algorithm for audiologists, for prior to taking an impression, is suggested.

Assessment of quality of life outcomes with the Glasgow Children's Benefit Inventory following cochlear implantation in children.

BACKGROUND: This study investigated health-related quality of life outcomes for children with cochlear implants in India using the Glasgow Children's Benefit Inventory questionnaire. Cochlear implantation is associated with improved language outcomes. Some studies show this gives higher quality of life after implantation. Previous research demonstrates that India presents unique circumstances that impact perspectives regarding cochlear implantation. METHOD: Children (aged under 18 years) who had undergone cochlear implantation were recruited from Vani Pradan Kendra, an organisation for individuals with hearing loss based in Bangalore, India. Demographic data including age, sex, duration of hearing loss and age at implant were collected, and the children's parents or caregivers completed the Glasgow Children's Benefit Inventory questionnaire. RESULTS: Sixty-nine children (mean age: 8.0 ± 3.89 years) were recruited, and all reported improved quality of life after cochlear implantation. There was no effect of age, gender or education on reported benefits. However, a younger age at implant and longer experience with an implant were associated with greater quality of life improvements. CONCLUSION: Cochlear implantation leads to improved quality of life, with greater improvements associated with earlier implantation. This supports early intervention in children with profound hearing loss.

Hearing results of 1145 stapedotomies evaluated with Amsterdam hearing evaluation plots.

AIMS: To evaluate the hearing results of a large series of primary stapedotomies, according to American Academy of Otolaryngology, Head and Neck Surgery guidelines and Amsterdam hearing evaluation plots. STUDY DESIGN: Retrospective chart review. METHODS: The charts for 1369 consecutive stapedotomy cases were reviewed; 1145 cases of primary stapedotomy were included. Raw data from the audiometric database were evaluated using Amsterdam hearing evaluation plots. The effect on outcomes of using different audiological parameters was analysed. RESULTS: A significant improvement was demonstrated in mean post-operative air conduction and speech reception thresholds, with no change in bone conduction. Air-bone gap closure of 10 dB or more was achieved in 82 per cent of cases. A 'dead ear' occurred in one patient (0.1 per cent). CONCLUSION: This study reports the largest series of primary stapedotomies evaluated with Amsterdam hearing evaluation plots. This method enables visual identification of successful and unfavourable results, providing more accurate and detailed presentation of surgical outcomes.

Anterior inferior cerebellar artery syndrome: fact or fiction.

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).

Iatrogenic perforation in endoscopic stapling diverticulotomy for pharyngeal pouches.

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.

A retrospective review of pharyngeal pouch surgery in 56 patients.

We retrospectively reviewed 56 consecutive patients treated surgically for a pharyngeal pouch at our institution between 1989-1999 (10 years). Various surgical procedures were performed including endoscopic stapling (20), external excision (23), Dohlman's procedure (9), pouch inversion (3), cricopharyngeal myotomy only (3), and pouch suspension (1). There were 12 patients (18%) with complications and one mortality (2%). Four patients (7%) had a recurrence with 2 requiring further surgery. Over the latter 3 years, endoscopic stapling has emerged as the primary procedure for pharyngeal pouch surgery in our unit; with the advantages of an earlier commencement of diet and earlier hospital discharge. However, results were not as good as for external excisions. Furthermore, there were difficulties with 3 cases that commenced as endoscopic stapling procedures but had to he converted to external excisions due to inaccessibility in one case and iatrogenic perforations in two cases. As with any new technique, problems may occur and a learning curve has been appreciated in our unit. Surgeons must he prepared, with informed consent, to convert to an external approach should difficulties arise during endoscopic stapling. Elderly and frail patients who are at risk from a general anaesthetic may benefit from endoscopic stapling. External excision of pharyngeal pouches may be more appropriate in the young, the medically fit, and when malignancy is a concern.

Minimum dataset for recording myringotomy and ventilation tube insertion.

A minimum dataset for recording of findings during myringotomy and ventilation tube insertion for cases of otitis media with effusion is presented. With increasing pressures on surgeons to audit existing practices and hence improve standards of health care, it is appropriate to produce such a set of guidelines for a surgery that is frequently performed world-wide. We believe that the data presented is not too exhaustive and can be readily incorporated into the operative notes.

Maffucci's syndrome: clinical and radiological features of a rare condition.

Maffucci's syndrome is a rare congenital non-inherited condition, characterized by multiple enchondromata, cutaneous haemangiomata and more recently spindle-cell haemangioma-endotheliomata. It is associated with an increased risk of malignancy including intracranial chondrosarcomas. Early diagnosis is crucial; screening patients with Ollier's disease is recommended. The treatment of choice for these intracranial cartilaginous tumours is complete surgical excision but this is often difficult to achieve due to difficult access and relationships with neurovascular structures. An alternative treatment is proton-beam therapy. We report a case of Maffucci's syndrome; illustrating the typical clinical and radiological features as well as the known complications of the condition.

Transcanal atticoaditotomy and transcortical mastoidectomy for cholesteatoma: the Farrior-Olaizola technique revisited.

