Cochlear nerve canal stenosis and associated semicircular canal abnormalities in paediatric sensorineural hearing loss: a single centre study.

OBJECTIVE: This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis. METHOD: This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated. RESULTS: Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent). CONCLUSION: Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

Prevalence of allergic rhinitis in children with otitis media with effusion.

Summary: Introduction.The prevalence of allergic rhinitis in children with persistent otitis media with effusion in different countries varies between 82% to 93%. Many risk factors of otitis media with effusion has been studied and proven. However, its association with allergic rhinitis remains controversial. Objective. The main objective of this study is to determine the prevalence of allergic rhinitis in children with persistent otitis media with effusion. This study is also aimed to identify the risk factors of otitis media with effusion, common allergens associated with allergic rhinitis and determine the hearing threshold of children with otitis media with effusion. Methods.A hundred and thirty children were recruited. History taking, physical examination and hearing assessment were done in the first visit. Those with allergic rhinitis underwent skin prick test and treated with intranasal corticosteroid and antihistamine. A second examination and hearing assessment were then repeated after 3 months. Results.The prevalence of allergic rhinitis in children with persistent otitis media with effusion in this study was noted to be 80.3%. Among these children, dust mites appeared to be the most common allergen (87.7%). Another risk factor appeared to be families with more than 4 members per-household (96%). It is noted that that otitis media with effusion caused a hearing loss up to 33 dB. However, there was a statistically significant improvement of the hearing threshold during second visit after commencement of allergy treatment. It was also noted that the hearing threshold in allergic rhinitis group was significantly impaired compared to the non-allergic rhinitis group. Conclusions.Allergic rhinitis and larger family household appeared to be common risk factors in children with persistent otitis media with effusion. There is significant hearing loss noted in children suffering from otitis media with effusion and allergic rhinitis. The hearing threshold improved remarkably with medical therapy. This study hence clarifies the controversy on the association between allergic rhinitis and otitis media with effusion.

Long-term status of middle-ear aeration post canal wall down mastoidectomy.

BACKGROUND: Retraction pocket theory is the most acceptable theory for cholesteatoma formation. Canal wall down mastoidectomy is widely performed for cholesteatoma removal. Post-operatively, each patient with canal wall down mastoidectomy has an exteriorised mastoid cavity, exteriorised attic, neo-tympanic membrane and shallow neo-middle ear. OBJECTIVE: This study aimed to clinically assess the status of the neo-tympanic membrane and the exteriorised attic following canal wall down mastoidectomy. METHODS: All post canal wall down mastoidectomy patients were recruited and otoendoscopy was performed to assess the neo-tympanic membrane. A clinical classification of the overall status of middle-ear aeration following canal wall down mastoidectomy was formulated. RESULTS: Twenty-five ears were included in the study. Ninety-two per cent of cases showed some degree of neo-tympanic membrane retraction, ranging from mild to very severe. CONCLUSION: After more than six months following canal wall down mastoidectomy, the degree of retracted neo-tympanic membranes and exteriorised attics was significant. Eustachian tube dysfunction leading to negative middle-ear aeration was present even after the canal wall down procedure. However, there was no development of cholesteatoma, despite persistent retraction.

Aetiological profile of paediatric stridor in a Malaysian tertiary hospital.

BACKGROUND: Conditions causing stridor in paediatric patients can range from minor illnesses to life-threatening disorders. Proper evaluation and correct diagnosis are essential for timely intervention. The objective of this study was to determine the aetiological profiles and the management of paediatric patients with stridor referred to the Otorhinolaryngology Department of Hospital Serdang. METHODS: Medical records of all paediatric patients presenting with symptom of stridor from January 2010 to February 2015 were reviewed retrospectively. The patients' demographic data, clinical notes, laryngoscope findings, diagnosis and management were retrieved and analysed. RESULTS: Out of the total 137 patients referred for noisy breathing, 121 patients had stridor and were included in this study. There were 73 males and 48 females-most were of Malay ethnicity (77.7%). The age of presentation ranged from newborn to 10 years, with a mean of 4.9 months. Eighteen patients (14.9%) had associated congenital pathologies. The majority were congenital causes (90.9%), in which laryngomalacia was the commonest (78.5%), followed by subglottic stenosis (5.0%), vallecular cyst (2.5%) and congenital vocal fold paralysis (2.5%). Twelve patients (9.9%) had synchronous airway lesion. The majority of the patients were managed conservatively. Thirty-one patients (25.6%) required surgical intervention, of which only one needed tracheostomy. CONCLUSION: Laryngomalacia was the commonest cause of stridor among paediatric patients. A synchronous airway lesion should be considered if the child has persistent or severe symptoms. The majority of the patients were managed conservatively.

