Introduction: Cholesteatoma is an aggressive disease and its management poses a greater challenge in children than in adults. This study reviews the experience of Universiti Kebangsaan Malaysia Medical Centre in the clinical presentation and management outcome of acquired cholesteatoma in paediatrics that required surgical interventions. Materials and Methods: A retrospective review of case records of patients below 18 years old who underwent surgery from 1999 to 2010. Results: A total of 46 patients presented with 53 cases of cholesteatoma in which seven patients had bilateral disease. The age of presentation ranged from four to 18 years old with a mean age of 12 years. Male and female patients were 65% and 35% respectively. Otorrhoea or previous history of otorrhoea on presentation was found in 94% and 96% of them had hearing impairment. Cerebellopontine angle abscess, sigmoid sinus thrombosis and mastoiditis were among the complications. Tympanic membrane was retracted in 64% while 47% having had attic retraction and 53% had total atelectasis. A majority (85%) underwent canal wall down surgery with or without tympanoplasty. Post-operatively, 71% had improvement or preserved hearing level. The duration of follow up ranged from one month to 13 years and a quarter had recurrent disease and underwent revision surgeries. Conclusion: Majority of the cholesteatoma patients suffered from hearing loss and otorrhoea. Tympanic membrane retraction remained the most common clinical finding. Hence, children with persistent otorrhoea after adequate treatment may represent cholesteatoma. Surgical options of canal wall up and canal wall down procedures have equal risk of recurrence.
Cholesteatoma ; Hearing Loss ; Recurrence

Background: Mastoid abscess remains a recognised complication of otitis media despite the advent of antibiotics. The objectives of this study were to describe the risk factors in patients with mastoid abscess following acute and chronic otitis media and discuss the management of this infection. Method: A retrospective analysis was done on all patients who underwent mastoidectomy for mastoid abscess from January 2002 to December 2007. Data on the patients’ presentation, associated complications, management, and follow-up were analysed. Results: A total of 12 patients were enrolled in this study population. Group A consisted of patients with mastoid abscess preceded by acute otitis media, while Group B consisted of patients with mastoid abscess and chronic otitis media. In Group A (n = 7), 4 patients had a pre-morbid immunocompromised condition, but they did not have cholesteatoma. None of the patients in Group B (n = 5) had any pre-morbid illnesses. Out of 12 patients, 7 patients had associated extracranial complications, and 1 patient had intracranial complications. Most patients recovered well after mastoidectomy. Recurrence was noted in 1 patient who had acute lymphoblastic leukaemia. Conclusion: Mastoid abscess is still a recognised complication of acute otitis media, especially in patients who are immunocompromised. Immunocompetent patients may also develop mastoid abscess following chronic otitis media associated with cholesteatoma. Thus, early treatment of otitis media and close vigilant follow-up are advocated to ensure prompt detection of mastoid abscess complications.

A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication. KEY WORDS: Microtia; canal atresia; cochleovestibular nerve aplasia; inner ear anomaly
Congenital Microtia

Bauxite mining is not known to most Malaysian except recently due to environmental pollution issues in Kuantan, Pahang. Potential impacts are expected to go beyond physical environment and physical illness if the situation is not controlled. Loss of economic potentials, and the presence of unpleasant red dust causing mental distress, anger and community outrage. More studies are needed to associate it with chronic physical illness. While evidences are vital for action, merely waiting for a disease to occur is a sign of failure in prevention. All responsible agencies should focus on a wider aspect of health determinants rather than merely on the occurrence of diseases to act and the need to emphasize on sustainable mining to ensure health of people is not compromised.

Nasal obstruction in neonates is a potentially fatal condition because neonates are obligatory nasal breathers. Bilateral choanal atresia is therefore a neonatal emergency. Several approaches for corrections of choanal atresia are available including the helium laser: YAG. A 5-year-old Chinese girl born with bilateral choanal atresia, had birth asphyxia that required intubation. She underwent multiple surgeries for correction of choanal atresia at other hospitals but failed to improve. She was referred to Universiti Kebangsaan Malaysia Medical Center (UKMMC) after presenting with intermittent respiratory distress and cyanosis following an upper respiratory tract infection. A repeat computed tomography (CT) scan done preoperatively showed complete bony stenosis over the left choana and finding was confirmed by examination under general anesthesia. She underwent endoscopic transnasal removal of left bony atretic plate. There was no intra or postoperative complications. During follow up 10 years later, the airway on both sides remains patent.