Conservative mastoid surgery in the management of middle ear cholesteatoma has been a subject of controversy for decades. Proponents of the combined-approach tympanoplasty for cholesteatoma do not entirely support the conservative approach in children, especially in the hands of an inexperienced surgeon. We describe here our experience with a modified technique of conservative mastoid surgery involving an atticoaditotomy and a separate simple mastoidectomy with lateral attic wall reconstruction (modified Farrior technique) that can be relatively safely used in the majority of cases of chronic squamous otitis media, even in children. This was a prospective analysis of the therapeutic effects and benefits of the technique used in 103 cases of cholesteatoma at Staffordshire General Hospital from 1991 to 1997. The follow-up was between 3 and 9 years. The study group consisted of 55 male patients and 48 female patients, including 28 children less than 16 years of age. The age ranges were 9 to 61 years in male patients and 10 to 48 years in female patients. After an audiological assessment, all patients underwent the described mastoid procedure, and some of them required revision surgery, mostly for a second look or for ossiculoplasty. The chief outcome measures used were complete eradication of disease, incidence of disease recurrence, and restoration of hearing. The steps of the procedure, its pros and cons, and the results of the series are presented. Also discussed is an overview of the literature pertaining to intact-canal wall mastoid surgery. Of the 103 patients, only 3 had a recurrence of disease, and 9 patients showed no hearing improvement. We conclude that this modified technique has several advantages over the previously described mastoidectomy procedures. It is a relatively simple and effective technique that can be performed even in children.

Mandibular advancement prosthesis: first-line alternative to surgery in snoring.

Mandibular advancement prosthesis (MAP) is infrequently used in the UK at present for snoring. First-line measures include dietary and weight modification for those that require it. Where such measures are unlikely to be useful or have already failed, surgery is sometimes utilized as a second-line treatment modality. We evaluate the use of MAP as an adjunct to first-line measures, with emphasis on efficacy, side-effects and patient compliance. Case notes of 30 snorers were reviewed and followed up with a questionnaire. Despite being useful in alleviating snoring, the prosthesis was poorly tolerated. Side-effects include increased salivation, temporomandibular joint pain, intra-oral and myofacial discomfort. Patients who persevered with the prosthesis found the early side-effects resolved after a few weeks and snoring reduced. MAP can be used in the initial management of snorers but patients need to be educated and encouraged, especially in the first few weeks.

The aetiology of nasopharyngeal carcinoma.

Nasopharyngeal carcinoma is a disease with a remarkable racial and geographical distribution. In most parts of the world it is a rare condition and in only a handful of places does this low risk profile alter. These include the Southern Chinese, Eskimos and other Arctic natives, inhabitants of South-East Asia and also the populations of North Africa and Kuwait.

Middle cranial fossa approach for the repair of spontaneous cerebrospinal fluid otorrhoea using autologous bone pate.

Tegmen plate defects causing cerebrospinal fluid (CSF) leaks and brain hernias have conventionally been repaired using soft tissue grafts by a transmastoid approach. A review of the literature reveals that the results of transmastoid repairs have been less than satisfactory. We present here four patients with spontaneous CSF otorrhoea who had tegmen defects repaired using the middle cranial fossa approach. An autologous bone pate slab mixed with fibrin glue with additional temporalis fascia reinforcement was used for the repairs. All four patients had uneventful postoperative periods and have had no subsequent CSF leak (follow-up range: 1-3 years). We believe that the middle cranial fossa approach is more effective than the transmastoid approach for the repair of CSF leaks and brain hernias through tegmen plate defects. An important additional benefit is hearing preservation. We also recommend the use of bone pate along with soft tissue for the repair in order to achieve a secure seal. Bone pate can effectively seal multiple defects in the tegmen plate without any risk of migration.

Tumefactive fibroinflammatory lesion of the neck.

Fibrosclerotic and fibroinflammatory diseases are pelicular and unusual conditions, which may present in any part of the body. Howeverm, in the region of the head and the neck they are seldom seen and hence easily misdiagnosed. To date, about twenty-two such cases have been reported in the head and neck region. Presented here is a case of tumefactive fibroinflammatory disease of the neck in an 85-year-old woman with a detailed account of the clinical features, cytology, histology and postmortem findings. Also presented is a brief overview of the literature pertaining of this condition.

Antrochoanal polyp presenting with obstructive sleep apnoea and cachexia.

A rare presentation of an antrochoanal polyp is reported. A 14-year-old boy presented with obstructive sleep apnoea and subnormal growth velocity for height and weight over a 1-year period. Examination revealed a post-nasal mass which following removal was confirmed histopathologically as an antrochoanal polyp. Relief of the airway obstruction was promptly followed by catch-up growth and subsequent normal growth velocities. The possible mechanisms underlying the cachexia are explored including the possible association with the obstructive sleep apnoea.

Intraductal papilloma of the submandibular gland.

Salivary tissue intraductal papillomas are rare, benign tumours that predominantly affect minor salivary glands. We report a case of an intraductal papilloma arising in the unusual site of the submandibular gland. The tumour was completely excised and recurrence is not expected. A brief review of this histologically distinct lesion is presented.

Desmoplastic fibroma of the temporal bone.

An unusual case of a desmoplastic fibroma of the temporal bone is presented. Although classified benign, this intraosseous lesion exhibits local aggressiveness and has a high potential for recurrence. This rare condition occurs predominantly in the mandible and in the long bones and is seldom seen in the calvarium. Here we add another case to the previously described eight in the skull and this is the fourth such case reported in the temporal bone. The clinical features, radiology, histopathology and the therapeutic considerations of this lesion in a 72-year-old female are discussed. In addition, a literature review of all the cases affecting the skull bones is presented.