Effect of transverse temporal bone fracture on the fluid compartment of the inner ear.

Five temporal bones exhibiting transverse fractures were studied with a view to determining whether such fractures could lead to symptomatic endolymphatic hydrops. Four out of the five temporal bones showed the fracture line traversing the vestibular aqueduct. Two of these four showed complete obstruction of the endolymphatic duct. One of these had an intact membranous labyrinth and severe endolymphatic hydrops. The other had ruptures of the membranous labyrinth and no hydrops. Three ears exhibited partial preservation of auditory and vestibular hair cells and neurons. These findings are consistent with the concept that a transverse fracture may produce endolymphatic hydrops by obstructing the vestibular aqueduct while preserving enough audiovestibular epithelium and neurons to present as symptomatic Menière's disease.

Integrity of the auditory pathway in young children with congenital and postmeningitic deafness.

This study compares the functional integrity of the auditory pathways of congenitally deaf and postmeningitically deaf children. We used the electrical auditory brain stem response evoked by promontory stimulation to assess 49 profoundly deaf children before cochlear implantation. The age at implantation ranged from 21 months to 15 years (mean 4.5 years). The onset of deafness was either congenital or up to the age of 2 years (mean 5 months). The cause of deafness was meningitis in 19 children (39%) and congenital in 30 (61%). The number of children with identifiable waveform components (eV, eIII, and eII) was significantly greater in the congenitally deaf group. We also analyzed the amplitudes, the latencies, and 4 parameters of the amplitude input-output functions. All the statistically significant differences were in favor of better responses in the congenitally deaf children. These results suggest that the functional status of the peripheral neurons of the auditory pathways may be more intact in congenitally deaf children than in postmeningitic children.

Congenital midnasal stenosis in an infant.

Neonates and infants are obligate nasal breathers and nasal obstruction in this age group can be a life threatening emergency. Even though the commonest cause for nasal obstruction is nasal oedema, bony stenosis of the posterior choanae or pyriform aperture is quite common and may be seen on CT scans. We describe a case of mid-nasal stenosis in a neonate and discuss the aetiology and management of nasal obstruction in this age group.

Retrospective study of the prevalence of bilateral sensorineural deafness in childhood.

This retrospective study was undertaken to estimate the causes of severe sensorineural deafness (greater than 40 dB HL) in children. The children were identified from case notes of all referrals made to the Children's Hearing Assessment Centre during a 5-year period (from 1st January 1981 to 31st December 1985). Results were tabulated and conclusions drawn following a literature search. It was deduced that following better antenatal screening, a more active immunisation program and improvements in resuscitation of small preterm infants, there was a shift of cases from prenatal to perinatal causes. The proportion of cases of severe sensorineural deafness was much the same as in previous studies.

Cochlear implantation in children with large vestibular aqueduct syndrome and a review of the syndrome.

OBJECTIVE: Children with Large Vestibular Aqueduct Syndrome (LVAS) frequently develop speech and language skills prior to deterioration of their hearing. Operations designed to halt the progression of hearing loss have largely failed so the question of Cochlear Implantation in these children has arisen. It had been suggested that there would be technical difficulties in implanting these patients and, therefore, there had been an initial reluctance to proceed to implantation. The aim of the present paper is to assess surgical and functional outcomes in implanted children with LVAS and review the related literature. MATERIAL AND METHODS: From the 170 children assessed by MRI in the Nottingham Paediatric Cochlear Implant Programme, seven (4%) were identified as having LVAS. Four of these children were implanted and had at least 12 months follow up. Two of the children are on the waiting list for implantation and one child was not implanted because of absence of the cochlear nerve. Operative findings, complications and outcome measures were recorded. The auditory skills of the children were assessed before implantation and 1 year following implantation. A literature search was done to identify other series with experience in implanting children with LVAS. RESULTS: Full insertion of the electrode array was achieved in all our cases. After cochleostomy two patients experienced a mild CSF leak that was easily controlled by the muscle graft. On the first day post-operation two patients were nauseous and one had an episode of vomiting, however, all were discharged within 24 h of surgery. Initial outcome measures at 12 months post-implantation were encouraging showing significant progress in children's auditory skills. CONCLUSIONS: The results of the present study and the review of the literature suggest that LVAS is not a contraindication to implantation as initial concerns about severe perilymph leaks and surgical complications have proved to be unfounded. The post-operative progress of these children in listening skills also suggest that these children are suitable for cochlear implantation

Prolongation of the cochlear microphonic in man. Cochlear microphonic ringing.

