Kimura's disease (KD) is an angiolymphoid proliferative disease of soft tissue with peripheral blood eosinophilia and elevated serum immunoglobulin (Ig) E. The treatment options range from conservative observation for the asymptomatic patient to surgical excision, steroid therapy and radiotherapy for symptomatic patients. Surgical excision is the most common diagnostic measure and is the treatment of choice. A case of KD in a 13-year-old Malay girl is presented. Clinically there was painless right jugular digastric mass measuring 3cm by 3cm. Her blood investigation showed pronounced eosinophilia. She underwent excision biopsy uneventfully. The biopsy from the swelling showed reactive follicular hyperplasic with prominent eosinophilia. There was no evidence of malignant change. Postoperatively after 3 years follow up, she was asymptomatic and no signs of tumor recurrence.
Ethylmalonic encephalopathy is a rare metabolic disease presenting in infancy with developmental delay, acrocyanosis, petechiae, chronic diarrhea and early death. The biochemical characteristics of this autosomal recessive disease are urinary organic acid abnormalities. Recently it has been found to be caused by mutations in the ETHE1 gene, located on Ch19q13. Only about 30 patients have been reported, and we describe two additional cases. The first patient showed a typical clinical picture and biochemical abnormalities, with additional atypical clinical features. Neuroimaging studies showed extensive changes. A new homozygous mutation in exon 3 of the ETHE1 gene was found. The second patient was not investigated genetically; however besides the typical clinical picture and biochemical profile he was found to have cytochrome C oxidase deficiency.
This study was aimed at regenerating autologous elastic cartilage for future use in pediatric ear reconstruction surgery. Specific attentions were to characterize pediatric auricular chondrocyte growth in a combination culture medium and to assess the possibility of elastic cartilage regeneration using human fibrin.
Proper management of chronic otitis media may reduce the incidence of otogenic brain abscess. The aim of this study was to describe the clinical profile, treatment and surgical outcome of patients presenting with otogenic brain abscess. The medical record of patients in Universiti Kebangsaan Malaysia Medical Centre (UKMMC) with otogenic brain abscess were retrospectively analyzed from January 1997-January 2006. Within this eriod we had approximately 10,800 of follow up cases of chronic otitis media (COM) in our clinic. Ten patients (2 females, 8 males) with an average age of 42 (age range 11 to 69 years) were identified with otogenic brain abscess and included in this study. The mean follow-up period was 14 months. All patients had cholesteatoma. All patients had a history of chronic ear discharge, headache, otalgia and fever. Six of the 10 patients had cerebellar abscess and 4 had temporal lobe abscess. Cerebellar signs were present in 3 patients All the patients were treated with broad-spectrum antibiotics. In 5 patients, mastoid exploration was the primary surgical treatment and the brain abscesses were treated conservatively. In the other 5 patients, craniotomy and drainage were performed followed by mastoid exploration when their neurological conditions had stabilized. All our patients had uneventful recovery. There were no permanent cerebellar signs during the follow up and no mortality reported in our series. In this series we demonstrated that early diagnosis and proper treatment of otogenic abscess leads to good neurological outcome.
This study was designed to compare the sensitivity and specificity of detecting sensorineural hearing loss (SNHL) using the transient-evoked otoacoustic emissions (OAE) machine (the Madsen TE Echoscreen) and automated auditory brainstem response (AABR) machine (the Sabre Compac portable AABR) in term neonates exposed to severe hyperbilirubinaemia.
Chondrocytes were isolated from normal and microtic human auricular cartilage after ear surgery carried out at Universiti Kebangsaan Malaysia Medical Centre. Chondrocytes were cultured and expanded until passage 4. After reached confluence, cultured chondrocytes at each passage (P1, P2, P3 and P4) were harvested and assigned for growth profile analysis. There was no significant difference in cell viability between both normal and microtic samples (p = 0.84). Both samples showed no significant differences for growth profile parameters in terms of growth rate, population doubling time and total number of cell doubling, except in passage 1, where there is significant difference in cell growth rate (p = 0.004). This preliminary data has indicated that chondrocytes from microtic cartilage has the potential to be used in the reconstruction of human pinna in the future.
