Bilateral microtia, canal atresia and aplasia of cochleovestibular nerve.

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.

The Correlation Between Pre and Postoperative Hearing Level with High Resolution Computed Tomography (HRCT) Findings in Congenital Canal Atresia (CAA) Patients.

In managing patient with congenital congenital aural atresia (CAA), preoperative high resolution computed tomography (HRCT) scan and hearing assessment are important. A grading system based on HRCT findings was first introduced by Jahrsdoefer in order to select appropriate candidates for operation and to predict the postoperative hearing outcome in CAA patients. The score of eight and more was considered as a good prognostic factor for hearing reconstruction surgery. However previously in our center this score was not used as the criteria for surgical procedure. This study was conducted at Center A to evaluate the correlation between pre and postoperative hearing level with HRCT based on a Jahrsdoefer grading system in patients with CAA. All records and HRCT films with CAA from January 1997 until December 2007 at Center A were evaluated. The demographic data, operative records, pre and post operative hearing levels and HRCT findings were analyzed. Hearing level in this study was based on a pure tone average of air-bone gap at 500 Hz, 1 kHz and 2 kHz or hearing level obtained from auditory brainstem response eudiometry. This study was approved by Research Ethics Committee (code number, FF-197-2008). Thirty-two ears were retrospectively evaluated. The postoperative hearing level of 30 dB and less was considered as successful hearing result postoperatively. Of the six ears which underwent canalplasty, three had achieved successful hearing result. However, there was no significant correlation between preoperative hearing level (HL) with HRCT score and postoperative HL with HRCT score at 0.05 significant levels (correlation coefficient = -0.292, P = 0.105 and correlation coefficient = -0.127, P = 0.810) respectively. Hearing evaluation and HRCT temporal bone are two independent evaluations for the patients with CAA before going for hearing reconstructive surgery.

Cholesteatoma in patients with congenital external auditory canal anomalies: retrospective review.

OBJECTIVE: To review cases of congenital external auditory canal anomaly with cholesteatoma, documenting clinical presentation, cholesteatoma site and extent, complications, and surgery. METHOD: Retrospective review of all cases of congenital canal anomaly with cholesteatoma treated between 1998 and 2009. RESULTS: Of 41 cases with canal anomalies, 17 (43.9 per cent) had associated cholesteatoma. Medical records were unretrievable for four cases. Of the remaining 13 patients (five females and eight males, age range four to 73 years, mean 21 years), 10 presented chiefly with recurrent otorrhoea, two with postauricular discharge from mastoid abscess, and one with otalgia, postauricular tenderness and neck stiffness. Hearing loss was conductive in 10 patients (76.9 per cent) and sensorineural (severe to profound) in three. No facial nerve palsy was documented. Cholesteatoma was seen in all cases on high resolution computed tomography, and confirmed intra-operatively. Six patients underwent canalplasty with split skin grafting, and seven modified radical mastoidectomy. Six patients recovered well, two needed repeated canalplasty for soft tissue restenosis, and five needed cautery and split skin grafting for mastoid cavity granulation tissue. CONCLUSION: Congenital canal anomaly is uncommon. Canal cholesteatoma should be suspected in all cases, and high resolution temporal bone computed tomography undertaken in all patients aged four years or more. In patients with otorrhoea, the risk of cholesteatoma is greater. Treatment is generally surgery; the type depends on the disease extent.

A Single Blinded Randomized Controlled Study of the Effect of Conventional Oral Hypoglycemic Agents Versus Intensive Short-Term Insulin Therapy on Pure Tone Audiometry in Type II Diabetes Mellitus.

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 < 0.05). This study has shown that glycaemic control does not have significant impact on hearing. The hearing threshold is neither affected by insulin treatment nor by the glycaemic control.

Complete congenital third branchial fistula: does the theoretical course apply?

The course of a third branchial fistula is derived from its embryological origin, in accordance with the branchial apparatus theory. Treatment of this condition requires complete removal of the tract in order to avoid recurrence; however, this can pose a risk to the surrounding structures. We report the case of a complete third branchial fistula as well as a literature review on its theoretical course and management.

Tracheal agenesis: a rare cause of unsuccessful tracheal intubation during resuscitation.

Tracheal agenesis is a rare congenital airway anomaly that usually results in a fatal outcome. The diagnosis is usually made through post-mortem examination. In the current literature, there has been no reported long-term survival although a few reports claimed prolongation of life of several hours to days. This condition is commonly associated with premature birth, polyhydramnios and a male predominance. In 90% of the cases, it is associated with multiple cardiovascular, gastrointestinal and genitourinary tract anomalies which are incompatible with life. We report a case of a premature newborn with severe respiratory distress, absent cry and cyanosis soon after birth. Attempts at endotracheal intubation failed as it was no possible to negotiate the tube beyond the vocal cords. Needle cricothyrotomy and attempted tracheostomy also failed to secure the airway. The diagnosis was confirmed at post-mortem examination.

Necrobiotic xanthogranuloma of the parotid gland.

