Three cases of embryonal rhabdomyosarcoma in the middle ear and mastoid in children are presented. Diagnosis was confirmed by histopathology. A multidisciplinary approach employing surgery, chemotherapy and radiation therapy is the method of choice in the management of this rare and highly lethal condition.
A patient with Turner's syndrome presented with a rare anomaly of absent oval window, inferiorly placed facial nerve, and abnormal stapes. To our knowledge, this is the first report of this combination of malformations.
Clinical presentation of Menière's disease in children is not as typical as in adults. The triad of vertigo, tinnitus and deafness are not usually elicited, diagnosis often being made after years of follow up and batteries of investigation. A case of Menière's disease in a 3-year-old boy is presented. The diagnosis was only obvious at the age of 8 when the triad of vertigo, deafness and tinnitus were present. His disease progressed despite a trial of intratympanic gentamicin injections and endolymphatic sac decompression. Vestibular nerve section was subsequently performed for his intractable disease. Following the procedure he was asymptomatic and able to attend school.
Two cases of glomus tympanium tumor of the middle ear stage 'type A' according to Fisch classification, are presented due to their rarity in this part of the world. These tumors were excised by end aural tympanotomy approach and there has been no recurrence in both patients to date.
Inverted papilloma is a rare, benign tumour representing only 0.5 to 4 per cent of all sinonasal neoplasms; its involvement of the middle ear is extremely rare. We present a case of multicentric inverted papilloma in the sinonasal region and middle ear in a 54-year-old man. The patient later developed neck metastasis secondary to malignant transformation of the inverted papilloma in the middle ear.
Paragangliomas are slow growing hypervascular tumour arising from neural crest cell derivatives throughout the body. In the head and neck region, the major paraganglial cells are located at the carotid bifurcation (carotid body), along the ganglia of the vagus nerve and along the nerves supplying the middle ear and jugular bulb. Less common locations include the larynx, orbit, nose and the aortic arch. Carotid body tumours are very rare neoplasms constituting less than 0.5% of all tumours. The true nature of the tumour is established at the time of attempted biopsy or surgical resection, sometimes with disastrous consequences. Only a few of the more than 500 cases reported in the literature have been studied and diagnosed preoperatively. These tumours must be considered in the evaluation of any lateral neck mass, even one located far from the carotid bifurcation. We report a case of bilateral carotid body tumours detected using 16-slice MDHCT in a patient who presented with pulsatile neck swelling for two years. This tumour is rarely malignant; however it produces serious problems by its progressive enlargement and impairment of adjacent structures in the neck. The treatment of choice is surgical resection; preferably to excise this lesion when it is small, because large, very vascular tumours are intimately attached to the carotid vessels and make surgical resection more hazardous. Pre-operative embolization can be performed in large tumours, as to facilitates surgery and reduce complication. In most of the cases, there is strong attachment of the tumour to the carotid arteries. When this is the situation, removal of the tumour means complete excision of both great arteries as well, and the mortality rate is 40 to 45 %. When surgical removal is not feasible, radiotherapy may help to control the tumour growth, although the results have not been encouraging. As it is very slow growing tumour with the growth rate of less than 5 mm per year tumours in old patients with significant risk factors for surgical intervention can be managed by observation alone.
To determine the characteristic presenting symptoms, otoscopic findings, audiological profiles and the intraoperative findings of children with chronic otitis media with effusion who required surgical intervention. A prospective cross sectional study was undertaken in the otorhinolaryngology clinic of USM Hospital (HUSM) involving 25 cases (50 ears) of children with chronic otitis media with effusion requiring surgical intervention from June 1999 to September 2001. Their ages ranged from 3 to 12 years old. The gender distribution included males at 72 % and females at 28 %. The presenting symptoms noted were hearing impairment (52%), otalgia (18%), ear block (16%) and tinnitus (14%). The otoscopic findings were fluid in the middle ear (40%), dullness (32%) and retraction of the tympanic membrane (28%). On audiometry, 24 ears had moderate deafness (48%), 16 ears had mild deafness (32%) while 4 ears had severe deafness (8%). With tympanometry, 42 ears out of the total 50 had a flat type B curve (84%) while 6 ears had type As curve (12%). During myringotomy, middle ear secretion was seen in 38 ears (76%) out of the 50 ears; 22 ears had mucoid secretion while 16 ears had serous secretion. Clinically, the commonest presenting symptom was hearing impairment (52%) while the most common otoscopic finding was fluid in the middle ear (40%). Audiologically, most patients had moderate conductive hearing loss (48%) and a type B curve (84%) on tympanometry. On myringotomy middle ear fluid was found in 76 % of the ears.
