Nasal sarcoidosis: a cause for a medical rhinoplasty?

Objective: We report the use of triamcinolone injections to correct severe nasal deformity due to sarcoidosis, as an alternative to formal surgical rhinoplasty. Case report: A 30-year-old woman with a long-standing history of sarcoidosis presented to a tertiary referral rhinology clinic complaining of breathing difficulty and nasal deformity. Flexible nasoendoscopy revealed red nasal plaques typical of nasal sarcoidosis, together with significant widening of the nasal bridge. Triamcinolone, a long-acting corticosteroid, was injected both intralesionally and subcutaneously over the nasal dorsum, at zero, three and eight months, resulting in long-lasting improvement of the nasal shape. Conclusion: Sarcoidosis is a non-caseating, granulomatous, epithelioid inflammation. Otorhinolaryngological manifestations occur in approximately 10 per cent of patients; however, there is little published experience of nasal reconstruction in such patients. We describe a quick, simple and relatively cost-effective technique, with little or no co-morbidity, with which to improve the aesthetic and symptomatic outcomes of nasal sarcoidosis.     

Comentarios sobre minusvalía en patología vestibular

Handicap of the patient with vestibular disease can be measured by means of scales and questionnaires. The utility of a questionnaire developed and validated in Spanish language is commented (CEMPV). The CEMPV consists of 46 questions distributed in three scales (emotional, functional and organic) whose obtained scores are related to diverse degrees of handicap (global, emotional, functional and organic). The CEMPV is self-employed and evaluates the evolution of the patient and allows to accredit the results obtained between different investigators.     

Esthesioneuroblastoma — a rarity

A young male presented with visual complaints of one year duration, epistaxis and nasal blockage of 3 months duration. He had bilateral proptosis and bitemporal hemianopia and a mass near the; roof of nasal cavity. Skull skiagram showed enlarged sella and computed tomography showed erosion of sella and a central mass. Partial excision of supra and parasellar tumour was done which on histopatho-logical examination revealed Esthesioneuroblastoma. This was followed by radiotherapy and CCNU twice, at 6 weeks interval. He is doing well after 1 year and 9 months of follow up.     

Miringoplastias primarias. Resultados a los 2 años de seguimiento

Introduction and goals: The goals of myringoplasty are closure of the perforation in the tympanic membrane and improvement in hearing levels, with varying results in the published literature. Our aim was to determine the results of this procedure at our centre and compare them with the literature.Methods: Retrospective analysis of 217 primary myringoplasties carried out in the Otorhinolaryngology Department of the Ramón y Cajal University Hospital between 1998 and 2003, describing the characteristics of the perforations, surgical technique and post-operative results (perforation closure and hearing improvement).Results: The majority were adults with perforations secondary to recurrent middle ear infections (91 %). Most were less than sub-total (64 %) and did not involve the tympanic annulus (79 %). Grafts were inserted using lateral (45 %), mixed (29 %) and medial (26 %) techniques, using retroauricular (66 %) and endaural (34 %) approaches. Temporal muscle fascia was the graft most frequently used (87 %). Perforation closure was achieved in between 78 % and 91 % of cases throughout the 24-month follow-up period, with an overall closure value of 78 % after 2 years of follow-up. Hearing improvement, established as an air bone gap difference of less than 20 dB, was seen in proximately 56 % of cases.Conclusions: At our centre, myringoplasty achieves anatomical (78 %) and functional (56 %) success comparable to the results described in the literature. We consider medium-term follow-up to be of the utmost importance because of reperforation phenomena, which may occur as much as 2 years or more after surgery.     

When is a biopsy justified in a case of relapsing polychondritis?

Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.     

Absceso de cavum y oftalmoplejía secundarios a otitis externa maligna

We present a rare case of ophthalmoplegia, labyrinthitis and abscess of cavum secondary to skull base osteomyelitis by malignant external otitis. Since symptoms persisted in spite of antibiotic therapy, surgical drainage using a transnasal endoscopic approach was performed.     

Is hearing loss a feature of Joubert syndrome, a ciliopathy?

Objective

To assess if hearing loss is a feature of Joubert syndrome (JBS), one of the ciliopathies and therefore possibly associated with hearing loss.

Design

Retrospective case series.

Setting

University Children's Hospital.

Patients

Dutch patients with JBS.

Main outcome measures

Audiological data.

Results

Data from 22 Dutch Joubert syndrome (JBS) cases (17 males, 5 females) aged 3-40 years were available. Audiological tests were successfully performed in 14 cases. Three cases (aged 17-26 years) showed very mild sensorineural hearing loss (SNHL) at different frequencies. Conductive hearing loss due to middle ear infections occurred frequently in young JBS children (6 out of 22 cases). In three cases (aged 3-13 years) the parents reported the child was hypersensitive to sound.

Conclusion

We found no evidence for significant hearing loss in Joubert syndrome patients. However, given the compromised speech development in JBS, conductive hearing loss due to middle ear infections should be treated vigorously. SNHL at a later age cannot be excluded on the basis of our data, given the sample size. Three of the older cases showed discretely increased hearing thresholds. Analogous to the ciliopathy Bardet-Biedl syndrome, where hearing thresholds were reported to be subclinically increased in a group of adolescents patients, we recommend follow-up of JBS patients in view of the possibility of progressive, late-onset SNHL.     