The importance of universal newborn hearing screening (UNHS) in identifying hearing-impaired infants as early as possible is already well recognized. Transient evoked otoacoustic emissions (TEOAE) have been established as a reliable method for UNHS in full term infants. This is a cross sectional study between April 2003--December 2005. Thirteen thousand five hundred and ninety eight (13,598) newborns were screened for hearing loss with portable otoacoustic emission (OAE) before discharge. The initial coverage rate during the 3 years study period was 85.9% (13,598) with 89.2% (3762), 79.0% (4480) and 90.3% (5356) for 2003, 2004 and 2005 respectively. The mean age when hearing loss was diagnosed using ABR were 3.56 months old, 3.08 months old, and 2.25 months old and 3.01 months old for 2003, 2004, 2005 respectively and it was statistically significant. The defaulter rate at the third stage during the 3 years study period was 35% (21), 15.2% (7) and 18.2% (2) for 2003, 2004 and 2005 respectively. This study showed significant improvement in initial referral rate, coverage rate and age of diagnosis. However, we need to improve on high defaulter rates.
To demonstrate a simple, practical, cheap method of preventing potentially fatal aspiration of a dislodged voice prosthesis; this method was developed by a laryngectomised patient.
The main objective of this study was to determine the motives underlying the selection of foods between husbands and wives in an urban community. Thiscross-sectional study was carried out in Bandar Baru Bangi, Selangor, Malaysia among 150 married couples aged 20 and above, who voluntarily agreed to participate and were not on any special diet. Data were collected using the Food Choice Questionnaire (FCQ) which measured the health-related and non health related factor that influence people's food choices. It consisted of 36 items designed to assess the reported importance of nine factors: health, mood, convenience, sensory appeal, natural content, price, weight control, familiarity, and ethical concern. In this study, the FCQ was adapted and a new factor, religion (religious guidelines), was included. Demographic characteristics including age, occupation, education, household income and household size were also collected. Data were analysed using SPSS version 16. Results showed that 40.7% of husbands (mean age= 43.33 + 11.16 years) and 55.3% of wives (mean age= 41.28 + 10.93 years) perceived themselves as the main food shopper while 12.0% of the husbands and 85.3% of the wives perceived themselves as the main meal planner. Husbands rated religion as the most prominent factor in food choice motives with a mean average rating of 4.56 + 0.59 on a 5-point rating scale, followed by health and convenience factor. Meanwhile, the wives rated health as the most essential factor with mean average rating of 4.49 + 0.58, followed by religion and convenience factor. Sensory appeal, ethical concerns and familiarity were rated as the bottom three factors of food choice motives among these two groups. Price of foods was not considered as an important factor in making food choices for the subjects in this study. In conclusion, the husbands and wives of this urban community rated religion, health and convenience as the three most important food choice motives in food selection.
Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.
Hearing loss and tinnitus are the main symptoms of otosclerosis. Little is known about the cause of tinnitus in otosclerosis and the factors influencing the effect of surgery on tinnitus. Though by surgery, we are able to inform patient about probable hearing gain and even benefit concerning bilateral hearing, it is however difficult to predict the course of tinnitus. The principle aim of stapes surgery is to restore hearing but some patients also report reduction in the severity of tinnitus and even complete cessation of it. We describe a case report of a 37 year old male who underwent a second stapes surgery. We wish to illustrate that for our patient, tinnitus represents a major disturbance and the patient is as much concerned with the improvement of hearing as with the improvement of tinnitus.
Temporal bone carcinoma may masquerade as an infective process causing late diagnosis. A delay in treatment as a result of missed diagnosis would carry a poor prognosis as the disease progresses to an advanced stage. We present a lady with history of chronic otorrhea, who developed left sided otalgia associated with hearing loss in her sixth decade. She underwent surgery which revealed left mastoiditis and cholesteatoma. After a year, she had a mass in her left ear and pus discharge which was initially treated as an infection. The biopsy of the mass was proven to be squamous cell carcinoma. High index of suspicion is necessary when encountering patients presenting with a mass in the ear canal with prior history of chronic otorrhea or cholesteatoma. Proper tissue biopsy is crucial. Early referral to tertiary centre is required for further management of the patient.
This is a retrospective review of congenital cholesteatoma cases that were managed surgically. There were 5 cases. The age of presentation ranged from 5 to 18 year old. Three patients presented with complication of the disease. Three patients had intact tympanic membrane, two had perforation at the anterior superior quadrant. All patients had cholesteatoma medial to tympanic membrane. Four cases had extensive ossicular erosion with preoperative hearing worse than 40 dB. Four cases underwent canal wall down mastoid surgery and one underwent canal wall up surgery. One patient had recurrence which required revision surgery. In conclusion, congenital cholesteatoma presented late due to the silent nature of disease in its early stage. Extensive disease, ossicular destruction with risk of complication at presentation were marked in our study. Hence, more aggressive surgical intervention is recommended in the management of congenital cholesteatoma.
