Laryngeal dysplasia: a long-term follow-up study.

PURPOSE: To assess the progression of precancerous laryngeal lesions to squamous cell carcinoma (SCC), defined by specific histopathological criteria, in patients with longterm follow-up. METHODS: Patients with laryngeal dysplasia, followed/ treated between 1985 and 2008, were retrospectively evaluated and classified according to the World Health Organization classification system (WHO). The investigated outcome parameters were progression of dysplasia to SCC, time interval to malignant transformation and continuation of smoking as potential risk factors. RESULTS: Fifty-nine patients were studied. Progression of dysplasia to SCC between the first and the final histological examination was statistically significant (p<0.0001). Malignant transformation appeared in 29 patients (49.2%). Serious dysplasia was more likely to progress to SCC (64.8%) compared to mild (41.7%) or moderate (44.4%) (p<0.0001). However, the time interval needed in these 29 cases to progress to cancer was not statistically related to the initial histological diagnosis. Continuation of smoking did not affect the progression of disease. However, the mean time from dysplasia to laryngeal cancer was much longer in patients who quitted smoking (33.5 months) vs those who continued smoking (19.5 months), with a marginal statistical difference (p=0.057). CONCLUSION: All patients with laryngeal dysplasia should be followed up at regular intervals. The progression of dysplasia to SCC did not seem to be directly related to the continuation of smoking or not. However, large long-term follow- up studies taking into account the degree of exposure (e.g. time of exposure, number of cigarettes) are needed in order to clarify risk factors and proper management. Consensus guidelines in diagnosis, follow-up, and treatment would improve substantially the current clinical practice.

Inner ear dysfunction in myotonic dystrophy type 1.

OBJECTIVES: Myotonic dystrophy type 1 is associated with various oculomotor, vestibular, and auditory abnormalities. However, auditory system investigation has been mainly performed with the subjective method of pure-tone audiometry. In this study, a detailed vestibular and audiological evaluation was undertaken, including the objective and more sensitive method of transiently evoked otoacoustic emissions (TEOAEs). MATERIALS AND METHODS: Twenty-four patients with genetically diagnosed myotonic dystrophy type 1 and 21 controls were studied. Audiological and vestibular investigations included pure-tone audiometry, tympanometry, auditory brainstem responses (ABRs), TEOAEs, and electronystagmography. RESULTS: Hearing impairment was evident in 15 (62.5%) patients and in nine of them (37.5%) ABR abnormalities were found. However, subclinical cochlear damage was found in all patients, as evidenced by absent emissions or lower otoacoustic emission amplitude. Vestibular hypesthesia was found in nine patients (37.5%), accompanied by spontaneous nystagmus in four of them (15.6%). CONCLUSIONS: Auditory and vestibular abnormalities are quite common in patients with myotonic dystrophy type 1. However, it appears that subclinical cochlear damage is an ubiquitous finding of the disease.

Inner ear disease and benign paroxysmal positional vertigo: a critical review of incidence, clinical characteristics, and management.

Background. This study is a review of the incidence, clinical characteristics, and management of secondary BPPV. The different subtypes of secondary BPPV are compared to each other, as well as idiopathic BPPV. Furthermore, the study highlights the coexistence of BPPV with other inner ear pathologies. Methods. A comprehensive search for articles including in the abstract information on incidence, clinical characteristics, and management of secondary BPPV was conducted within the PubMed library. Results. Different referral patterns, different diagnostic criteria used for inner ear diseases, and different patient populations have led to greatly variable incidence results. The differences regarding clinical characteristics and treatment outcomes may support the hypothesis that idiopathic BPPV and the various subtypes of secondary BPPV do not share the exact same pathophysiological mechanisms. Conclusions. Secondary BPPV is often under-diagnosed, because dizziness may be atypical and attributed to the primary inner ear pathology. Reversely, a limited number of BPPV patients may not be subjected to a full examination and characterized as idiopathic, while other inner ear diseases are underdiagnosed. A higher suspicion index for the coexistence of BPPV with other inner ear pathologies, may lead to a more integrated diagnosis and consequently to a more efficient treatment of these patients.

GSTT1, GSTM1, GSTM3 and NAT2 polymorphisms in laryngeal squamous cell carcinoma in a Greek population.

