Otitis media with effusion as an initial manifestation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis is a systemic vasculitis of unknown etiology, characterized by necrotizing granulomas. It is an autoimmune disease affecting small- and medium-sized vessels of upper and lower respiratory tract, kidneys, and other organs. We described a case of a patient with otitis media with effusion as the first manifestations of granulomatosis with polyangiitis. A 54-year-old female presented as an urgent case with history of a severe otalgia, hearing loss, vertigo, and fever. The patient was treated with diagnosis of otitis media with effusion and acute rhinosinusitis, but without significant success. She developed an acute kidney dysfunction as a sign of glomerulonephritis with rapidly progressive renal failure. Diagnosis of granulomatosis with polyangiitis was confirmed after the histopathological analysis of kidney tissue, not by analysis of middle ear and paranasal sinus mucosa specimens. The patient was treated according to generally accepted protocol, and over time, there was an almost complete recovery.

Recurrent malignant otitis externa with multiple cranial nerve involvement: A case report.

Introduction: Necrotizing otitis externa is a rare but conditionally fatal infection of external auditory canal with extension to deep soft tissue and bones, resulting in necrosis and osteomyelitis of the temporal bone and scull base. This condition is also known as malignant otitis due to an aggressive behavior and poor treatment response. Early diagnosis of malignant otitis is a difficult challenge. We present an illustrative case of necrotizing otitis externa and suggest some strategies to avoid diagnostic and treatment pitfalls. Case Outline: A 70-year-old patient presented with signs of malignant otitis externa, complicated by peripheral facial palsy. Adequate diagnostic and treatment procedures were performed with clinical signs of resolution. The recurrence of malignant infection had presented three months after previous infection with multiple cranial nerve neuropathies and signs of jugular vein and lateral sinus thrombosis. An aggressive antibiotic treatment and surgery were carried out, followed by substantial recovery of the patient and complete restoration of cranial nerves' functions. Conclusion: Necrotizing otitis externa is a serious condition with uncertain prognosis. The suspicion of malignant external otitis should be raised in cases of resistance to topical treatment, especially in patient with predisposing factors. Evidence-based guideline for necrotizing otitis externa still doesn't exist and treatment protocol should be adjusted to individual presentation of each patient.

Bilateral congenital cholesteatoma of the temporal bone in Crouzon syndrome.

INTRODUCTION: Crouzon syndrome is an autosomal dominant genetic disease characterized by bicoronal craniosynostosis, exorbitism with hypertelorism, and maxillary hypoplasia with mandibular prognathism. CASE OUTLINE: We present the first reported case of Crouzon syndrome associated with a bilateral con- genital cholesteatoma of the temporal bone and discuss about the potential pathogenesis. CONCLUSION: Early diagnosis and management are crucial to prevent complications and an otologist should be an integral part of the multidisciplinary team.

Anatomical variations and relations of large blood vessels to the tympanic cavity.

PURPOSE: The aim of this study was to assess the protrusion of the upper bulb of the internal jugular vein (UBJV) and the internal carotid artery (ICA) into the cavum tympani, the thickness and the structure of the bone wall that separates these blood vessels from the middle ear cavity, as well as the bone wall absence between these blood vessels and the cavum tympani. METHODS: The study included a total of 150 samples of temporal bones of elderly people, both sexes. The methods used in the study were anatomic and histologic, while analyses were done by a surgical microscope. RESULTS: In 38 (25.3%) of the 150 studied temporal bones, the UBJV protruded into the cavum tympani, elevating its bottom. In 3 (7.8%) of the samples the bulb had a high position, thus filling the hypotympanum, and closing the lower half of the fenestra rotunda. The most frequent thickness of the bone wall that separates the ICA and the Eustachian tube was 2 mm (48.2%), less frequently it was 2-4 mm (29.6%), while least frequently it was of paper consistency (22.2%). CONCLUSION: High jugular bulb, aberrant ICA and anomalies of their wall structures are a pitfall and risk for middle ear surgery. Awareness of this variation is very important in the presurgical evaluation of the temporal bone to avoid vascular injury.

Congenital cholesteatoma of mastoid origin--a multicenter case series.

BACKGROUND/AIM: The mastoid is the rarest site for the onset of congenital cholesteatoma (CC). The symptoms are atypical and minimal. The aim of this multicenter retrospective descriptive study was to define this extremely rare condition and its clinical presentation, diagnosis and management. METHODS: We analyzed data files for a 15-year period in 4 tertiary otology centers and discovered 6 patients with the diagnosis of CC of the mastoid. RESULTS: The clinical presentation of CC varied from incidental findings in patient to patient with otogenic meningitis. The most common findings during surgical procedures were mastoid cortex erosion, sigmoid plate dehiscence, dural exposure and external canal wall destruction. CONCLUSION: CC of mastoid origin tends to occur in adult patients probably because of minimal symptoms and the delayed diagnosis. It can exist for years in a nonaggressive state and develop to giant sizes. In children it is almost incidentally diagnosed. Early imaging is necessary in order to prevent serious complication.

