Biomedical subjects

I J Johnson

Publications and source records attributed to I J Johnson.

At least 19 recordsLinked to original sources

PubMed2004

Cochlear implantation in patients with MELAS syndrome.

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL) that is genetic in origin. Mitochondrial cytopathies can present with a variety of symptoms, but they occasionally present with SNHL as their first manifestation. Two cases of MELAS patients who responded well to cochlear implantation are presented. A review of the literature is also carried out focusing mainly on diagnosis, anesthetic considerations and management of these patients.

Adult↗

PubMed2004

The MELAS syndrome. Review of the literature: the role of the otologist.

The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare congenital disorder of mitochondrial DNA (mt-DNA). Patients with this syndrome may present to the otolaryngologist with sensorineural hearing loss (SNHL), which is genetic in origin. A high index of suspicion is required because this hearing loss is part of a syndrome for which early diagnosis and intervention is required.

DNA, Mitochondrial↗

PubMed2001

Bone-anchored hearing aid quality of life assessed by Glasgow Benefit Inventory.

OBJECTIVE/HYPOTHESIS: The bone-anchored hearing aid (BAHA) is a well established mode of treatment and many studies show the audiological benefit, but none has assessed the benefit to the quality of life of patients. This study uses the validated Glasgow Benefit Inventory to quantify the changes in quality of life. STUDY DESIGN: Retrospective questionnaire study. METHODS: Sixty consecutive patients receiving treatment with BAHA were enrolled in the study. The male/female ratio was 1.26 to 1; mean patient age was 45 years. The most common indication was hearing loss secondary to mastoid disease and surgery followed by congenital atresia and chronic discharge from the ear. RESULTS: The response rate was 85%, which is high and adds weight to the results. The general benefit score was +34 (range, +27-+48), which is comparable to middle ear surgery but just below benefit from cochlear implantation. The social benefit was +21 (range, +12-+37) with only +10 (range, +2-+26) for the physical score. This pattern mirrors that reported for other ear interventions. Maximum benefit was noted in patients with congenital atresias followed by discharging mastoid cavities. CONCLUSION: This study is the first to demonstrate significant quality of life benefit from BAHA surgical intervention as measured by the Glasgow Benefit Inventory.

Adolescent↗

PubMed2001

Acute epiglottitis: a presentation of haemophagocytic lymphohistiocytosis.

The incidence of acute epiglottitis in children has declined with the introduction of the Haemophilus influenzae b vaccine in 1992. We report a case of acute epiglottitis in a child secondary to an immunocompromised state. We suggest that when acute epiglottitis is diagnosed in a child we should ensure there is no underlying predisposing condition.

Acute Disease↗

PubMed1999

Gustatory sweating of the external auditory canal.

Gustatory sweating of the external auditory canal is extremely rare. A clinical case, that is only the second in the English literature, is presented. The potential pathogenesis and its treatment options are discussed.

Aged↗

PubMed1998

Idiopathic diffuse erosion of the skull base presenting as cerebrospinal fluid rhinorrhoea.

We present an unusual case of generalized erosion of the skull base. We have not found a similar case reported in the world literature. The presenting symptom was spontaneous cerebrospinal fluid (CSF) rhinorrhoea which arose from a bony defect associated with herniation of the right temporal lobe into the sphenoid sinus. We discuss the management of such a case including imaging of the skull base and the endoscopic repair of the bony defect.

Aged↗

PubMed1998

Chondroma of the external auditory canal.

Chondroma, a benign tumour which is common at many sites in the body, has not been reported in the English literature in the bony external auditory canal. The first such case at this anatomical site is described and the pathogenesis is considered.

Adolescent↗

PubMed1997

Aberrant common carotid artery encountered during free jejunal graft repair of oesophageal stricture.

The origin and course of the carotid arterial system in the superior mediastinum and neck are remarkably constant. Although variations are rare, they can have important implications in certain clinical problems. In this report, a patient underwent free jejunal graft repair of a cervical oesophageal stricture caused by radiotherapy for a post-cricoid carcinoma. The common carotid artery on the side of the surgical approach was found to cross the midline to the other side of the neck, and created difficulties both with the procedure and future management. This has not been previously reported.