OBJECTIVES: To highlight the first reported case of necrobiotic xanthogranuloma of the parotid gland. We also review the clinical presentations and treatments for this rare condition. METHOD: Case report and review of necrobiotic xanthogranuloma. RESULTS: A 48-year-old man presented with a right parotid mass. Fine needle aspiration cytology was suggestive of Warthin's tumour, for which the patient underwent a subtotal parotidectomy. The final histopathological diagnosis was necrobiotic xanthogranuloma. CONCLUSIONS: Necrobiotic xanthogranuloma may clinically mimic commoner tumours such as Warthin's tumour. Once diagnosed, the clinician should be wary of extracutaneous manifestations and paraproteinaemias. Because of the variability of presentation, there is no consensus on the best treatment for necrobiotic xanthogranuloma, which may include surgery, chemotherapy, interferon, plasmapheresis and radiation therapy.

Oncocytic Schneiderian Papilloma: A Rare Sinonasal Papilloma Removed via Endoscopic Medial Maxillectomy

There are three types of papilloma which arises from the Schneiderian membrane namely inverted, fungiform and oncocytic. Oncocytic papilloma is the rarest type and occurs predominantly in the older age group. Clinically, its behaviour is similar to inverted papilloma in having both local recurrence and associated malignancy. We report a rare case of oncocytic papilloma arising from the maxillary sinus, extending into the nasal cavity and nasopharynx, in a 78-year-old lady. Despite the longstanding history there was no evidence of associated malignancy in this lesion. An endoscopic medial maxillectomy was successfully utilized for approach and complete tumour excision. In conclusion, oncocytic papilloma should be considered as a differential diagnosis in patients presenting with unilateral polypoid nasal lesions especially in the elderly. In addition, endoscopic medial maxillectomy can provide an adequate approach for complete tumour removal.

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.

Iatrogenic facial nerve palsy: lessons to learn.

INTRODUCTION: This study aims to review the management and discuss the outcome of patients with iatrogenic facial nerve palsy. METHODS: 11 patients with iatrogenic facial nerve palsy (FNP) were evaluated retrospectively in a tertiary centre between June 1995 and September 2008. All the cases were referred from other centres. RESULTS: Ten patients had iatrogenic immediate FNP secondary to mastoidectomy and one had FNP secondary to superficial parotidectomy. Of the ten cases, three had concomitant profound sensorineural hearing loss and one had concomitant labyrinthine fistula. Ten patients underwent facial nerve exploration and one patient was managed conservatively. The second genu was the commonest site of injury (60 percent). Facial nerve recoveries were achieved to Grade I House Brackmann classification in five cases, Grade II in two cases and Grade III in two cases postoperatively. One case defaulted follow-up. One patient, managed conservatively, recovered to FNP Grade II after five months post-injury. CONCLUSION: Mistakes that most likely occurred during mastoid surgery are drilling towards the antrum, causing injury to the facial nerve at the second genu. Early facial nerve exploration and neurolysis resulted in good facial nerve recovery.

Retropharyngeal Abscess: A Seven-Year Review - Clinical Presentation, Diagnosis and Management

This was a retrospective study of operative records between 2000 – 2007 from two ear, nose and throat (ENT) referral centers in Malaysia to review demographic patterns, clinical presentations and management of retropharyngeal abscess (RPA). Our case series comprised of eight patients with five females and three males with a median age at presentation of 47 years. Comorbid factor in this series was diabetes mellitus, with four patients having diabetes. There were three cases of fish bone ingestion. The commonest presentation was dysphagia or odynophagia. None of them had fever. All patients were treated with broad spectrum intravenous antibiotics (IV) and drainage was performed for the abscess. Four patients underwent transoral drainage with three having the cervical approach and one having spontaneous rupture. All patients recovered well from the infection and only one patient passed away due to septicaemia. In conclusion, patients with retropharyngeal abscess may present with vague symptoms of sore throat, odynophagia without fever due to partial antibiotics treatment. A correct diagnosis should be made so that adequate treatment can be given to prevent mortality.

Derivation of cochlea hair cell for in vitro expansion and characterization.

A potential cure for hearing loss would be to regenerate hair cells by stimulating cells of the damaged inner ear sensory epithelia to proliferate and differentiate into hair cells. Here, we investigated the possibility to isolate, culture-expand and characterize the cells from the cochlea membrane of adult mice. Our results showed that the cultured cells isolated from mouse cochlea membrane were heterogenous in nature. Morphologically there were epithelial like cells, hair cell like, nerve cell like and fibroblastic cells observed in the culture. The cultured cells were immunopositive for specific hair cell markers including Myosin 7a, Calretinin and Espin.

Sinonasal hybrid tumour involving the anterior skull base.

OBJECTIVE: We report a rare case of sinonasal hybrid tumour within an inverted papilloma. METHOD: The patient's case history and course of management are presented. The relevant medical literature are reviewed and discussed. RESULTS: A 60-year-old man presented with unilateral nasal obstruction associated with anosmia. An ENT examination revealed a unilateral, left nasal mass. A provisional diagnosis of inverted papilloma was made. The patient underwent a transnasal endoscopic excision of the tumour, which was unfortunately complicated by a cerebrospinal fluid leak. Further treatment of this patient is described. We highlight the features of associated malignancy in inverted papilloma, and the importance of thorough histopathological examination of tissue specimens. CONCLUSION: Treatment of inverted papilloma with limited involvement of the skull base can be successfully achieved by endoscopic excision. A high index of clinical suspicion, together with meticulous histopathological examination, will enable diagnosis of associated malignancy. This will ensure that appropriate adjuvant treatment is given, resulting in a good clinical outcome.