Why do treatment failure and recurrences of benign paroxysmal positional vertigo occur?

OBJECTIVE: To investigate the potential risk factors associated to the treatment failure and recurrence of benign paroxysmal positional vertigo (BPPV). STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center. PATIENTS: Four hundred patients with benign paroxysmal positional vertigo, 119 men and 281 women, aged 27 to 88 years. INTERVENTION: Patients were treated once a week, with only one, appropriate, depending on the affected canal, repositioning maneuver (modified Epley, Semont, barbecue/inverted Gufoni, Kim). The control Dix-Hallpike test and the roll test were performed on 7 days. MAIN OUTCOME MEASURES: The treatment outcome and recurrence were evaluated with regard to sex, age, duration of symptoms, etiologic factors, migraines, osteoporosis, vascular risk factors, endocrine diseases, localization of otoconia, and simultaneous involvement of multiple canals. RESULTS: The results indicate that treatment was negatively affected by patients' age, osteoporosis, and head trauma, without them causing recurrent symptoms. The highest number of uncured patients was observed in the 73- to 88-year-old age group (14.8%). The application of more than one maneuver was necessary in 27.5% of cases with primary BPPV and 88.9% with secondary BPPV. The highest treatment success was achieved in the group with BPPV of the posterior semicircular canal (F = 3.668, p = 0.026). The recurrence rate was 15.5%. CONCLUSION: Potential risk factors associated to the treatment failure were as follows: the age older than 50, secondary BPPV, head trauma, the occurrence of osteoporosis, and localization of otoconia in the anterior semicircular canal. The analyzed factors did not have impact on the recurrence.

Randomized prospective trial of hyperbaric oxygen therapy and intratympanic steroid injection as salvage treatment of sudden sensorineural hearing loss.

OBJECTIVE: To compare the effects of hyperbaric oxygen (HBO) and intratympanic (IT) steroid injection on hearing after the failure of primary treatment in patients with idiopathic sudden sensorineural hearing loss (ISSHL). STUDY DESIGN: A prospective randomized trial. SETTING: Tertiary referral center. PATIENTS: Fifty patients with failure of primary therapy for ISSHL. INTERVENTION(S): After primary treatment with systemic steroids and failure of therapy, defined as less than 10-dB hearing gain, 50 patients were enrolled in the study and received either hyperbaric oxygen or intratympanic steroid treatment. The patients were not matched and not similar. MAIN OUTCOME MEASURE(S): Hearing gain at 0.25, 0.5, 1, 2, and 4 kHz after treatment. RESULTS: There were significant differences between hearing thresholds at all frequencies before and after the HBO treatment. Similarly, there were significant differences between hearing thresholds at most frequencies (except 2 kHz) before and after the treatment in the IT group. The subgroups of patients with pure tone average less than 81 dB and were younger than 60 years had better response to HBO treatment than those with profound deafness and in the elderly. CONCLUSION: HBO and IT steroid therapy could be successfully used as salvage therapies in patients with sudden deafness. Further study is needed to demonstrate superiority of one of the treatments.

External ear canal cholesteatoma after ventilation tube insertion and mastoidectomy.

INTRODUCTION: Etiopathogenetically, there are two types of chollesteatomas: congenital, and acquired. Numerous theories in the literature try to explain the nature of the disease, however, the question about cholesteatomas remain still unanswered. The aim of the study was to present a case of external ear canal cholesteatoma (EEC) developed following microsurgery (ventilation tube insertion and mastoidectomy), as well as to point ant possible mechanisms if its development. CASE REPORT: A 16-year-old boy presented a 4-month sense of fullness in the ear and otalgia on the left side. A year before, mastoidectomy and posterior atticotomy were performed with ventilation tube placement due to acute purulent mastoiditis. Diagnosis was based on otoscopy examination, audiology and computed tomography (CT) findings. CT showed an obliterative soft-tissue mass completely filled the external ear canal with associated erosion of subjacent the bone. There were squamous epithelial links between the canal cholesteatoma and lateral tympanic membrane surface. They originated from the margins of tympanic membrane incision made for a ventilation tube (VT) insertion. The position of VT was good as well as the aeration of the middle ear cavity. The tympanic membrane was intact and of normal appearance without middle ear extension or mastoid involvement of cholesteatoma. Cholesteatoma and ventilation tube were both removed. The patient recovered without complications and shortly audiology revealed hearing improving. Follow-up 2 years later, however, showed no signs of the disease. CONCLUSION: There could be more than one potential delicate mechanism of developing EEC in the ear with VT insertion and mastoidectomy. It is necessary to perform routine otologic surveillance in all patients with tubes. Affected ear CT scan is very helpful in showing the extent of cholesteatoma and bony defects, which could not be assessed by otoscopic examination alone.