OBJECTIVE: It is well known that laryngeal squamous cell carcinoma is strongly related to tobacco and alcohol consumption. Accumulating evidence suggests that alterations of detoxification enzymes, such as glutathione S-transferases and N-acetyltransferases, influence the risk of cancers associated with tobacco smoke and alcohol. METHODS: This was a retrospective case-control study. The study group consisted of 88 Greek patients with laryngeal squamous cell carcinoma; there were also 102 control subjects. Frequencies of the genotypes GSTT1, GSTM1, GSTM3 and NAT2 were evaluated by polymerase chain reaction restriction fragment polymorphism. RESULTS: The distribution of overall genotypes was 55.68 per cent rapid acetylator and 44.32 per cent slow acetylator in patients, and 36.27 per cent rapid acetylator and 63.72 per cent slow acetylator in controls. The odds ratio for rapid acetylator status in cases versus controls was 2.207 (95 per cent confidence interval 1.23-3.95, p = 0.0087). CONCLUSION: This study demonstrated a significant relationship between rapid acetylator genotypes and laryngeal squamous cell carcinoma in a Greek population.

Semicircular canal versus otolithic involvement in idiopathic sudden hearing loss.

OBJECTIVE: To evaluate the results of vestibular evoked myogenic potential testing in patients with idiopathic sudden hearing loss, and to correlate these results with the findings of caloric testing, the clinical appearance of vertigo and the influence of age. MATERIALS AND METHODS: Eighty-six patients with unilateral idiopathic sudden hearing loss and 35 healthy controls underwent a standard protocol of neurotological evaluation. Vestibular evoked myogenic potential responses were measured and compared with caloric responses. RESULTS: On the affected side, 30.2 per cent of patients showed abnormal vestibular evoked myogenic potential responses, while 52.3 per cent had abnormal caloric responses. A statistically significant relationship was found between the results of these two tests. A statistically significant relationship was also found between the type of vestibular lesion and the occurrence of vertigo. Advancing age correlated statistically with more extensive labyrinthic lesions. CONCLUSIONS: A combination of vestibular evoked myogenic potential and electronystagmography testing indicated the existence of vestibular involvement in many patients with idiopathic sudden hearing loss. Both tests are necessary in order to obtain a more thorough and in-depth knowledge of the pathophysiology of idiopathic sudden hearing loss.

Relative diagnostic importance of electronystagmography and magnetic resonance imaging in vestibular disorders.

OBJECTIVE: The aim of this study was to investigate the contribution of electronystagmography and magnetic resonance imaging to the aetiological diagnosis of vertigo and unsteadiness, in a population in which the history and clinical examination provide no conclusive diagnosis of the origin of the dysfunction (i.e. peripheral or central). PATIENTS AND METHODS: This retrospective study included 102 patients, who underwent full ENT clinical evaluation, history and neurotological assessment (including pure tone audiography, auditory brainstem response testing, electronystagmography and magnetic resonance imaging). RESULTS: Electronystagmography contributed to establishment of a diagnosis in 53/102 patients (52 per cent), whereas magnetic resonance imaging did the same in four of 102 patients (3.9 per cent). CONCLUSION: Electronystagmography remains the most useful examination for aetiological diagnosis of patients with vertigo and unsteadiness, since the actual number of patients with vertigo and unsteadiness of central origin is small (3.9 per cent), even in a population in which history and clinical examination may indicate an increased probability of central nervous system dysfunction.

Screening for hearing loss and middle-ear effusion in school-age children, using transient evoked otoacoustic emissions: a feasibility study.

INTRODUCTION: The characteristics of otoacoustic emissions that make them ideally suited for universal newborn hearing loss screening could potentially be useful for the screening of older children. This study was performed in order to assess the role of otoacoustic emissions in a screening programme for middle-ear disorders and hearing loss in school-age children. METHODS: Cross-sectional, preliminary screening study. SETTING: Primary schools of Argolida municipality, south-east Greece, between December 2004 and March 2005. PATIENT SELECTION AND RECRUITMENT: All the primary school students of Argolida were invited, by press releases and individually by their teachers, to attend a session of otological and audiological screening. RESULTS: One hundred and ninety-six children were evaluated using transient evoked otoacoustic emissions. Twenty per cent failed in both ears, while in 32 per cent otoacoustic emissions could not be produced in at least one ear. Younger children had higher rates of absent transient evoked otoacoustic emissions. The absence of otoacoustic emissions was highly correlated with tympanic membrane changes seen on otoscopy and the presence of a type B tympanogram. As a single screening modality, otoacoustic emissions had a 100 per cent sensitivity in diagnosing hearing loss worse than 30 dB, and a 90 per cent sensitivity and 64 per cent specificity in diagnosing hearing loss worse than 25 dB, which did not improve by adding tympanometry to the screening protocol. CONCLUSION: These results strongly suggest the potential usefulness of otoacoustic emission testing in screening school-age children for hearing loss. Further studies, taking into account cost-effectiveness issues, are indicated.

Temporal bone study of development of the organ of Corti: correlation between auditory function and anatomical structure.

