Clinics in diagnostic imaging (111): X-linked congenital mixed deafness syndrome.

Two siblings, boys aged five and six years old, presented with mixed hearing loss. Computed tomography of the temporal bones showed bulbous dilatation of the internal auditory canals and incomplete separation with the basal turn of the cochlear, consistent with the diagnosis of X-linked congenital progressive mixed deafness syndrome. The diagnosis and management of this rare condition is discussed.

Endolymphatic sac tumour: a rare cause of recurrent vertigo.

Endolymphatic sac tumour occurring in a 32-year-old man presenting with Meniere's like symptoms of recurrent vertigo, hearing loss and tinnitus is described. Magnetic resonance imaging and computed tomography showed a vascular bone tumour centred over the retrolabyrinthine aspect of the temporal bone where the endolymphatic sac was located. Surgical excision via a translabyrinthine approach was performed. Endolymphatic sac tumours are rare papillary adenocarcinomas that arise from the endolympatic sac. It can be mistaken both on radiology and histology for other tumours such as paragangliomas, renal or papillary thyroid carcinoma metastases. Surgical excision is the treatment of choice but sacrifice of the auditory and facial nerve may be needed in advanced cases to achieve tumour clearance.

Vestibular rehabilitation therapy for the dizzy patient.

A customised vestibular rehabilitation therapy (VRT) programme is an important treatment modality in patients with vestibular dysfunction resulting in motion-provoked vertigo, oscillopsia (gaze instability), disequilibrium and gait disturbances. We discuss in this paper the patient selection criteria for VRT, rehabilitation strategies for unilateral and bilateral vestibular deficits, and some of the compelling evidence to support the use of VRT in treating such patients.

Why generic and disease-specific quality-of-life instruments should be used together for the evaluation of patients with persistent allergic rhinitis.

BACKGROUND: The importance of assessing health-related quality of life (HRQL) of patients with allergic rhinitis (AR) has been well established, but the specific roles of rhinitis-specific or general health instruments have not been delineated. OBJECTIVE: We analysed the psychometric properties of a disease-specific instrument, the Rhinoconjunctivitis Quality-of-Life Questionnaire (RQLQ) and the general health instrument, the Medical Outcome Short-Form 36 (SF-36) as they are employed in combination in patients with persistent AR in clinical practice. METHOD: We analysed the data collected from a prospective study of 43 newly diagnosed patients with persistent AR and 44 controls. We interviewed the patients four times, at baseline, weeks 4, 8 and 10. RESULTS: The RQLQ and SF-36 have good discriminative property, internal consistency, and test-retest reliability. The RQLQ is superior to the SF-36 as an evaluative instrument because more of its domains respond to change, the magnitude of change was greater, and the response was faster. The SF-36 is more susceptible to floor and ceiling effects. Both instruments are unsuitable for mildly symptomatic patients based on Rasch model analysis. Each questionnaire assesses a distinct and significant portion of the total HRQL of persistent AR. CONCLUSION: The SF-36 and RQLQ are good for discriminating rhinitis patients from controls, but the former is poor for detecting changes in QOL. Both are inappropriate for mildly symptomatic patients. Each instrument measures non-overlapping halves of the measurable HRQL. For an assessment of the HRQL in persistent AR that is complete and responsive both instruments should be employed together.

The value of pre-operative high resolution CT scans in cholesteatoma surgery.

INTRODUCTION: Cholesteatoma is traditionally diagnosed by otoscopic examination and treated by explorative surgery. The need for imaging in an uncomplicated case is contentious. This study assesses the usefulness of a pre-operative high-resolution CT scan in depicting the status of the middle ear structures in the presence of cholesteatoma. MATERIALS AND METHODS: The surgical findings of 36 ears with cholesteatoma operated on by the first author were retrospectively compared with the CT findings reported on by the second author. The following were analysed: diagnostic features of cholesteatoma on CT, status of the middle ear structures (ossicles, facial nerve canal, semicircular canals and tegmen tympani), and presence of any anatomical variations and disease complications. RESULTS: All cases had at least 1, and 30 cases (83.3%) had all, of the following radiological features: (a) a non-dependent tissue mass, (b) a location typical for cholesteatoma and (c) bony erosion. The radiosurgical agreement was excellent for the malleus (kappa statistics, k=0.83), stapes (0.94) and semicircular canals (0.8), good for the incus (0.62) and tegmen (0.65), but poor for the facial nerve canal (0.3). Potential surgical hazards detected by the scans included: low lying dura, high jugular bulb, anterior lying sigmoid sinus, facial nerve dehiscence and other situations brought about by the destructive nature of the lesion. CONCLUSION: There is good to excellent radiosurgical correlation in cholesteatoma for most middle ear structures except for the integrity of the facial canal. The scan alerts the surgeon to potential surgical dangers and complications of disease. High-resolution CT scan is an important investigative tool prior to cholesteatoma surgery.