When is a conductive hearing loss not a conductive hearing loss? Causes of a mismatch in air-bone threshold measurements or a "pseudoconductive" hearing loss

The otolaryngologist is sometimes faced with a patient with an apparent conductive hearing loss on audiometric testing who appears to have normal and mobile middle ear structures on ear exploration. Some causes of these "pseudoconductive" hearing losses can be diagnosed without resorting to surgery. This article reviews several possible mechanisms for this condition with diagnostic procedures to distinguish etiologic diseases. Conditions discussed include poor audiometry, functional hearing losses, "third window" causes, obliterated round window, abnormal inner ear connection with the cerebrospinal fluid space, inner ear mechanical conductive hearing loss, and apparent inner ear loss owing to missed middle ear pathology.     

Angiolipoma presenting as a nasal mass: how rare a presentation?

Angiolipomas are benign adipose tumors. There are two types: infiltrating and noninfiltrating. An angiolipoma presenting as a nasal mass is rare. To our knowledge, this is only the second documented case of angiolipoma presenting in the nasal dorsum. Total surgical excision by an external rhinoplasty technique with close follow-up is advised. Recurrence is not common.     

Deafblindness: a challenge for assessment--is the ICF a useful tool?

The International Classification of Functioning, Disability and Health (ICF) is considered to be a big step forward in relation to the possibilities for describing disabilities. The aim of this paper is to highlight some strengths and limitations in the ICF that have been found during a study of people with deafblindness. Thirty-two adults aged 19-92 years, totally deaf and blind, as well as those with some remaining vision and hearing, were included. Questionnaires and personal interviews were used. The participants had difficulties in activities and participation on almost all investigated items. The following five circumstances could not be taken account of: fast variation in functioning due to different personal and environmental factors; choosing not to do an activity because of a health condition; time loss impacting on quality of life; health risks related to particular impairments; and obligations. The ICF needs to address these questions and needs to be further developed.     

Review of a single surgeon’s stapedotomy cases performed with a nickel titanium prosthesis over a 14-year period

Abstract

Conclusion: Stapes surgery with a nickel titanium prosthesis is a safe and well-tolerated procedure that leads to a significant improvement in hearing outcomes.

Objective: To identify the efficacy and safety of stapedotomy procedures performed with a nickel titanium prosthesis for patients with otosclerosis.

Methods: A review of 431 unique stapedotomies performed over 14 years by a single surgeon at an academic tertiary care center yielded 312 cases with nickel titanium prosthesis that met inclusion criteria of otosclerosis diagnosis, initial surgery in operative ear, and presence of pre-operative and post-operative audiograms. Pure-tone averages (PTA) at baseline and 8 weeks after surgery were calculated over four frequencies; 0.5, 1, 2, and 4?kHz. Average air–bone gaps (ABG) were calculated from pre-operative and post-operative audiograms.

Results: Average pre-operative baseline PTA was 56.7?dB in the affected ear. Post-operative PTA was 30.1?dB, a 26.6?dB improvement. Initial average ABG was 29.7?dB, while post-operative ABG averaged 5.4?dB, a 24.2?dB improvement. Surgical success (closure of ABG within 10?dB) was achieved in 263 (84%) patients. Rate of surgical success was not correlated with age, gender, race, or affected ear. Complications included recurrent conductive hearing loss (14), progressive SNHL (4), and post-operative BPPV (3).     

Who should be a stapes surgeon? An otologist or a generalist?

PURPOSE OF REVIEW: The overall experience with stapes surgery has declined, both within residency training programs, as well as in clinical practice. Does this change in the environment suggest that subspecialists rather than generalists manage patients with otosclerosis? RECENT FINDINGS: A decreasing availability of patients with clinical otosclerosis has encouraged trainees and practitioners to adopt strategies that will enable the maintenance of quality care to these patients. SUMMARY: Well trained generalists should be prepared to perform stapes surgery. Lack of experience or infrequent exposure to disease suggests that optimal care can be achieved by referring the patient to an experienced otologic surgeon.     

Teratoma of the middle ear: a real entity or a non-entity?

Space-occupying lesions of the middle ear range from wholly benign developmental anomalies to highly virulent and aggressive malignancies. Amongst the benign entities classed with the family of developmental anomalies, the middle ear choristoma is well known. But what of the middle ear teratoma? Recent reports suggest that, while rare, teratomas of the middle ear do exist. Middle ear teratomas are defined as tumors consisting of benign tissues derived from all three embryologic layers: endoderm, mesoderm and ectoderm. They are lesions of the neonatal period and early infancy. While complete surgical excision will be curative, a thorough histologic examination of the specimen is recommended to exclude the possibility of immature and/or frankly malignant elements being admixed with the benign teratomatous elements.     

Tonsillar carcinoma — a review

Tonsillar carcinoma which is a not uncommon form of head-neck malignancy is reviewed.     

Kimura’s Disease — report of a case masquerading as a parotid tumor

Kimura’s Disease is a part of an ill- defined spectrum of vascular lesions of the subcutis. It is a benign chronic inflammatory condition producing subcutaneous tumour like nodules chiefly in the head and neck region with predilection for periauricular areas. It is characterised histologically by dense fibrosis, lymphoid infiltration, vascular proliferation and eosinophilia. The condition has a preponderance in the for eastern countries. It is extremely uncommon in Indian subcontinent. There has been no reported case from the Indian subcontinent in the English Literature. We report such a rare case in which the condition presents as parotid masses. We also present its CT, MRI & histopathology features and a brief review of literature.     

Why do we have a uvula?: literature review and a new theory

From ancient times up to the present day many different functions and conditions have been attributed to the uvula, many speculative and some with a more scientific basis. The uvula has been shown to have the ability to produce and secrete large quantities of thin saliva. A common complication of surgery involving removal of the uvula is pharyngeal dryness. We have observed that on phonation and swallowing the uvula swings back and forth in the oropharynx. We present a review of the literature on the uvula and propose a theory that the uvula bastes the throat and thereby helps keep it moist and well lubricated.