Acute mastoid abscess is common in daily practice. In children, it is usually caused by unsuccessful treatment or partially treated acute otitis media (AOM). Some authors had reported that chronic suppurative otitis media (CSOM) can be the source of infection but it is usually associated with the presence of cholesteatoma. A case of an acute mastoid abscess in a 7 year old girl is presented. She had a history of severe otalgia with left post auricular swelling for 3 days. Clinically she was febrile, the left otoscopy showed diffuse post auricular swelling and sagging of the posterior wall of the external canal. She underwent an emergency cortical mastoidectomy for the left mastoid abscess and had an uneventful recovery. In conclusion, this patient was partially treated with antibiotics which increased the risk for ‘masked mastoiditis’, and she later developed a mastoid abscess. Therefore we advocate that all AOM patients should be treated with antibiotics at least for a duration of one week.
Facial nerve schwannoma is a rare slow growing benign tumour which arises from the Schwann cell of the neurilemma. A retrospective review of 6 patients who had been diagnosed with facial nerve schwannoma between 1998 and 2008 was conducted. There was equal distribution of male and female patients. The mean age was 42 years (range 19 to 66 years). The tumour originated in the internal auditory canal (2 patients), intra-temporal (3 patients) and intraparotid (1 patient) segments of the facial nerve. All tumours were successfully removed and facial nerve continuity was pre-served in 2 cases. The presenting symptoms of facial nerve schwannoma are non specific and dependent on the site of tumour origin. It is a great mimicker of other lesions that can present at the same location. The surgeon should have a high index of suspicion when patients present with progressive facial nerve palsy. Patients should always be counselled regarding risk of facial paralysis because the diagnosis of facial nerve schwannoma is often confirmed intra-operatively.
Congenital cholesteatoma (CC) is rare clinical condition and It is known as a pearly white mass medial to an intact tympanic membrane. In this case report, we describe a 6-year-old boy who presented with a one year history of left ear discomfort, reduced hearing, followed by ear discharge two months prior to admission. Otoscopic examination revealed a large attic perforation. Radiological investigation showed extensive cholesteatoma in the mastoid, mesotympanum and epitympanum with destruction of the ossicles including the stapes footplate. He underwent radical mastoidectomy in view of extensive cholesteatoma with tympanoplasty type V. In this case report, the classification, treatment and theories related to CC are discussed.
Introduction: The aim of this study was to assess the overall diet quality of husbands and wives in a selected urban area in Selangor.
Methodology: This cross-sectional study was carried out in Bandar Baru Bangi, Selangor among 150 married couples aged 20 and above, who voluntarily agreed to participate and were not practicing any special diet. Data were collected using a 2-day 24-Hour Dietary Recall and a Food Frequency Questionnaire (FFQ) to evaluate the quality of diet among husbands and wives using the Diet Quality Index Revised (DQI-R).
Result: Majority of husbands and wives in this study were in the middle-age group, had received tertiary education, had household monthly income of more than RM3, 500 and encompass household size of 3 to 5 persons. The mean DQI-R score for husbands (mean age= 43.33 + 11.16 years) and wives (mean age= 41.28 + 10.93 years) were 67.8 + 9.1 and 64.4 + 9.3 respectively and there was a significant different in scores between the husbands and wives (p < 0.001). In general, the diet quality of this study population was not satisfactory and that the diet quality of husbands was better compared to their wives.
Conclusion: This study gives an insight picture of husbands and wives’ quality of diet. It can be used as a guideline to improve health intervention programs in the future.
Neuropathy is frequently a late complication of diabetes mellitus. Auditory neuropathy and microangiopathy of inner ear are the possible causes of hearing loss in diabetics. To study the correlation between glycaemic control and hearing threshold in patients with type 2 diabetes mellitus and to determine the differences of hearing threshold between groups treated with different modality. This single blind randomized controlled study was performed at the Department of Medicine and Department of Otorhinolaryngology, Hospital Universiti Kebangsaan Malaysia (UKM) between 1st May 2003 and 31st September 2004. This study was approved by Research Ethics Committee (code number FF-137). Subjects were randomized into two groups. Group 1 were patients treated with conventional oral hypoglycemic agents. The patients in group 2 were those treated with insulin injection. The subjects were seen 4 weekly for 3 months. Audiometric test were performed in all subjects at each visit. Blood were taken for fasting blood glucose, Hb1Ac, and fructosamine at every visit to determine the glycaemic controls of the subject. They were 11 patients (22 ears) treated with oral hypoglycemic agents and 17 patients treated (34 ears) with subcutaneous insulin. There is no significant difference between mean pure tone threshold before and after treatment at all frequencies in both groups. There is also no significance different in fasting glucose level and fructosamine. However, there is significant difference HbA1c levels between the two groups after treatment (P