Tracheoesophageal puncture outcomes and predictors of success in laryngectomised patients.

INTRODUCTION: Prosthetic voice restoration has been widely used for vocal rehabilitation in laryngectomised patients. The objectives of this study were to examine success rates, predictors of success and complications for our patients who had undergone tracheoesophageal punctures (TEPs) and voice prosthesis placement after laryngectomy. To our knowledge, this is the first analysis of the use and complications of TEPs in Singapore. PATIENTS AND METHODS: Thirty-eight patients seen in our centre were analysed in this retrospective case review. Factors analysed included voice quality with age, timing of insertion, type of closure, use of radiotherapy, regular maintenance and attendance at our clinic. Twenty-eight had undergone primary TEP surgery and 10 had undergone secondary TEP surgery for voice restoration after laryngectomy for cancer. Thirty-five patients had primary closure of the pharynx with 13 vertical, 19 horizontal and 3 T-shaped closures. The other 3 patients required reconstructive surgery. Thirty-five patients had radiotherapy. A numerical assessment of voice production was made of patients immediately and at 6 months post-insertion. A review of the complications was also done. RESULTS: TEPs provided a fair to good voice in 74% of our laryngectomees. Patients who attended the voice restoration clinics and who regularly cleaned their prostheses were found to have statistically better voices (P = 0.044 and P = 0.002, respectively). Patients less than 60 years old had better results, as did patients with horizontal or T-shaped closures; however, these were not statistically significant. Secondary TEPs provided fair/good voices in 90% of cases compared to 68% of primary TEPs. Voice quality during radiotherapy was diminished but recovered at 6 months postradiotherapy. The commonest complications noted included crusting (seen in 40% of cohort), candida infections and leaks which were seen in 16% of our patients. There was no mortality attributable to the use of TEPs in our study.

Quality of life of patients with perennial allergic rhinitis: preliminary validation of the Rhinoconjunctivitis Quality of Life Questionnaire in Singapore.

Though sufferers of perennial allergic rhinitis do not die from their ailment, they endure years of chronic nose disease that Interferes with many important aspects of their lives. A rhinitis-specific instrument to gauge the quality of life of patients with this disease was published in 1991. Here, we validated the Rhinoconjunctivitis Quality of Life Questionnaire (RQLQ) for use in English-speaking patients with perennial allergic rhinitis. We established that the RQLQ distinguishes between patients and control, demonstrates internal consistency and is sensitive to change. This study suggests that the RQLQ can be used to assess the quality of life of patients with perennial allergic rhinitis in Singapore.

Clinical audit on tonsils and adenoid surgery. Is day surgery a reasonable option?

Four hundred and five patients who underwent tonsillectomy and/or adenoidectomy over a 27-month period were reviewed retrospectively to determine local patient characteristics and complication rates. The predominance of adult patients (mean age of 23.8 years), male subjects (male to female ratio of 2:1), and tonsillectomy cases (85.4%) were in contrast to studies reported in Western countries. Chronic tonsillitis or adenoiditis was the most common indication for surgery and the most frequent complications encountered were fever (20.0%) and haemorrhage (5.2%). Three patients (0.7%) had significant haemorrhage requiring a return to the operating theatre for haemostasis. Bleeding occurred within 6 hours in 2 patients and on the third postoperative day in 1 patient. The rate of haemorrhage was significantly higher in male patients (P = 0.038). The average duration of hospitalisation was 3.6 days. The main reasons for delayed discharge from hospital were retention for observation of pain and fever or for social or administrative purposes. Based on the results of the survey, day surgery for tonsillectomy and adenoidectomy would be feasible in Singapore provided patients are carefully selected and actively monitored for at least 6 hours after the operation.