Sodium fluoride in otosclerosis treatment: review.

OBJECTIVES: To review the current literature on the use of sodium fluoride in the treatment of otosclerosis. DESIGN: A literature review was conducted, searching the Medline and PubMed database from 1966 to 2009, using the terms 'otosclerosis' and 'fluoride'. Article abstracts were reviewed and relevant full articles acquired. RESULTS: There has been only one double-blind, placebo-controlled trial of the use of sodium fluoride in otosclerosis patients, and this found a reduced incidence of deterioration in hearing after two years in the treatment group. Several case-control series have described a hearing benefit in the sodium fluoride treated group. Treatment doses vary greatly, and there is no evidence regarding the optimum duration of treatment. CONCLUSIONS: There is low quality evidence suggesting that sodium fluoride may be of benefit to preserve hearing and reduce vestibular symptoms in patients with otosclerosis.

Tuberculosis presenting as a laryngeal tumour.

As the prevalence of tuberculosis in the U.K. increases, uncommon clinical presentations of the disease may be encountered by the otolaryngologist. We describe just such a case, where a caucasian patient presented with laryngeal lesion, considered to be highly suspicious of a neoplastic process rather than an infective one. We discuss the pathogenesis of tuberculous laryngitis, and review the differential diagnosis and management of the condition.

Gonadotropin-releasing hormone immunoreactivity in the nasal epithelia of adults with Kallmann's syndrome and isolated hypogonadotropic hypogonadism and in the early midtrimester human fetus.

GnRH-secreting neurons are known to originate in the epithelium of the medial olfactory placode, whence they migrate along the axons of the terminal nerve via the forebrain and into the hypothalamus. Synaptic contact between the developing olfactory bulbs and fascicles of the vomeronasal, terminal, and olfactory nerves does not occur in Kallmann's syndrome. Consequently, there is migration arrest of GnRH cells and partial or complete failure of formation of the olfactory bulbs, resulting in severe olfactory deficit and hypogonadotropic hypogonadism. In the present study, using an immunofluorescent, double immunostaining technique and confocal laser scanning microscopy, we observed GnRH-immunoreactive neurons in the hypothalamus of a 14-week-old human fetus. However, migration of GnRH neurons was not complete, and indeed, such cells were seen to be migrating along terminal nerve fascicles beneath the cribriform plate in a 16-week-old fetus. The same immunofluorescent technique demonstrated the presence of GnRH cells in biopsies of nasal mucosa obtained from three adults with Kallmann's syndrome, one normosmic subject with hypogonadotropic hypogonadism, and a eugonadal male cadaver. These findings are consistent with two different interpretations: the nasal GnRH neurons may be vestigial, representing cells that failed to migrate during embryogenesis; alternatively, they may have been generated de novo later in life, a possibility consistent with the recognized plasticity of human postnatal olfactory neuroepithelium. They also reveal that subjects with the normosmic (i.e. non-Kallmann's) form of GnRH deficiency are able to synthesize immunologically recognizable GnRH, implying that failure of GnRH synthesis is not responsible for this type of hypogonadotropic hypogonadism.

Oculopharyngeal myopathy with sensorineural hearing loss.

A case is reported of a 59-year-old Caucasian male with oculopharyngeal myopathy and sensorineural hearing loss. He presented with progressive ptosis, sensorineural hearing loss over several years and symptoms of mild dysphagia. Further enquiry into his family history revealed that every male member in his family that lived beyond the age of 60 exhibited identical symptoms. Symptoms of ptosis and dysphagia are consistent with the rare autosomal dominant condition of oculopharyngeal myopathy, believed to be due to mitochondrial disease. The combination of ptosis, dysphagia and sensorineural hearing loss with normal distant muscle group biopsy has not been described before.

Retropharyngeal abscess-an unusual complication of anorexia nervosa.

An unusual case of an adult female with anorexia nervosa, who developed a retropharyngeal abscess, is presented. This case is reported both because of the paucity of reports on retropharyngeal abscess and because of the dramatic unusual aetiology.