Study site: otorhinolaryngology clinic, Hospital Universiti Sains Malaysia (HUSM), Kelantan, Malaysia
Cholesterol granuloma (CG) is a histologic description of foreign body giant cell formation toward cholesterol crystals. The majority of temporal bone CG is unilateral and most common in the petrous apex. Middle ear CG is usually the result of underlying ear diseases. Primary middle ear CG is very rare. Most reported CG has not been associated with familial hypercholesterolemia (FH). FH, an autosomal dominant disorder, manifests as high levels of serum cholesterol and low density lipoprotein (LDL) cholesterol. We report a rare case of FH and bilateral aggressive primary middle ear CG. This publication has been approved by the IRB, Hospital Alor Setar.
We report a case of High grade Mucoepidermoid carcinoma of the middle ear. A 67 year old gentleman was referred to our centre for persistent otalgia associated with facial weakness for four months. Examination revealed a middle ear mass with House Brackmann Grade 3 Facial Palsy and ipsilateral lymhadenopathy. Biopsies suggested a squamous cell carcinoma of the middle ear, and he successfully underwent an Extended Temporal bone resection with External canal reconstruction and neck dissection. Postoperative Histopathology revealed that the tumour was in fact a High Grade Mucoepidermoid carcinoma. He received postoperative radiotherapy and is well one year post operatively.
A 12-year-old boy with moderate to severe bilateral mixed hearing loss was planned for hearing aid placement. During the process of making ear mould impression, the impression material accidentally entered the right middle ear. Removal of the ear mould impression was possible permeatally under general anaesthesia.
Patients who have had middle-ear or mastoid surgery are at an increased risk of developing cerebrospinal fluid (CSF) otorrhoea. The CSF leak is usually from defects in the tegmen or posterior cranial fossa. We present a patient with CSF otorrhoea following a modified radical mastoidectomy seven years ago. There was an unusual communication between the internal auditory meatus (IAM) and the middle ear. Radiologic imaging like the MRI is useful in identifying the site of leak.
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.
Temporal bone cancer, a relatively rare disease, accounting for less than 0.2% of all tumors of the head and neck and is associated with a poor outcome; often presents in a subtle manner, which may delay diagnosis. It should be suspected in any case of persistent otitis media or otitis externa that fails to improve with adequate treatment. Despite advances in operative technique and postoperative care, long-term survival remains poor). It includes cancers arising from pinna that spreads to the temporal bone, primary tumors of the external auditory canal (EAC), middle ear, mastoid, petrous apex, and metastatic lesions to the temporal bone. Here is a report on a case of temporal bone carcinoma presenting with right otalgia, otorrhea and facial paralysis. The patient was initially diagnosed as mastoiditis and later the clinical impression was revised to temporal bone carcinoma (undifferentiated type), based on the pathologic findings.
Chronic discharging ear, mostly due to middle or external ear infection, is one of the leading causes for seeking healthcare among the paediatric population in a developing country. However, a long-standing forgotten middle ear foreign body forms a rare cause for such presentation demanding a high index of suspicion from the clinicians. Most of them are iatrogenic or accidental, and are removed by conventional permeatal approach; need for tympanotomy is rarely documented in the recent literature. We report the first case where a large stone was introduced into the middle ear through a pre-existing tympanic membrane perforation by the child himself, and only the second documentation of removal of a middle ear foreign body by tympanotomy in a child.
Otitis media with effusion (OME) is a condition characterised by a collection of fluid within the middle ear without signs of acute inflammation. It is common in young children, with a bimodal peak at two and five years of age. Eighty percent of children have at least one episode of OME by the age of 10 years. This disease is a common ear problem among children with craniofacial anomalies including cleft palate and Down syndrome (DS).
This study aims to assess the association between microbial composition, biofilm formation and chronic otorhinolaryngologic disorders in Malaysia. A total of 45 patients with chronic rhinosinusitis, chronic tonsillitis and chronic suppurative otitis media and 15 asymptomatic control patients were studied. Swab samples were obtained from these subjects. Samples were studied by conventional microbiological culturing, PCR-based microbial detection and Confocal Laser Scanning Microscopy (CLSM). Haemophilus influenzae, Staphylococcus aureus, Streptococcus pneumoniae, coagulase-negative staphylococci (CoNS) and other Streptococcus species were detected in subjects of both patient and control groups. Biofilm was observed in approximately half of the smear prepared from swab samples obtained from subjects of the patient group. Most of these were polymicrobial biofilms. S. aureus biofilm was most prevalent among nasal samples while H. influenzae biofilm was more common among ear and throat samples. Results from this study supported the hypothesis that chronic otorhinolaryngologic diseases may be biofilm related. Due to the presence of unculturable bacteria in biofilms present in specimens from ear, nose and throat, the use of molecular methods in combination with conventional microbiological culturing has demonstrated an improvement in the detection of bacteria from such specimens in this study.