Hearing impairment in adolescents is a major public health problem. According to the World Health Organization (WHO) deafness and hearing impairment are common health problems throughout the world. Hearing impairment generally impairs emotional, social, communication and educational function. The aim of this study was to determine the correlation between duration of hearing aid use and improvements in the quality of life. The cross sectional study was conducted at Jalan Peel Primary Special School and Universiti Kebangsaan Malaysia Medical Center (UKMMC) from July 2010 until June 2011. A total of 21 students with hearing impairment involved in this study with mean age of 12.57 (10 to 19 years old). The subjects were divided into 2 groups: first-time hearing aid users and long standing hearing aid users. The hearing assessment was conducted in the first group and hearing aids were fitted. After 1 month hearing aid fitting, the questionnaires were distributed to both groups. Statistical analysis had showed no relation (p>0.05) between duration of hearing aid use and the improvement in the quality of life. However, regardless of the duration of hearing aid usage, there was improvement in the quality of life as shown by the scores of the questionnaires. In conclusion there was no significant relation between duration of hearing aid use and the improvement in the quality of life. Hearing aids were beneficial for hearing loss students regardless of the duration of the hearing aid usage.

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.
Adult ; Biopsy, Fine-Needle ; Combined Modality Therapy ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Neoplasm, Residual ; Pharyngeal Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Radiotherapy, Adjuvant ; Sarcoma, Synovial ; diagnosis ; pathology ; radiotherapy ; surgery ; Tomography, X-Ray Computed

Bone metabolism requires a balance mechanism between intricate processes of bone formation and resorption, which is affected by the essential interaction between osteoblasts and osteoclasts. Interleukin-17 (IL-17) is a family of cytokines consisting of six isoforms: IL-17A, IL-17B, IL-17C, IL-17D, IL-17E, and IL-17F. Among these isoforms, IL-17A has shown promising and novel roles in the regulation of bone metabolism. IL-17A has also captivated the interest of many researchers since its discovery because of its role as a pro-inflammatory cytokine which mediates a few inflammatory processes. This review describes the biology of IL-17A and its receptor as well as summarises the regulatory role of IL-17A in bone metabolism and diseases through some known pathways. Understanding the function of IL-17A in bone metabolism may lead to the development of therapeutics or diagnostic strategies for some bone diseases; as well as future therapy using tissue engineering and regenerative medicine approaches.

Intermaxillary tooth size discrepancy (TSD) is the disproportion in patients’ tooth size that is usually referred to the Bolton analysis. This size imbalance may also affect the outcome of orthodontic treatment. Therefore, this retrospective study was conducted to determine the prevalence of intermaxillary tooth size discrepancy (TSD) beyond 2 standard deviation (2 SD) from Bolton’s mean in orthodontic patients and to evaluate the effect of TSD beyond 2 SD of Bolton’s mean on orthodontic treatment outcomes. A total of 226 sets of orthodontic study models that met the selection criteria were selected from 3077 orthodontic cases. Bolton's analysis of the anterior and overall ratios was performed on each of the pre-treatment models. The orthodontic treatment outcomes for the cases which were identified of having TSD beyond 2 SD were evaluated and any interventional techniques to improve the outcome of the treatment were recorded. The results showed that 29.20% of cases had TSD beyond 2 SD of Bolton’s mean. 26.54% of cases had anterior ratio of more than 2 SD. Meanwhile, 7.08% of cases had an overall ratio greater than 2 SD. Of all cases with TSD beyond 2 SP, most of these cases (62.12%) had affected the outcome of orthodontic treatment. In conclusion, there is more than one quarter of orthodontic patients present with TSD beyond 2 SD of Bolton’s mean which may influence the outcome of orthodontic treatment. Intervention techniques should be carefully planned to achieve satisfactory treatment outcomes.

Background: Ventriculoperitoneal (VP) shunting is a permanent form of cerebrospinal fluid (CSF) diversion that can be performed for hydrocephalus. Sterility of the CSF is an important prerequisite for permanent shunt placement. It has been hypothesised that in early stage of meningitis, ventricular CSF remains sterile. A study is conducted on the first CSF sample taken from patients suspected to have meningitic hydrocephalus. Method: A retrospective review case records of patients who had undergone external ventricular drainage (EVD) for suspected meningitic hydropcephalus in Hospital Sultanah Aminah Johor Bahru (HSAJB), Johor, Malaysia. Results: Fifty-one cases were analysed. Mean age of patients was 37.27 years old, with 64.7% of them was male. Univariate analysis revealed that the main parameters to determine CSF sterility were CSF glucose (95% CI, 0.852, 10.290, P = 0.001), CSF protein (CI 95%, 0.722, 14.898, P < 0.001), CSF gram stain (95% CI, 16.437, 0.877, P < 0.001 ) and CSF appearance ( 0.611, 6.362, P = 0.012). Multivariate analysis had proven that gram stain was the main parameter in the CSF analysis (CI 95%, 16.437, 0.029, P = 0.016). No significant differences in CSF results were observed from EVD and lumbar puncture. Conclusion: The most significant parameter in CSF to determine infection was gram stain.