Derivation of cochlea hair cell for in vitro expansion and characterization

A potential cure for hearing loss would be to regenerate hair cells by stimulating cells of the damaged inner ear sensory epithelia to proliferate and differentiate into hair cells. Here, we investigated the possibility to isolate, culture-expand and characterize the cells from the cochlea membrane of adult mice. Our results showed that the cultured cells isolated from mouse cochlea membrane were heterogenous in nature. Morphologically there were epithelial like cells, hair cell like, nerve cell like and fibroblastic cells observed in the culture. The cultured cells were immunopositive for specific hair cell markers including Myosin 7a, Calretinin and Espin.

Pleomorphic Adenoma Originating from Submandibular Salivary Gland in an 8-year-old Girl: A Case Report

Pleomorphic adenoma (PA) typically presents as a benign slow growing, painless neoplasm of the parotid gland. PA arising from the submandibular gland in the paediatric age group is rare. Surgical excision is the treatment of choice. A case of PA in an 8-yearold Malay girl is presented. Clinically she presented with a painless right submandibular mass measuring 2x2cm. A diagnosis of Pleomorphic adenoma was made on Fine Needle Aspiration Cytology (FNAC). She underwent submandibulectomy uneventfully. Postoperatively after two years follow up, she is asymptomatic.

Neck abscess: five year retrospective review of Hospital University Kebangsaan Malaysia experience.

The most commonly involved space was the parapharyngeal and superficial anterior triangle followed by submandibular, retropharyngeal, posterior triangle and submental spaces respectively. Thirty-three percent of patients had diabetes mellitus as a predisposing factor. More than half of them had no known aetiological cause for the neck abscess. We encountered one mortality in an elderly patient with diabetes who succumbed to overwhelming septicaemia despite early abscess drainage and intensive medical treatment.

Endonasal endoscopic repair of anterior skull-base fistulas: the Kuala Lumpur experience.

The purpose of this retrospective study is to determine the pattern of cerebrospinal fluid (CSF) rhinorrhoea presenting to our tertiary referral centre in Kuala Lumpur and to assess the clinical outcomes of endonasal endoscopic surgery for repair of anterior skull base fistulas. Sixteen patients were treated between 1998 and 2004. The aetiology of the condition was spontaneous in seven and acquired in nine patients. In the acquired category, three patients had accidental trauma and this was iatrogenic in six patients (five post pituitary surgery), with one post endoscopic sinus surgery (ESS). Imaging included computed tomography (CT) scan and magnetic resonance imaging (MRI). Endoscopic repair is less suited for defects in the frontal sinuses with prominent lateral extension and defects greater than 1.5 cm in diameter involving the skull base. Fascia lata, middle turbinate mucosa, nasal perichondrium and ear fat ('bath plug') were the preferred repair materials in the anterior skull base, whereas fascia lata, cartilage and abdominal fat obliteration was preferentially used in the sphenoid leak repair. Intrathecal sodium flourescein helped to confirm the site of CSF fistula in 81.3 per cent of the patients. Ninety per cent of the patients who underwent 'bath plug' repair were successful. The overall success rate for a primary endoscopic procedure was 87.5 per cent, although in two cases a second endoscopic procedure was required for closure. In the majority of cases endoscopic repair was successful, and this avoids many of the complications associated with craniotomy, particularly in a young population. Therefore it is our preferred option, but an alternative procedure should be utilized should this prove necessary.

Sequelae of traumatic laryngotracheal separation: the need for individualized long term treatment plan.

Laryngotracheal separation is a rare variant of laryngeal trauma. However it is life threatening and potentially fatal. Patients with this injury usually succumb at the site of the accident itself. Here we present two cases of laryngotracheal separation of different etiology and of different outcomes. The treatment advocated for laryngotracheal separation is initially airway stabilization followed by formal repair of the transected trachea. However both our cases illustrates that the outcomes can be different and that a long term treatment plan should be individualized to each patient.

Cervical necrotizing fasciitis due to klebsiella.

Necrotizing fasciitis is an aggressive infection involving fascia and subcutaneous tissue thus causing infectious thrombosis of the nutrient vessels to the overlying skin ultimately leading to extensive necrosis. Although necrotizing fasciitis is common in the perineum, abdominal wall and extremities it is relatively uncommon in the head and neck region. This is a case report of a 65 year old female visiting from India who presented with diabetic ketoacidosis precipitated by cervical necrotizing fasciitis. Computed tomography imaging showed involvement of the left parapharyngeal space and there was soft tissue gas present. This patient subsequently underwent an emergency neck exploration and desloughing. Repeated pus culture studies isolated Klebsiella species as the sole pathogen. Our case illustrates that prompt diagnosis and treatment of cervical necrotizing fasciitis can ensure a good outcome.