Prognostic significance of tympanosclerotic plaques localization and their morphological and histological characteristics for the outcome of surgical treatment.

BACKGROUND/AIM: Tympanosclerosis is a sequela of inflammation of the middle ear usually causing conductive hearing loss. The aim of the study was to determine the significance of tympanosclerotic plaques localization in the middle ear and their morphological and histological characteristics for surgical treatment outcome. METHODS: This retrospective study included a total of 73 patients operated on for tympanosclerosis in the Clinic for Otorhinolaryngology, Military Medical Academy (MMA) in a period 1996-2010. The results of surgical treatment as well as the last audiometry findings were analyzed considering follow-up periods of 6 months to 8 years. The patients were divided into 4 groups according to tympanosclerotic plaques localization in the middle ear and the classification suggested by Wieling and Kerr. The patients were also divided based on intraoperatively noticed morphological characteristics of tympanosclerotic plaques, while the third division was done as per histological findings. Surgical success was assessed using the suggestions of the Japan Otological Society. RESULTS: The analyzed results showed the surgical success especially in the group II according to Wieling and Kerr, while histological findings had no impact on the outcome of the surgery. CONCLUSION: Surgical treatment has good results especially in patients with the mobile stapes. Results are satisfactory in other localizations, while various morphological and histological characteristics do not have impact on the surgery outcome.

[The impact of hearing loss on the quality of life in adults].

INTRODUCTION: The hearing apparatus is one of the most important factors related to the development of oral communication. Thus, hearing disorders and deafness lead to severe handicap. Hearing impairment in adults cause verbal communication disorders that influence psychical, emotional and social functioning. Nowadays, there is a noticeable world tendency towards improving hard of hearing person's quality of life. OBJECTIVE: Objective was to assess the association between hearing impairment and health-related quality of life. METHODS: A hundred adults with billateral hearing impairment underwent hearing examination and answered the Hering Handicap Inventory for the Elderly-Screening (HHIE-S, Ventry and Weinstein), specific for hearing impairment. RESULTS: Almost half of all participants (44%) had a moderate hearing loss, and 36% had a mild loss. Infrequently, participants had a severe degree of hearing loss (13%) and deafness (7%). Self reported hearing handicap revealed significant emotional, social and situational dysfunctions (chi2 = 40.960; df = 1; p < 0.01). Severity of hearing loss was significantly correlated with hearing handicap (r = 0.212; p < 0.05). More often, participants revealed social and situational than emotional hearing handicap (Chi2 = 131.89; df = 100; p < 0.05). Only 12% of all participants habitually used hearing aids, and they observed a significantly better quality of life scores (chi2 = 6.23; df = 1; p < 0.05). CONCLUSION: Health-related quality of life must be estimated as a factor of great importance. Investigations should involve a more extansive population with hearing loss and a national programme should be started.

[Chronic suppurative inflammation of the middle ear in children].

INTRODUCTION: Chronic suppurative inflammation of the middle ear (HGO) in children represents one of the most frequent infections in childhood, even in children from developed industrial countries. We can distinguish two types of chronic suppurative inflammation: first--HGO without cholesteatoma and second HGO with cholesteatoma. OBJECTIVE: The objective of the paper was to point out the characteristics of chronic suppurative inflammation of the middle ear in children and possible methods of treatment. METHOD: From 2000 to 2005, our retrospective study involved 92 children, aged 2 to 16 years, treated for chronic suppurative inflammation of the middle ear and subsequent otogenic complications. The diagnosis was established based on clinical symptoms, otoscopic and otomicroscopic findings. Treatment included the removal of the pathological process, reconstruction of the hearing chain and prevention recurrence. RESULTS: We performed 111 surgical interventions. Children were divided into two groups: in the first group, we performed 56 surgical interventions in 48 children diagnosed with chronic suppurative otitis without cholesteatoma, and in the second group we performed 55 surgical interventions in 44 children who were diagnosed with chronic otitis with cholesteatoma. In the first group we performed mastoidectomy in 17 (32.5%) children, mastoidectomy and posterior aticotomy in 5 (9.5%), miringoplasty in 22 (35.8%), timpanoplasty type II in 5 (9.5%), timpanoplasty type III in 3 (5.6%) and timpanomastoidectomy in 4 (7.1%) children. In the second group, consisting of children diagnosed with cholesteatoma of the middle ear, we performed 47 first act surgeries, using closed technique in 17, and open in 30 cases. We performed timpanoplasty type III in 17 (30.9%) and timpanomastoidectomy in 17 (30.9%), and timpanoplasty type II in 9 (16.4%), timpanoplasty type IV in 10 (18.2%) and mastoidectomy in 2 (3.6%) children. In the group treated by closed surgical technique recurrence occurred in 5 (29.4%), and in those treated by open technique in 3 (10.3%). Two children had bilateral cholesteatoma. CONCLUSION: Further research should be directed toward the identification of different types of factors and pathogenesis of HGO. Treatment of this decease is still considered controversial. Hinolon drops could represent a promising option in the treatment of HGO. The main objective in the treatment of cholesteatoma should be the improvement of the open technique to prevent retraction pockets, decrease of the rate of residual or atelectatic process and the improvement of hearing results.