An unexpected lesion in cerebellopontine angle: hemangiopericytoma.

hemangiopericytoma (hPC) is a rare tumor by definition and intracranial hPC makes up to less than one percent of all the intracranial tumors. It is a dural base tumor and its clinical features and radiological findings are similar to meningiomas. however, cerebellopontine angle hemangipericytoma had only been reported twice and would almost always be misdiagnosed. definite diagnosis is important, as the treatment of hPC is different from meningiomas and acoustic neuromas. we report a case of a young female who presented with atypical symptoms of left cerebellopontine angle mass. A literature review of the nature of the disease, radiological findings, immunohistochemical features and treatment options of the tumor are described.

Partial Cricotracheal Resection (PCTR), a Rewarding Outcome for Paediatric Subglottic Stenosis: An Early Experience.

This is a retrospective study examining the outcome of paediatric patients with subglottic stenosis who underwent partial cricotracheal resection (PCTR) as a primary open procedure from 2004 to 2012. There were 5 patients identified aged from 3 to 18 years old. All the subglottic stenosis were acquired type. All of them were secondary to prolonged intubation. Three patients were classified as Myer-Cotton grade III and the other two were Myer-Cotton grade IV. Two of the patients had concomitant bilateral vocal cord immobility. All patients underwent two staged PCTR . All patients underwent two staged PCTR, and one patient underwent posterior cordectomy apart from partial CTR at different setting. All patients were successfully decannulated at various durations postoperatively. Although this is an early experience in our institution, PCTR has shown to be effective and safe procedure in patients with subglottic stenosis especially those with Myer-Cotton grade III and IV.

The Prevalence of Sensorineural Hearing Loss in ß-thalassaemia patient treated with Desferrioxamine.

OBJECTIVE: This study aimed to evaluate the prevalence of sensorineural hearing loss (SNHL) in ß-thalassaemia patients treated with Desferrioxamine (DFO) and determine the correlation of SNHL with average daily DFO dosage, serum ferritin level and Therapeutic index (T.I). METHODS: This is a cross sectional descriptive study carried out for a period of 14 months and 54 patients were recruited. The recruited patients are transfusion dependant ß- thalassaemia patient aged 3 years and above treated with DFO. An interview, clinical examination and hearing assessment, which included tympanogram, and Pure Tone Audiometry (PTA) or behaviour alaudiometry were performed. The data on age started on DFO, average daily DFO, duration of DFO intake, serum ferritin past 1 year and Therapeutic Index (T.I) were obtained from patients' case notes. RESULTS: The prevalence of SNHL was 57.4% and majority has mild hearing loss (93.6%). Fourteen patients (25.9%) have bilateral ear involvement and as many as 17 patients (31.5%) have SNHL in either ear. A total of 23 patients (42.6%) have normal hearing level. Although the prevalence of SNHL was 57.4%, only a small percentage of the patient noticed and complained of hearing loss (11.1%). There is no association between age started on DFO, average daily DFO and duration of DFO intake with normal hearing group and those patients with SNHL. Positive correlation was seen between average daily DFO with 2000 and 4000Hz on PTA in the left ear and between serum ferritin level past 1 year with 4000 and 8000Hz in the right ear and 8000Hz in the left ear. No significant correlation was seen between T.I on PTA. CONCLUSION: The prevalence of SNHL from hearing assessment is high in ß-thalassaemia patients in this study. However, it is manifested clinically in a smaller percentage. We suggest a baseline hearing assessment should be carried on all ß-thalassaemia patients prior to DFO chelation therapy.

Quality of life assessment in patients with moderate to severe allergic rhinitis treated with montelukast and/or intranasal steroids: a randomised, double-blind, placebo-controlled study.

