Delayed-onset haematoma formation after cochlear implantation.

OBJECTIVE: This paper presents our experience on delayed-onset haematoma formation after cochlear implantation, a topic which has not been well discussed in the literature. METHOD: Retrospective case review study. RESULTS: Five children who had undergone cochlear implant surgery at 1.5 to 4 years of age (median, 2.5 years) were studied. The haematoma episodes occurred 2-12 years (median, 6 years) after cochlear implantation. Two patients had recurrent episodes. Two of the seven haematoma episodes were managed by needle aspiration alone, four by incision and drainage alone, and one by both needle aspiration and incision and drainage. Other than one patient with coagulopathy, there were no obvious predisposing factors, including trauma. CONCLUSION: The majority of delayed-onset haematomas occurred without obvious predisposing factors. Needle aspiration can differentiate a haematoma from an abscess or cerebrospinal fluid leakage, and it provides an effective immediate therapeutic solution. However, aseptic techniques are emphasised to minimise the chances of an uncomplicated haematoma converting into a septic one.

Musical ear syndrome in adult cochlear implant patients.

OBJECTIVE: Except for a single case report, musical ear syndrome in cochlear implantees has not been studied. We aimed to study the prevalence and nature of musical ear syndrome among adult cochlear implant patients, as well as the effect on their emotional well-being. STUDY DESIGN, PATIENTS AND INTERVENTION: A cross-sectional survey of patients aged 18 years and above who had received cochlear implants for profound hearing loss between 1997 and 2010. RESULTS: Of the 82 patients studied, 18 (22 per cent) were found to have experienced musical ear syndrome. Seven and 11 patients had musical ear syndrome prior to and after cochlear implantation, respectively. The character of musical ear syndrome symptoms was described as instrumental music (n = 2), singing (6) or both (10). Fourteen patients reported an adverse emotional effect, with three expressing 'intolerance'. CONCLUSIONS: In this study, 22 per cent of cochlear implantees experienced musical ear syndrome. These symptoms affected patients' emotional state, but most coped well. Musical ear syndrome can occur prior to and after cochlear implantation.

Effect of radiotherapy on retro-cochlear auditory pathways.

OBJECTIVES: The cochlea may be damaged by modern conventional radiotherapy (RT) for head and neck cancers when the ear is included in the radiation field. It is unclear however, if the retro-cochlear auditory pathways are affected as well, which has clinical significance in cochlear implantation. This study aims to investigate the integrity of the retro-cochlear auditory pathways in patients who had received RT for nasopharyngeal carcinoma. STUDY DESIGN: Prospective study. METHODS: Patients who were newly diagnosed with nasopharyngeal carcinoma and treated by RT alone were studied. Evoked response audiometry and PTAs were carried out prior to and after RT (at 3, 18, and 48 months postRT). In addition, evoked response audiometry was also performed during the 3rd, 5th, and 7th week of RT. Waves 1 to 5, 1 to 3, and 3 to 5 latencies were measured. The values recorded during and postRT were compared with those recorded before RT. In addition, a subset of ears that demonstrated postRT sensorineural hearing loss were identified so that their respective wave 1 to 5 interwave latencies could be similarly compared. Wilcoxon signed ranks test was used in the statistical analysis. To confirm that the cochlea and internal auditory meatus receive significant doses of radiation, the RT treatment plans of nine other nasopharyngeal carcinoma patients treated by the same RT technique were analyzed to derive dose-volume histograms of these structures. RESULTS: Twenty-seven patients (20 males and 7 females) with a mean age of 51.2 (range 36-75) years participated in the study. There was no statistically significant difference in waves 1 to 5, 1 to 3, and 3 to 5 interwave latencies recorded during RT and postRT as compared with those recorded before RT (P > .05). Pre- and postRT wave 1 to 5 latencies of the 16 ears that had postRT hearing deterioration were also not statistically significant (P = .366). The mean radiation doses delivered to the cochlea and internal auditory meatus ranged from 24.1 to 62.2 Gy and 14.4 to 43.4 Gy, respectively. CONCLUSION: This study suggests in patients who have had RT for nasopharyngeal carcinoma, the retro-cochlear auditory pathways are functionally intact even in the longer term.

Managing hearing loss in children and adults: Singapore context.