[Newborn hearing screening].

INTRODUCTION: Prevalence of sensorineural hearing loss is 1-3 per 1,000 newborns. Transient evoked otoacoustic emission (TEOAE) and automated auditory brain stem responses (AABR) are most frequently used methods in newborn hearing screening programmes. OBJECTIVE: The aim of this study was to examine hearing function in newborns with and without risk factors for hearing loss. We investigated accuracy and feasibility of two automated technologies: transient otoacoustic emissions (TEOAE) and auditory brain stem response (AABR) in early detection of hearing loss. METHOD: In prospective study, 907 newborns were tested on both ears with transient evoked otoacoustic emissions (TEOAE). If results were "refer" we performed automated brain stem response (AABR). Two stage screening protocols were used with two screening technologies (TEOAE, AABR). RESULTS: Results showed screening pass of 86.3% of the newborns in the first protocol and 99.3% in the second. Six (0.7%) newborns had positive screening results for hearing loss. They were referred for additional audolologic tests (otoacoustic emissions, tympanometry, and auditory brain stem response) to confirm or exclude hearing loss. Audiologic examination was performed up to the third month of life. We confirmed unilateral sensorineural hearing loss in two babies. Average test time per ear was 21.3 +/- 19.4 s forTEOAE and 135.3 +/- 67.9 s for AABR. CONCLUSION: TEOAE, AABR tests are confidential, noninvasive and feasible methods and can help to detect hearing impairment.

Stapes pathology in otosclerosis: scanning electron microscopic examination.

The human stapes is still a subject of considerable interest both to the clinician and to the research investigator. This is due to the stapes position between the middle and inner ear, and because of its involvement in the otosclerotic process. The purpose of this study was to determine scanning electron microscopic and histopathological features of normal and otosclerotic stapes. The specimens were obtained from adult human temporal bones and during operation of patients with otosclerosis. Our results show that the surface of the normal stapes had various degrees of structural features. The otosclerotic stapes is characterized by a typical irregular pattern. Histological examination of the stapedial footplate or its fragments showed three types of otosclerotic lesions: fibrotic, sclerotic, and spongiotic. Our observations could support the hypothesis that otosclerosis is not a static disease.

Anatomical study of the human middle ear for the design of implantable hearing aids.

OBJECTIVE: To generate anatomical data on the human middle ear and adjacent structures to serve as a base for the development and optimization of new implantable hearing aid transducers. Implantable middle ear hearing aid transducers, i.e. the equivalent to the loudspeaker in conventional hearing aids, should ideally fit into the majority of adult middle ears and should utilize the limited space optimally to achieve sufficiently high maximal output levels. For several designs, more anatomical data are needed. METHODS: Twenty temporal bones of 10 formalin-fixed adult human heads were scanned by a computed tomography system (CT) using a slide thickness of 0.63 mm. Twelve landmarks were defined and 24 different distances were calculated for each temporal bone. RESULTS: A statistical description of 24 distances in the adult human middle ear which may limit or influence the design of middle ear transducers is presented. Significant inter-individual differences but no significant differences for gender, side, age or degree of pneumatization of the mastoid were found. Distances, which were not analyzed for the first time in this study, were found to be in good agreement with the results of earlier studies. CONCLUSION: A data set describing the adult human middle ear anatomy quantitatively from the point of view of designers of new implantable hearing aid transducers has been generated. In principle, the method employed in this study using standard CT scans could also be used preoperatively to rule out exclusion criteria.