Histopathology of vocal cord palsy from recurrent laryngeal nerve damage.

The detailed postmortem laryngeal findings of a man with an established vocal cord palsy from an inoperable bronchial carcinoma is presented. Fine dissection of the monoblock specimen from skull base to superior mediastinum allowed sampling of vagus, recurrent and superior laryngeal nerves at different levels for fiber counts in order to compare the affected left and unaffected right side. Horizontal slicing of the whole larynx showed that the main cause of lateral displacement of the paralyzed left cord was gross atrophy of the underlying intrinsic laryngeal muscles. Cricothyroid muscle and superior laryngeal nerves were unaffected. Lateral cord drift due to underlying muscle atrophy is a better explanation of paralyzed cord position in this case than the Wagner and Grossmann theory of cord palsy.

Craniofacial resection for orbital tumours.

Craniofacial resection for nasal and paranasal sinus tumours is now well established and performed increasingly because it has several advantages over other surgical approaches. More recently a different craniofacial approach has been developed to allow better access to orbital tumours and permit their complete excision as a single block. The experience of nine patients is reported. Results so far suggest this operation, with its excellent exposure, gives a good oncological resection, low postoperative morbidity and satisfactory cosmesis. Large malignant tumours that previously would have been removed inadequately and in piecemeal fashion can now be excised surrounded by a margin of normal tissue.

Laryngeal electromyography: a useful technique for the investigation of vocal cord palsy.

Percutaneous needle laryngeal electromyography was used in 22 patients with an established vocal cord palsy of non-laryngological cause. Recordings of action potentials from both vocalis and cricothyroid muscles produced expected results in 8 patients with skull base lesions. In 6 patients with known lesions below the clavicles, ipsilateral cricothyroid activity was found to be normal despite the vocal cord lying in the lateral (or cadaveric) position. In 2 patients electromyography helped establish the site of the lesion. Eight patients with idiopathic cord palsies showed varying cricothyroid function but in all vocalis activity was absent.

Carcinoma of the ear: retrospective analysis of 61 patients.

Malignant tumours of the ear canal and middle ear retain a poor prognosis despite the development of increasingly radical procedures. We report the combined experience of 61 patients presenting to our hospital over the past 30 years with this condition. Radiotherapy was the preferred initial treatment in almost all patients. Early surgical treatment by local resection just before or just after radiotherapy and radical mastoidectomy produced disease free survival for two to 20 years in 59 per cent (16 of 27) patients. Long term survival was achieved in 50 per cent of patients with carcinoma of the external auditory meatus and only 24 per cent of those with middle ear carcinoma. Salvage surgery produced a variable degree and duration of palliation but few survivors. Little was gained in the quality of life by petrosectomy.

Laryngeal papillomatosis: correlation between severity of disease and presence of HPV 6 and 11 detected by in situ DNA hybridisation.

A technique using a biotin-streptavidin polyalkaline phosphatase complex was applied to routinely fixed and processed biopsy specimens of laryngeal papillomata from 45 patients taken over the past 20 years to detect human papilloma virus (HPV) types 6 and 11. Two thirds of both adult and juvenile onset cases were positive for HPV 6 or HPV 11 or both. Five specimens of normal vocal cord epithelium were negative for HPV 6 and 11. The detailed clinical history, endoscopic findings, success of treatment and eventual prognosis were compared with the HPV state of biopsy material for each patient. Patients with multiple confluent lesions when first seen, whose histology showed florid koilocytosis and who had strongly positive reactivity for HPV 6 or 11 present in the surface epithelial cell nuclei, had a poor prognosis requiring multiple endoscopies to control their disease.

Laryngeal papillomatosis: analysis of 113 patients.

Despite the low incidence of these diseases, both juvenile onset and adult onset laryngeal papillomatosis continue to concern otolaryngologists because treatment is often prolonged and unsatisfactory. Over many years various treatments have met with varied claims of success, yet no treatment today is universally accepted. We review the underlying clinical problems, the presentation, the treatment and eventual outcome of 113 patients seen at this hospital over the last 20 years with laryngeal papillomatosis. Using multivariate analysis of age at presentation, sex, smoking habits, initial site of disease, frequency of treatment and eventual outcome, two distinct patterns are revealed both between juvenile and adult onset disease and between male and female patients. Single-site lesions in adults have the best prognosis. Female children with multiple confluent lesions at presentation have the worst prognosis.

