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.

Vascular loop in the cerebellopontine angle causing pulsatile tinnitus and headache: a case report.

Tinnitus is a common disorder, it can be classified as pulsatile and non-pulsatile or objective and subjective. Pulsatile tinnitus is less common than non-pulsatile and can be due to vascular tumour such as glomus or vascular abnormality. We presented an interesting case of a 30 year-old Malay lady with a two-year history of pulsatile tinnitus which was worsening in three months duration. It was associated with intermittent headache. Clinical examination and tuning fork test were unremarkable. Apart from mild hearing loss at high frequency on the left ear, the pure tone audiogram (PTA) was otherwise normal. In view of the patient's young age with no risk factor for high frequency loss, a magnetic resonance imaging (MRI) was performed to look for any abnormality in the cerebellopontine angle. It revealed a single vessel looping around the left vestibulocochlear and facial nerves at the cisternal portion, likely a branch of the anteroinferior cerebellar artery (AICA). Literature review on the pathophysiology and treatment option in this condition is discussed.

Management of congenital choanal atresia (CCA) after multiple failures: A Case Report.

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.