Hearing loss is common in Singapore, with as many as 360,000 persons affected. With Singapore's ageing population, this prevalence is expected to increase. Modern hearing devices have the potential to restore hearing in most patients. Cochlear implants can restore hearing in those with even profound total hearing loss, by stimulating the auditory nerve directly without the need for viable cochlear hair cells. A holistic approach to managing deafness is necessary, including addressing psychosocial issues. Many of the problems experienced by the hearing-impaired are caused by the ignorance and lack of understanding from other people around him/her. "Reverse education therapy" refers to the effective education of these people, to directly benefit the hearing-impaired. In children, hearing restoration together with early detection and effective rehabilitation enable up to 80% to 90% of those born with significant hearing loss to cope well in mainstream education. The infrastructure to support them and facilitate their integration into mainstream society is necessary, and some of these are in place in Singapore. Leisure time noise-induced hearing loss in children is becoming increasingly important. In adults, societal support and understanding are just as important, particularly towards the elderly and pre-lingually deafened adults. Public education in Singapore is necessary, including that on the recognition of the abilities of successfully treated individuals, as well as that on showing more compassion, support and patience towards those who have not benefited sufficiently from treatment. In the future, there is potential for the clinical application of cochlear hair cell/auditory nerve preservation and regeneration.

Universal newborn hearing screening in Singapore: the need, implementation and challenges.

With about 1 in 1000 born with severe to profound hearing loss and about 5 in 1000 with lesser degrees of loss, congenital deafness is the commonest major birth defect. It is the recommended standard that hearing loss in newborns be detected by 3 months of age and intervention implemented by 6 months of age. Delayed detection and intervention may affect speech, language and psychosocial development, resulting in poor academic achievements. Universal newborn hearing screening (UNHS) is the only effective way of detecting all babies with hearing loss, within the recommended time frame. A survey in Singapore revealed that traditional childhood hearing screening programmes resulted in late detection (mean age, 20.8 months; range, 0 to 86 months) and late intervention (mean age, 42.4 months; range, 1 to 120 months). Increasingly, UNHS is becoming standard medical care in developed countries. In Singapore, UNHS has been implemented in all hospitals with obstetric services. Although a screening rate of more than 99% has been achieved in public hospitals, private hospitals have a screening rate of only about 77%. Parents' awareness and acceptance of early detection is still lacking, and this needs to be addressed by appropriate public education. Support from obstetricians and paediatricians will significantly contribute towards this objective. Effective programme management is essential; this includes the use of data management systems, the maintenance of a team of experienced screeners, and efficient coordination between screening and diagnostic services. Early detection of childhood deafness, together with early and effective intervention, maximises the chances of successful integration into mainstream education and society.

First branchial cleft anomalies have relevance in otology and more.

INTRODUCTION: First branchial cleft anomalies account for less than 8% of all branchial abnormalities. Their rarity and diverse presentations have frequently led to misdiagnosis and inappropriate treatment. In a trend towards specialisation/subspecialisation, first branchial cleft duplication anomalies, with their varied clinical manifestations, may possibly present to an Otology, Head and Neck Surgery, Paediatric Otolaryngology, Maxillofacial or even a General Paediatric and General Surgery practice. There is a need to highlight the clinical features which can aid in accurate diagnosis. CLINICAL PICTURE, TREATMENT AND OUTCOME: A case of an adult with Work Type 2 first branchial cleft duplication anomaly presenting as a collaural fistula is described. It first presented as a recurrent upper neck abscess in childhood. The diagnosis had previously been missed although the patient was able to clearly establish a correlation between digging of the ipsilateral ear and precipitation of the abscess. Instead of an epidermal web, a myringeal lesion in the form of a fibrous band-like was present. The lesion was completely excised with no further recurrence. CONCLUSION: This case highlights useful diagnostic features both from the history and physical examination. The specialist/subspecialist must be aware of this condition and be mindful of its possible cross specialty/subspecialty symptoms and signs. Together with a good understanding of the regional embryology and anatomy, the lesion can be diagnosed early at initial presentation with the potential for best treatment outcomes.

Kimura's disease: a diagnostic and therapeutic challenge.

INTRODUCTION: Kimura's disease (KD) is a rare, benign, chronic inflammatory disease with unknown aetiology. Its manifestation is protean. KD has a predilection for the head and neck area, and typically presents as tumour-like lesions that could be easily misdiagnosed. We review our experience with four recent cases. METHODS: Over a four-year period, all patients admitted to Singapore General Hospital with KD of the head and neck region were retrospectively reviewed. Biodata, presenting symptoms and clinical parameters, especially serum eosinophil levels, preoperative investigations, type of surgical procedures and outcome were documented. RESULTS: Four patients presented with KD of the head and neck and displayed varied manifestations of the disease. All the patients had raised serum eosinophil levels. None of them had renal involvement. Preoperative computed tomography were performed in two of the patients and showed features suggestive of KD. Fine-needle aspiration cytology that was performed in two patients was not useful in the diagnosis. All the patients underwent surgical excision of the lesions. Only one patient had multiple recurrence, both at the original and remote sites in the head and neck. CONCLUSION: The clinical presentation and behaviour of KD is very variable. Preoperative imaging is useful in the diagnosis of the disease but the final diagnosis is histological. Surgical excision is the current treatment of choice but recurrence is common. A high index of suspicion and awareness is vital in the early diagnosis and management of KD.