OBJECTIVE: To study the development of the organ of Corti in the human cochlea, and to correlate our findings with the onset of auditory function. MATERIAL AND METHODS: Step sections of 81 human fetal temporal bones were studied, from eight weeks of gestation to full term. RESULTS: By the end of the 10th week, the tectorial membrane primordium could be traced even in the most apical turns. Individual hair cells became identifiable at the basal turn at 14 weeks. At the same time, a small but well formed oval space was observed between the inner and outer hair cells in the basal turn. This does not correspond to the tunnel of Corti, as is erroneously quoted in the literature, as the individual pillar cells develop at later stages. Between 14 and 15 weeks, Hensen's cells were recognised for the first time. Individual pillar cells were identifiable at 17 weeks and the tunnel of Corti opened at 20 weeks. By 25 weeks, the cochlea had reached its adult size, but continued to develop until full term. DISCUSSION AND CONCLUSIONS: A temporal coincidence of different developmental events is responsible for early fetal audition at 20 weeks, including growth of pillar cells, opening of the tunnel of Corti and regression of Kollicker's organ, with the subsequent formation of the inner spiral sulcus and then separation of the tectorial membrane. The fine structures of the organ of Corti continue to develop well after the 25th week, and this may well alter the mechanical properties of the vibrating parts of the cochlea, which may in turn account for the frequency shift observed in preterm infants. These changes will have to be taken into account in the development of prenatal hearing screening tests.

Idiopathic temporal bone encephalocele.

Meningoencephaloceles are herniations of brain tissue through dehiscences of the skull base. These skull defects are either acquired (otologic infection, trauma, surgery, neoplasia) or spontaneous. Spontaneous temporal bone meningoencephaloceles are quite rare conditions, usually congenital in origin presenting during childhood, and only occasionally idiopathic presenting during adulthood. We present a case of temporal bone meningoencephalocele of adult onset. The patient was treated with exploratory mastoidectomy, amputation of the herniated cele and closure of the defect with temporalis fascia and an inferiorly based pedicled muscular flap. No reconstruction of the bony defect was performed, as the layered closure was considered adequate. Twelve months' follow-up revealed no relapse of the condition or postoperative complications.

Idiopathic sudden sensorineural hearing loss: prognostic factors.

OBJECTIVES: Sudden sensorineural hearing loss (SSHL) remains a challenge for the clinician. In the majority of cases, no definite cause can be found and the prognosis is variable. METHODS: The present study assessed 114 patients suffering from idiopathic SSHL, with regard to the prognostic value of demographic, epidemiologic, neurotologic and audiometric factors. In addition, the relationship between the identification of wave V in auditory brainstem responses and the final hearing outcome was investigated. All patients received 75 mg/day intravenous prednisolone, divided into three daily doses, for 10 days, with gradual tapering of the dose over the next 10 days. RESULTS: The results (after one year follow up) revealed the following factors to be related to a better hearing outcome: younger age; male sex; less time elapsed between the onset of hearing loss and the beginning of treatment; and an upward-sloping or cupeloid audiogram contour. The detection of wave V early in recovery and within the first month of medical treatment might also constitute a significant favourable factor in respect to hearing recovery. CONCLUSIONS: The present study revealed that there are certain factors that affect prognosis in idiopathic SSHL. This is very important in counselling patients and may affect current clinical practice.

Cophenylcaine spray vs. placebo in flexible nasendoscopy: a prospective double-blind randomised controlled trial.

Practices vary across the UK on the use of topical preparation prior to flexible fibreoptic nasendoscopy. In this double-blind study, we randomised 98 patients to receive cophenylcaine or placebo nasal spray before flexible nasendoscopy. A visual analogue scale (1-100) was used to record pain, unpleasantness of taste and overall discomfort experienced. Overall, the procedure was associated with minimal pain and discomfort in both groups. There was no significant difference in pain or overall discomfort experienced between the two groups; however, the sensation of bad taste was significantly worse in the cophenylcaine group. In linear regression, factors that predicted the overall unpleasantness of the experience were primarily pain experienced and secondarily unpleasantness of taste. We conclude that the routine use of cophenylcaine for nasal preparation is not justified before flexible nasendoscopy.

Pituitary apoplexy: a pathologic entity from an otolaryngologist's view.

A case of pituitary apoplexy, which was initially misdiagnosed as 'acute frontal sinusitis', is reported. The presenting symptoms and signs of the patient were headache, moderate fever, left periorbital edema, marked tenderness over the left frontal sinus and purulent secretion over the left middle turbinate and nasopharynx. These clinical symptoms were wrongly perceived as complicated frontal sinusitis. The CT scan and the elective right carotid angiography showed a pituitary adenoma. Therefore pituitary apoplexy of a preexisting pituitary adenoma was diagnosed. The patient underwent surgical removal of the adenoma and his postoperative course was uneventful. Thus otolaryngologists should consider pituitary apoplexy in the differential diagnosis of pathologies concerning the anatomic area of the anterior cranial fossa.