Recurrent meningitis in children due to inner ear abnormalities.

Recurrent meningitis in children is not only a potentially life threatening condition, but often involves the child in the trauma of repeated hospital admissions and multiple and invasive investigations to try and find an underlying cause. Symptoms and signs of CSF rhinorrhoea or otorrhoea are infrequent in these patients. Unilateral deafness may be difficult to diagnose in the young child. Full ENT examination may be normal. We report seven cases of children with recurrent meningitis in whom inner ear abnormalities were only indicated as the site of entry of infection by hypocycloidal tomography or high resolution CT scanning of the temporal bone. Subsequent tympanotomy confirmed the site of the CSF leak as the oval window in the majority of cases; packing the vestibule with muscle halted further attacks in these patients.

Craniofacial resection for extensive paranasal sinus aspergilloma.

Craniofacial resection techniques were developed to allow complete monobloc removal of malignant tumours of the ethmoid region. Such a surgical approach may also occasionally be useful in certain non-malignant conditions. Extensive 'destructive' aspergillosis of the paranasal sinuses has a high mortality once the anterior cranial fossa dura is reached. Craniofacial surgery provides excellent access to excise such large infective 'tumours'.

Non-Hodgkin's extranodal lymphoma: a clinico-pathological study of 24 cases involving head and neck sites.

A series of 24 patients with a documented diagnosis of non-Hodgkin's extranodal lymphoma (ENL) involving head and neck sites (excluding the cranial cavity) has been studied. In view of recent advances in the diagnosis of lymphoma, a retrospective histopathological review has been undertaken to place all patients in standardized classifications for morphology (the working formulation) and immunology (Kiel). This resulted in a change of diagnosis from non-Hodgkin's lymphoma in four. The histopathology of these four cases, and the implication of such changed diagnoses are discussed. Of the 20 patients confirmed as having non-Hodgkin's ENL, 16 had primary ENL and four had an ENL focus secondary to disease elsewhere. Three patients in this series had an initial diagnosis made of another malignancy, but histopathological review prior to treatment changed the diagnosis to ENL. Details of presentation, pretreatment evaluation, immunohistochemistry, treatment and course have been analysed. The principal treatment policy for primary ENL has been radiotherapy. Of the 12 patients who received this treatment ten achieved a complete response (83 per cent). Eight remain free of disease at the time of writing. The role of adjuvant chemotherapy is also considered.

Intraorbital dermoid: an ophthalmological problem solved by an ENT approach.

A case of an intraorbital dermoid and its successful removal using a novel approach is reported. The approach used offers significant advantages in access over the standard approach.

Middle turbinate osteoma; an unusual cause of nasal obstruction.

Osteomas are common benign tumours of the paranasal sinuses, but are usually asymptomatic and found as a coincidental finding during routine radiological investigations. We present a case report of a patient with an isolated osteoma of the middle turbinate presenting with unilateral nasal obstruction which required removal via a lateral rhinotomy approach. The aetiology, pathology and presenting features of these tumours are reviewed and their subsequent management discussed. In particular, adequate pre-operative radiological assessment to determine involvement of the cribriform plate region by computerized axial tomography (CAT Scanning) is emphasized since this may indicate the need for a cranio-facial surgical approach rather than a lateral rhinotomy to achieve complete removal.

Cleft palate and glue ear.

The invariable presence of otitis media with effusion in children with cleft palate aged from 2 to 20 months is confirmed in a prospective trial, and the diagnosis was made in each case by myringotomy. Treatment planning should take account of this, and long term ventilation of at least one ear seems to be mandatory in order to correct bilateral congenital deafness. Close cooperation between otologist and plastic surgeon is essential for diagnosis and treatment of otitis media in these patients.