Leprosy: otolaryngologist's perspective.

A patient with hemi-facial erythematous swelling as a result of borderline leprosy and reversal reaction is reported. This uncommon presentation of the disease poses initial diagnostic difficulties to the otolaryngologist. The otolaryngologist must be familiar with otolaryngologic manifestations of leprosy, since early diagnosis and treatment reduces the risk of transmission of the disease and may avoid permanent nerve damage.

Cryptococcal meningitis: implications for the otologist.

Cryptococcal meningitis can present to the otologist with hearing loss and vestibular dysfunction. A man with cryptococcal meningitis presenting with bilateral profound sensorineural hearing loss and vestibular dysfunction is described. The difficulty in arriving at the diagnosis and consequences of misdiagnosis are discussed. Histological and clinical studies suggest that the cochlear nerve is likely to be damaged in patients who have deafness associated with this disease. Retrocochlear damage may result in cochlear implantation having a poor outcome.

Bone wax foreign body granuloma in the mastoid.

The sue of bone wax is commonly used to control bleeding during mastoid surgery. An unusual case of bone wax granuloma in the mastoid, with sigmoid sinus thrombosis, is reported. Although the use of bone wax in and around the mastoid is generally considered safe with few complications, caution should be exercised, particularly in infected fields and in patients known to have general immunohypersensitivity.

Leprosy: otolaryngologists perspective

A patient with hemi-facial erythematous swelling as a result of borderline leprosy and reversal reaction is reported. This uncommon presentation of the disease poses initial diagnostic difficulties to the otolaryngologist. The otolaryngologist must be familiar with otolaryngologic manifestations of leprosy, since early diagnosis and treatment reduces the risk of transmission of the disease and may avoid permanent nerve damage.

Isolation and characterization of skin-type, type I antifreeze polypeptides from the longhorn sculpin, Myoxocephalus octodecemspinosus.

The antifreeze polypeptides (AFPs) are found in several marine fish and have been grouped into four distinct biochemical classes (type I-IV). Recently, the new subclass of skin-type, type I AFPs that are produced intracellularly as mature polypeptides have been identified in the winter flounder (Pleuronectes americanus) and the shorthorn sculpin (Myoxocephalus scorpius). This study demonstrates the presence of skin-type AFPs in the longhorn sculpin (Myoxocephalus octodecemspinosus), which produces type IV serum AFPs. Using polymerase chain reaction-based methods, a clone that encoded for a type I AFP was identified. The clone lacked a signal sequence, indicating that the mature polypeptide is produced in the cytosol. A recombinant protein was produced in Escherichia coli and antifreeze activity was characterized. Four individual Ala-rich polypeptides with antifreeze activity were isolated from the skin tissue. One polypeptide was completely sequenced by tandem MS. This study provides the first evidence of a fish species that produces two different biochemical classes of antifreeze proteins (type I and type IV), and enforces the notion that skin-type AFPs are a widespread biological phenomenon in fish.

Cerebellopontine angle involvement by nasopharyngeal carcinoma.

OBJECTIVE: This article describes a series of patients with nasopharyngeal carcinoma involvement of the cerebellopontine angle and discusses the clinical significance of this entity. SETTING: Tertiary referral center. STUDY DESIGN: Retrospective case study. PATIENTS: Patients who were diagnosed with nasopharyngeal carcinoma with clinical features of cerebellopontine involvement by tumor. INTERVENTIONS: Cerebellopontine involvement by tumor confirmed by computed tomography, magnetic resonance imaging, or both. RESULTS: Patients with this entity either had advanced disease or had been treated previously for advanced nasopharyngeal carcinoma. They had varied clinical features attributable to cerebellopontine involvement, such as sensorineural deafness, dizziness, facial palsy, and facial numbness. CONCLUSIONS: Cerebellopontine angle involvement by nasopharyngeal carcinoma is a difficult entity, both from the diagnostic and therapeutic points of view. In high-risk patients, particularly in patients who were previously treated for advanced nasopharyngeal carcinoma, a high index of suspicion for nasopharyngeal carcinoma involvement of the cerebellopontine angle is warranted when they experience unexplained neurootologic symptoms such as sensorineural hearing loss, dizziness and facial palsy.