Aged↗

PubMed1997

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.

Adult↗

PubMed1997

Compositional differences between bilateral middle ear effusions in otitis media with effusion: evidence for a different etiology?

The aim of this study was to clarify the site of primary pathology in otitis media with effusion. Effusions were collected from 64 children with bilateral effusions at the time of myringotomy. The rheological properties and biochemical compositions of effusions were measured for 23 pairs of effusions, and the levels of the inflammatory mediators TNF alpha, IL-1beta, and IL-8 were measured in 41 pairs using specific enzyme-linked immunosorbent assays (ELISAs). Measurements from paired ears were compared using analysis of variance (ANOVA) tests and significant differences were found for reduced specific viscosity, mucin content, protein content, and levels of IL-8. The results demonstrate that the two ears have different immunological processes or rates of processes which might explain the significantly different rheological properties of effusions. This suggests that each ear undergoes pathological changes independently and has implications for using the opposite ear as a control in clinical trials.

Analysis of Variance↗

PubMed1997

Surgical aspects of cochlear implantation in young children: a review of 115 cases.

A review of the surgical complications of the first 115 patients who received a cochlear implant in this program demonstrate a negligible complication rate in the perioperative period and a low complication rate in the long term. The surgical technique is described in detail and the reasons for this low complication rate are analysed.

Child↗

PubMed1996

Glossal abscess presenting as unilateral tongue swelling.

Glossal abscesses are extremely rare, only 29 cases having been described in the last 28 years. Clinical and ultrasound examination should establish the diagnosis, and treatment should be by surgical incision and drainage. We report a case of glossal abscess presenting as a unilateral swelling of the tongue.

Abscess↗

PubMed1996

Fourth branchial arch fistula.

A case is presented of a rare congenital anomaly of the fourth branchial arch, which presented as an abscess in the anterior triangle, related to a fistula communicating with the pyriform fossa. Histopathological examination showed the fistula to be associated with thyroid tissue supporting the hypothesis that the ventral wing of the fourth pouch contributes to the thyroid gland.

Abscess↗

PubMed1996

Middle ear infection and sixth nerve palsy.

Three cases of patients with acute middle ear infections complicated by a sixth nerve palsy are described. The possible causes of sixth nerve palsy in such cases are discussed. We propose that where there is a sixth nerve palsy without petrositis and without raised intracranial pressure, it can arise as a result of spreading phlebitis along the inferior petrosal sinus from the lateral sinus.

Abducens Nerve↗

PubMed1996

Vocim--a system for measuring the larynx endoscopically.

Examination of the larynx with a flexible endoscope has been a standard procedure for over 20 years. Although excellent images have been obtained there has not been a method for achieving any objective measurements and there are clearly both research and clinical applications for this. The reason for this is firstly because of the inherent distortion of the lens and secondly because of an absence of a reliable method of assessing the relative position of the endoscope within the larynx. This paper illustrates the theory of how these problems have been overcome and describes the equipment used in the clinical situation. The first clinical trial of the Vocim system is detailed, demonstrating an error of only 4%.

Computers↗

PubMed1996

Assessment of pre-operative investigations of thyroglossal cysts.

The appropriate degree of investigation of thyroglossal cysts is uncertain. This retrospective study analyses the pre-operative investigations and correlates this with the post-operative results. The conclusion is that only radio-isotope studies are useful, but there is a limitation regarding the age of the patient and the authors only recommend this in patients over 10 years of age.

Adult↗

PubMed1995

Bilatral auriculotemporal syndrome in childhood.

A case of bilateral Auriculotemporal or Frey's syndrome in childhood is unique in the World literature. The clinical presentation and management is discussed. A conservative approach is advocated but other therapeutic options are considered. The literature is reviewed, only 17 cases all unilateral are known, and from the study the probable aetiology and long term follow-up is reported.

Child↗

PubMed1995

Nasal tuberculosis--an increasing problem?

Nasal tuberculosis represents a rare manifestation of infection by Mycobacterium tuberculosis: a case is presented together with a review of the literature. The correct management is documented, especially the need to treat the patients as if there was a generalized condition, and the problems concerning drug resistance are also considered. The incidence of this disease is rising and its importance must once again be highlighted.

Aged↗