Role of the new imaging modalities in the investigation of Meniere's disease.

The incidence of nonvisualization of the vestibular aqueduct and the endolymphatic duct-endolymphatic sac (ED-ES) complex as well as the type of periaqueductal pneumatization were evaluated in 23 patients with definite Meniere's disease (MD) by high-resolution CT and by MRI. Fifty subjects with no previous history of any ear disease were used as a control group. High-resolution CT results disclosed that in the control group the percentage of nonvisualized vestibular aqueduct (3.4%) was statistically significantly lower than in the MD group, when either the diseased ear (27.8%) or the nondiseased ear of the same group (22.2%) was examined. In addition, no differences were observed between the diseased and nondiseased ears of the patients with MD. The periaqueductal pneumatization was also found to be statistically significantly lower in the MD group. As regards the MRI examination, the results of the analysis showed that the ED-ES complex was visualized more frequently in the ears of the control subjects (64.1%) than in the diseased ears of the patients with Meniere's disease (39.1%). This difference had a marginal statistical significance (P approximately 0.05). We discussed the results in relation to possible underlying pathophysiologic mechanisms involving the flow of endolymph toward the ES during the different stages of the disease.

Monostotic fibrous dysplasia of the temporal bone.

Fibrous dysplasia is a slowly progressive bony disorder where normal bone is replaced by abnormal fibro-osseous tissue. Its monostotic variety in the temporal bone is very rare and such a case is presented here. Computed tomography (CT) may be adequate for the diagnosis and follow-up of these patients. Limited surgery should only be considered in cases of symptomatic disease.

Langerhans cell histiocytosis with involvement of the pons: case report.

Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI.

Primary lymphoma of the internal auditory canal. Case report and review of the literature.

We report a rare case of a primary intracranial B cell lymphoma originating in the internal auditory canal. The clinical manifestations were indistinguishable from those of other, more common tumors of the same region. We achieved total gross tumor removal with preservation of the facial nerve. A detailed histologic examination and a systemic workup confirmed the primary nature of this tumor. To our knowledge, this is the second case reported in the literature of a primary malignant lymphoma originating in the internal auditory canal. This is the first instance that includes immunohistochemical and cytometric studies of fresh tissue. We discuss the management of primary lymphomas of the central nervous system, with special emphasis on their association with acquired immunodeficiency syndrome and other immune system diseases. Awareness of primary central nervous system lymphomas is important, since a greater occurrence of these rare tumors in the cerebellopontine angle is probable in the future.

Lermoyez's syndrome: histopathologic report of a case.

This paper reports findings in a pair of temporal bones from a patient with clinical evidence of Lermoyez's syndrome, a rare variant of Meniere's disease. Endolymphatic hydrops is limited to the basal turn of the cochlea and saccule in Lermoyez's syndrome, but more generalized in Meniere's disease.

HLA antigens in the pathogenesis of Menière's disease.

In the Department of Otolaryngology of the London Hospital many clinical and laboratory investigations have been conducted over the past fifteen years in an attempt to unravel some of the mysteries of Menière's disease. Many of these have been directed towards diagnosis, prognosis or therapy. Some have had a bearing on the aetiology of the idiopathic disease--these were summarised by Morrison (1981), including the finding of an hereditary predisposition, of an association with migraine in a significant proportion, also familial, of a 'personality type' of patient with Menière's disease and of typical radiological changes in the skull base in the majority of sufferers. More recently Brookes (1985), writing from this department, drew attention to both the occasional finding of an association between endolymphatic hydrops and elevated levels of circulating immune complexes, and also to the immunological studies in progress in patients with Menière's disease. These latter early results were reported by Morrison (1984) and are summarised in Table I. There was a highly significant difference between the levels of circulating complement and of immune complexes in patients with Menière's disease compared with control patients. There was, however, no difference in the results of autoantibody screening nor in the levels of serum immunoglobulins between the groups.

Is the endolymphatic sac always accessible?

All surgical manipulations of the endolymphatic sac have a common requirement: to identify the intradural part. We studied the position of the intradural endolymphatic sac in relation to the posterior semicircular canal, the sigmoid sinus, the retrofacial air cell tract, and the jugular bulb in 50 temporal bones. The intradural sac was identifiable in 48 cases; in two the sac either was absent or would not have been identifiable surgically without jeopardizing other important structures, particularly the posterior semicircular canal.