Diagnostic value of Epstein-Barr viral serology in nasopharyngeal carcinoma.

Although Epstein-Barr virus (EBV) IgA serology has been established as an effective marker for nasopharyngeal carcinoma (NPC), it remains unclear how useful or cost-effective it is as a screening test. This article is aimed at establishing how these tests could be used most effectively in the diagnosis of NPC in an otolaryngology outpatient clinic. A total of 111 consecutive patients with NPC and an equal number of control subjects were studied. Forty-seven patients with NPC had early (AJCC stages 1 and 2) and 64 had advanced (stages 3 and 4) disease. A positive early antigen (EA) serology result was found in 81.2% of NPC patients and in none of the controls. Negative EA and viral capsid antigen (VCA) serology results were present in 2.7% of NPC patients and in 46.8% of controls. Negative EA and positive VCA serology results were found in 30.0% of NPC patients with early disease, 7.8% of NPC patients with advanced disease, and 53.2% of controls. Given its high specificity, serology for EA is recommended as a clinically useful screening test. Serology for VCA, although highly sensitive, has an unacceptably high false-positive rate, and its cost-effectiveness as a universal screening test is questionable.

Correlating clinical appearance of nasopharyngeal carcinoma with tumour staging.

Since there is a tendency for nasopharyngeal carcinoma (NPC) to exhibit sub-mucosal spread and endophytic growth, it would be useful to know if NPC remained inconspicuous in the post-nasal space (PNS) even in advanced stages of disease. The aim of this study was to find out if there was a correlation between tumour appearance in the PNS and tumour stage. One hundred and twenty-two consecutive patients with NPC were studied. The appearance of a tumour in the PNS was classified as to whether or not it was obviously malignant-looking (via naso-endoscopy). Tumour stagings were undertaken according to computerised tomography (CT) scan findings. Of the patients studied, 67 (54.9%) had obvious malignant-looking lesions. Tumour appearance was found to correlate with tumour-staging (p = 0.023, chi-squared test). There was, therefore, a tendency for early tumours to present as inconspicuous or innocuous-looking lesions, whereas advanced tumours tended to appear as obvious malignant-looking lesions in the PNS. The clinical significance of these findings is discussed.

Malignancy of the temporal bone and external auditory canal.

A retrospective study of 18 patients with malignant tumors of the external auditory canal and temporal bone was undertaken to gain an Asian perspective of this rare disease. Of these patients, 15 (83%) had squamous cell carcinoma (SCC) and 61% had stage T3 tumors at presentation. The mean age was 56 years (range 38-82 years). Seven (39%) of the 18 patients had radiation-associated tumors (RATs), and all had undergone radiotherapy for treatment of nasopharyngeal carcinoma. The 1-year cumulative recurrence for the RAT group was 100%, but there was no recurrence in the non-RAT group (P = 0.001). In malignancies of the external auditory canal and temporal bone, a different classification and staging system for patients with RATs may be warranted to better guide treatment strategies.

Factors contributing to delayed diagnosis in nasopharyngeal carcinoma.

Nasopharyngeal carcinoma (NPC) can be difficult to diagnose. Not only is the post-nasal space (PNS) inaccessible to examination, it is frequently occupied by normal lympho-epithelium which can make differentiation from NPC difficult. Together with its frequent atypical presentation, it is not surprising that the diagnosis is missed or delayed. This is undesirable as the treatment of early NPC carries an excellent prognosis. The aim of this study is to ascertain the extent of the problem of missed or delayed diagnosis and to study the factors responsible. This was a retrospective study of all newly diagnosed patients with NPC from the Singapore General Hospital and treated in the Department of Therapeutic Radiology in the year 1996 (1 January-31 December). A total of 126 patients were studied. Eighteen patients (14.3 per cent) were found to have delayed diagnosis of more than a month. The delay ranged from 1.2 to 25 months (mean 7.2 months). Factors identified which contributed to delayed diagnosis included i) Clinicians not considering a diagnosis of NPC ii) Clinicians suspecting NPC but misled by the results of investigations iii) Patients refusing investigation or defaulting follow-up. Nearly a fifth of patients with NPC had delayed diagnosis. Many of the factors responsible for the delays appear to be preventable by better patient education and counselling, doctors having sharper clinical acumen and skills in NPC diagnosis and the hospital administration having a system of tracking down high risk patients who default.