OBJECTIVE: This study investigated improvements in quality of life associated with eight weeks of montelukast and/or intranasal steroid treatment for moderate to severe allergic rhinitis. METHODS: A single-centre, prospective, randomised, double-blind, placebo-controlled study was carried out. Assessments were made using the Rhinoconjunctivitis Quality of Life Questionnaire and symptom scales. RESULTS: A total of 128 patients (aged 13-51 years) were randomly assigned to one of two groups. In the montelukast group, patients were treated with montelukast tablets and fluticasone propionate nasal spray (n = 64). In the placebo group, treatment comprised a placebo and fluticasone propionate. The results showed significant improvements in symptom scores and quality of life scores for both groups after one month and two months of treatment, compared with baseline values; these improvements were significantly greater for the montelukast group compared with the placebo group. The mean number of loratadine tablets taken by each patient during the study period was only 0.73 for the montelukast group compared with 9 for the placebo group. CONCLUSION: The combination of montelukast tablets and fluticasone propionate nasal spray improved symptom control and overall quality of life for moderate to severe allergic rhinitis patients.

Surgical outcome of bone anchored hearing aid (baha) implant surgery: a 10 years experience.

Bone anchored hearing aid (Baha) implant is an option for patient with canal atresia, single sided deafness(SSD) and chronically discharging ears despite treatments. This retrospective study was conducted from 2001 to 2011 to evaluate the surgical outcome of Baha implant surgery. Thirty-three patients were identified during this study period. Their age at implantation ranged from 5 to 40 years. Of 33 patients, 29 (87.9 %) patients had bilateral microtia and canal atresia, 3 (9.1 %) patients had unilateral microtia and canal atresia and 1 (3.0 %) patients have SSD following labyrinthitis. One patient (3.2 %) had major complication which is lost of implant due to failure of osseointegration. Soft tissue reactions were seen 7 patients (21.1 %). Of these 7 patients, 4 patients required 3-4 procedures as day care operation for excision of the skin overgrowth surrounding the abutment. Recurrent antibiotic treatment was required in 3 patients (9.7 %). None of our patient had history of intraoperative or peri-operative complication following Baha surgery. The commonest complications are local infection and inflammation at the implant site. None of our patient had history of intraoperative or peri-operative complication following Baha implant surgery.

Primary clear cell carcinoma of minor salivary gland of the soft palate: a case report.

Clear cells can be found in numerous salivary and non-salivary tumors in the head and neck region, including metastatic lesions. They are rare low-grade tumors accounting for less than 1% of all salivary gland tumors and occur almost exclusively in the intra-oral minor salivary glands. Hyalinizing clear cell carcinoma (HCCC) is an extremely rare and recently described neoplasm predominantly affecting the oral cavity. Histologically, it is characterized by nests of glycogen-rich monomorphic clear cells within a hyaline stroma. HCCC often follows an indolent course with a limited metastatic potential. It is therefore important to differentiate this entity from other more aggressive clear cell tumors including metastatic tumors such as renal cell carcinoma. We hereby report a case of HCCC localized in minor salivary glands specifically in soft palate for its rarity, as well as to discuss the role of immunohistochemical stains, essential for its definitive diagnosis.

Stem cell genes are poorly expressed in chondrocytes from microtic cartilage.