During routine extra-tympanic electrocochleographic (ECochG) recording we have observed that in many cases more cycles are seen in the cochlear microphonic (CM) recording than are present in the acoustic stimulus. We have termed this phenomenon CM 'ringing'. Ringing is present in 69% of our normal subjects and in 43% of patients with deafness of different aetiologies. In the cochlear and Ménière subgroups those cases showing ringing have a significantly larger CM amplitude (p less than 0.001 and p less than 0.01 respectively). The possible origin of this phenomenon is discussed.

Idiopathic sensory trigeminal neuropathy.

A series of seven cases of isolated facial numbness is presented in whom the final diagnosis is considered to be idiopathic trigeminal neuropathy. Although this is a benign condition, careful work-up is needed, preferably by a team of specialists, in order to exclude more serious pathology. The team should include a neurologist, an otolaryngologist and a dental surgeon. By this means all other possible underlying diagnoses can be excluded. A list of differential diagnoses is discussed and the need for careful long-term follow-up is stressed.

Recurrent meningitis due to congenital fistula of stapedial footplate.

A case of recurrent meningitis due to a stapes footplate fistula in a child with Klippel-Fiel syndrome is reported. The relationship of deafness to this latter syndrome is noted and theories for the aetiology of the fistula discussed. A stapedectomy was performed and the oval window was obliterated using a Schuknecht fat-wire prosthesis with further packing of the middle ear with muscle.

Bilateral vocal fold paralysis in infants: tracheostomy or not?

Tracheostomy has, in the past, been performed in the majority of children under one year with bilateral vocal fold paralysis. We present our experience of 11 cases over a ten-year period during which tracheostomy was avoided whenever possible. Ten cases were managed conservatively but in the youngest a tracheostomy was required. Full bilateral vocal fold mobility developed in all cases at a mean age of 11.5 months (range 5-26 months). Our experience suggests that the airway can commonly be managed expectantly without a tracheostomy.

Foetal warfarin syndrome--a complex airway problem. Case report and review of the literature.

Premature cartilaginous calcification and nasal hypoplasia following first trimester exposure to warfarin are known as the Foetal Warfarin Syndrome (FWS). There are over 40 cases reported in the literature, many of which describe breathing and feeding difficulties in the first few months of life. We report a case where a child had had difficulties breathing and feeding in the first months of life. These had been attributed to nasal hypoplasia. After proper ENT assessment the child benefitted from adenoidectomy. ENT surgeons should be aware of the syndrome as more women of child bearing age are taking warfarin following cardiac surgery and treatment of thromboembolic disease. ENT surgeons may be asked to review these children who often present with airway and feeding problems which have been attributed to nasal hypoplasia.

Vocal fold abductor paralysis as a solitary and fatal manifestation of multiple system atrophy.

A patient is presented who had bilateral abductor vocal fold paralysis pathologically proven to be due to multiple system atrophy (MSA) in the absence of other neurological features. MSA is a degenerative neurological condition that includes olivopontocerebellar atrophy, Shy-Drager syndrome and striatonigral degeneration. The usual predominant features of MSA are cerebellar ataxia, autonomic dysfunction and Parkinsonism. Stridor is present in over one third of patients and has been reported previously as a presenting symptom in MSA: however previously reported patients have always gone on to develop other neurological symptoms. The usual investigations of bilateral abductor vocal fold paralysis caused by MSA will not reveal the pathological process and we believe that magnetic resonance imaging (MRI) of the medulla and brain stem and autonomic function tests are probably the investigations of choice. It is a worthwhile exercise attempting to identify MSA as the cause of stridor as the prognosis is good in the medium term if appropriate support is offered.

Epistaxis, von Willebrand's type bleeding diathesis and Wilms' tumour: a case report.

We report a case of persistent epistaxis in a seven-month-old child with Wilms' tumour who had an associated von Willebrand's type bleeding diathesis. Correction of the bleeding diathesis to control the epistaxis required treatment of the tumour with chemotherapy.

Syrup formulations for post-tonsillectomy analgesia: a double-blind study comparing ibuprofen, aspirin and placebo.

Post-tonsillectomy analgesia from ibuprofen, aspirin and placebo is compared in a double-blind study. The results are reported showing ibuprofen to have greater therapeutic benefit than placebo whereas aspirin did not. Methods of providing pain relief after tonsillectomy and the relative clinical merits of ibuprofen and aspirin are discussed.