OBJECTIVES: This study was aimed to see the difference between chondrocytes from normal cartilage compared to chondrocytes from microtic cartilage. Specific attentions were to characterize the growth of chondrocytes in terms of cell morphology, growth profile and RT-PCR analysis. STUDY DESIGN: Laboratory experiment using auricular chondrocytes. METHODS: Chondrocytes were isolated from normal and microtic human auricular cartilage after ear reconstructive surgeries carried out at the Universiti Kebangsaan Malaysia Medical Centre. Chondrocytes were cultured in vitro and subcultured until passage 4. Upon confluency, cultured chondrocytes at each passage (P1, P2, P3 and P4) were harvested and subjected to growth profile and gene expression analyses. Comparison was made between the microtic and normal chondrocytes. RESULTS: For growth profile analysis cell viability did not show significant differences between both samples. There are no significance differences between both samples in terms of its growth rate, except in passage 1 where microtic chondrocytes were significant lower in their growth rate. Population doubling time and total number of cell doubling of all samples also did not show any significant differences. Gene expression is measured using Real Time-Reverse Transcriptase Polymerase Chain Reaction (RT-PCR). There is no significant differences in the expression of collagen type I, collagen type II, collagen type X, aggrecan core protein, elastin and sox9 genes in both samples. There are significant lower in the expression of sox2, nestin, BST-1 and OCT-4 gene in microtic chondrocytes compared to the normal chondrocytes. Stem cells markers are included in this study as stemness in cells may imply a greater proliferative potential and plasticity in vitro. CONCLUSION: Chondrocytes from microtic samples have the same properties as chondrocytes from normal samples and hold promises to be used as a starting material in the reconstruction of the external ear in future clinical application. The reduction in sox2, nestin, BST-1 and OCT-4 gene expression in microtic samples could be the possible cause of the arrested development of the external ear.

Unilateral tongue angioedema caused by angiotensin-converting enzyme inhibitor.

OBJECTIVE: We report a case of an elderly man receiving treatment with perindopril, who presented with angioedema of the left side of the tongue, floor of the mouth and upper neck. This affected his speech and swallowing, and occurred one day after a burr hole and evacuation procedure undertaken to treat a subdural haematoma. The patient was kept under close observation and treated with intravenous hydrocortisone. The angioedema resolved completely in two days. This is the third reported case of unilateral tongue angioedema occurring secondary to angiotensin-converting enzyme inhibitor use. METHOD: Case report and literature review concerning angiotensin-converting enzyme inhibitor induced angioedema. CONCLUSION: Unilateral angioedema of the tongue is a rare adverse reaction to angiotensin-converting enzyme inhibitors. Early recognition may prevent unnecessary surgical intervention and complications.

Congenital cholesteatoma: delayed diagnosis and its consequences.

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.

A ten year retrospective review of orbital complications secondary to acute sinusitis in children.

Acute sinusitis is most often a mild self-limiting disease. However, it may progress into severe and life threatening complications. One of the commonest being orbital complication of which visual loss is a direct consequence. In this 10 year retrospective study, the nature of orbital complication, clinical presentation and treatment modalities and outcome seen in children with acute sinusitis in a tertiary referral institute were reviewed. Of six patients, there was a case of preseptal cellulitis, 4 cases of subperiosteal abscess and one case of orbital abscess. Periorbital swelling was a common presenting feature. In 5 cases this was associated with proptosis with one case of impending optic nerve compression. The value of computed tomography and opthalmological examination as a component in the management plan is highlighted. All patients were treated with intravenous antibiotics but evidence of abscess collection warranted urgent surgical drainage in 5 patients, 3 being endoscopic drainage while external approach was done for the remaining 2 patients. Thus a child exhibiting orbital complication of acute sinusitis, prompt diagnosis and treatment is essential in obtaining the best outcome for the child.

Hearing rehabilitation in congenital canal atresia.

INTRODUCTION: The purpose of this study was to review the results of our patients with congenital canal atresia after implantation of bone-anchored hearing aids (BAHA). The occurrence of complications was also reviewed. METHODS: This was a retrospective analysis of the first 16 patients who had BAHA implantation at Universiti Kebangsaan Malaysia Medical Centre, Malaysia. Audiometric assessment was done preoperatively and postoperatively for each patient using the standard procedure. The surgical procedure was described and its complications discussed. RESULTS: The 16 patients consisted of 11 male and five female patients. Their mean age was 8.9 years at the time of the surgery. The main indication was bilateral canal atresia. 11 patients had implantation of BAHA performed in two stages, while the other five patients had it as a single-staged procedure. The complications that occurred were failure of osseointegration (one patient), granulation tissue overgrowth into the abutment (two patients) and cellulitis surrounding the abutment (three patients). The average preoperative unaided air conduction threshold was 64.9 dB and the average postoperative aided hearing threshold was 29.7 dB. The overall mean functional gain was 35.2 dB. CONCLUSION: BAHA has many advantages over the conventional hearing aid in the form of cosmesis, discomfort and hearing gain. It is a reliable hearing rehabilitation tool with good predictable hearing outcome in patients with bilateral canal atresia, especially those unsuitable for canalplasty. Despite its higher cost and the need for surgical implantation, its use is justifiable in properly selected patients.