Munchausen syndrome by proxy: otolaryngologists beware!

Munchausen syndrome by proxy (MSBP) is a rare condition in which a parent or guardian fabricates an illness in a child either by inducing physical signs or prevaricating. A case presenting as recurrent episodes of infant apnoea is reported. A high index of suspicion is required for the diagnosis and, therefore, the otolaryngologist must be familiar with the condition if the life of the infant is to be protected and potentially harmful unnecessary investigations are to be avoided.

Raised ABR threshold after suction aspiration of glue from the middle ear: three case studies.

Between 1991 and 1993, 13 children (25 hearing ears) underwent recordings of the auditory brain stem response (ABR) under a general anaesthetic. The anaesthetic technique was similar for each child. Fourteen of these ears had fluid aspirated after myringotomy with insertion of grommets prior to the auditory brain stem response investigation. On subsequent hearing assessment six of these 14 ears (43 per cent) showed clear evidence of a threshold shift of 15 dB or greater. Eleven ears had either dry myringotomies or did not have a myringotomy prior to ABR and none of these showed evidence of a temporary threshold shift. Using Fisher's Exact probability test this difference is significant (p = 0.034). We feel it is important to report these observations so that unexpected high ABR thresholds following aspiration of glue are interpreted with caution.

Nasopharyngeal tuberculosis.

Isolated nasopharyngeal tuberculosis is a rare disease with very few cases having been reported in recent years. We report a case presenting in the United Kingdom with unilateral otalgia and otorrhoea.

Susac's syndrome: a rare cause of fluctuating sensorineural hearing loss.

Susac's syndrome is extremely rare and is characterized by the clinical triad of encephalopathy, retinal artery occlusion and deafness. A case of this rare syndrome is presented and for the first time the fluctuating nature of the hearing loss is demonstrated. The site and nature of the hearing loss has been investigated. The value of neural hearing loss as a method of monitoring the clinical severity is discussed.

Inhalation of a Montgomery Safe T-tube plug.

The first case of an inhaled Montgomery Safe T-tube plug is reported. Both the surgeon and the patient should be aware of this possible complication and regular inspection of the plug is recommended. More than one plug should be provided with each T-tube.

Tracheostomy in children.

Tracheostomy is more hazardous in children than in adults, and carries special risks in the very young. The past 20 years have seen a large shift in the age distribution of tracheostomy. Whereas formerly the operation was done largely for management of epiglottitis and laryngotracheobronchitis, today the prime indication is subglottic stenosis in infants consequent upon intubation for respiratory distress syndrome and prematurity. We have reviewed experience with 57 tracheostomies in 56 children under 12 years old managed from a university hospital. All operations were done as elective procedures, in standard fashion, by otolaryngologists. Forty (70%) were in children under 1 year old, the indications being upper airways obstruction (41), failed extubation (11), and long-term assisted ventilation (5). Subglottic stenosis was the commonest cause of obstruction (21 operations). In 91.4 accumulated years with a tracheostomy there were 11 complications related to tracheostomy, one of which (a blocked tube) was fatal. Thirty-nine children were decannulated, the mean duration of cannulation being 21 months. In this series we suggest that the low morbidity and mortality rates were due to management by otolaryngologists; to postoperative intensive care; and, for the majority cared for at home, to careful education of parents and visits by specialist nurses.

Pediatric cochlear implants. Surgical aspects: the Nottingham pediatric cochlear implant programme.

The implantation of young children is widely accepted as a means of rehabilitating profoundly/totally deaf children. This paper will review the surgical aspects of implanting young children based on the first 50 children implanted in the Nottingham paediatric cochlear implant programme. Having thoroughly counselled the children's parents, the child is operated on under prophylactic antibiotic cover. The incision (extended endaural) is made directly down to bone and a full-thickness flap is elevated. Meticulous attention is paid to haemostasis. The steps involved in posterior tympanotomy, cochleostomy and implant insertion, often in the presence of osteogenesis, will be described. Electrophysiological testing is done systematically peroperatively. Surgical complications were few. There was no cases of facial weakness, haematoma, implant extrusion or infection. One patient, whose implant was laid in an extracochlear gutter, developed pain on electrical stimulation and was explanted. Another patient developed a retraction pocket cholesteatoma. These few complications are far outweighed by the overwhelming benefits accrued from implantation.