Metachronous inflammatory myofibroblastic tumour in the temporal bone: case report.

OBJECTIVE: We report an extremely rare case of metachronous inflammatory myofibroblastic tumour in the temporal bone. METHOD: Case report and review of the world literature on metachronous inflammatory myofibroblastic tumour. RESULTS: Inflammatory myofibroblastic tumour in the temporal bone is rare, and metachronous inflammatory myofibroblastic tumour in the temporal bone has never been reported in the English medical literature. We report a case of inflammatory myofibroblastic tumour in the right temporal bone in a 27-year-old woman presenting with right-sided otalgia and progressive hearing loss. A metachronous lesion was discovered in the left temporal bone one year later. The patient underwent surgical excision of the tumour via canal wall down mastoidectomy for both lesions. Long term steroids were prescribed after both surgical procedures. At follow up three years after the last procedure, the patient remained free of disease. CONCLUSION: To the best of our knowledge, this is the first reported case of metachronous inflammatory myofibroblastic tumour in the temporal bone.

Retrospective review of surgical management of foreign body ingestion.

Endoscopic examination and removal of foreign body under general anaesthesia are recommended for persistent symptomatic patient with or without significant findings on radiological examination. This report evaluates the management outcome of surgical removal of foreign body ingestion in upper gastrointestinal tract. A total of 70 cases with full documentation were reviewed retrospectively from June 1998 until December 2007. There were 32 male and 38 female with age range from 6 months to 87 years old (mean: 36.9 years). Sixty five patients (93%) were adults and 15 (7%) were below 13 years. Fish bones were the most common foreign body found (44.3%). Radiologically, foreign bodies were highly suspicious in 51 cases (76.1%). Intraoperatively, thirty six cases (70.6%) were positive. From 16 cases (23.9%) with normal radiograph, 10 cases (62.5%) were found to have foreign bodies. Therefore the plain radiograph is helpful, but clinical presentation is more reliable to determine surgical removal under general anaesthesia.

Growth kinetic study on normal and microtic chondrocytes of human auricular cartilage.

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.

Parotid abscess: a five-year review--clinical presentation, diagnosis and management.

Parotid abscess is an uncommon complication of suppurative infection of the parotid gland parenchyma, commonly bacterial or viral. Ductal ectasis, primary parenchymal involvement, or infection of the intraparotid or periparotid lymph nodes can result in abscess formation. Parotid abscess may arise from ductal ectasis, primary parenchymal involvement, or infection of the subcapsular lymph nodes. The operative records for all the patients who underwent surgeries in the Department of Otorhinolaryngology, Head and Neck Surgery of the National University Hospital, Kuala Lumpur, Malaysia between January 2001 and December 2005 were retrospectively reviewed. Our case series comprises 15 patients, with 10 males and five females with a median age at presentation of 51 years old. Diabetes mellitus is a significant comorbid factor, with six patients being diabetics. Among the diabetics, two patients presented with facial nerve palsy and one of them also died due to overwhelming septicaemia. Here, we discuss the presenting symptoms, predisposing factors, investigations, microbiology and complications of this condition.

Internal auditory canal stenosis in congenital sensorineural hearing loss.

OBJECTIVE: Sensorineural hearing loss (SNHL) due to internal auditory canal (IAC) stenosis with hypoplasia of the cochleovestibular nerve is a rare disorder. The diagnosis of the IAC stenosis requires both high resolution computed tomography scan (HRCT) and magnetic resonance imaging (MRI). METHODS: A retrospective review over 6 years in an academic tertiary referral center was performed. RESULTS: Six patients with congenital SNHL were diagnosed with congenital IAC stenosis. Four had unilateral and two had bilateral IAC stenosis after imaging. MRI showed hypoplastic vestibulocochlear nerve in all cases. CONCLUSIONS: This paper highlights the importance of imaging in diagnosing IAC stenosis and detecting the presence of cochleovestibular nerve in cases of congenital SNHL.