Chronic skin lichenification as unusual presentation of eosinophilic granulomatosis with polyangitis: case report and literature review.

Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually developed a full-blown ANCA-MPO positive EGPA in combination with blood hypereosinophilia, eosinophilic vasculitis at skin biopsy, subclinical asthma and chronic rhinosinusitis.

The incidence of cardiovascular events is largely reduced in patients with maximally tolerated drug therapy and lipoprotein apheresis. A single-center experience.

AIM: Lipoprotein apheresis (LA) is the elective therapy for homozygous and other forms of familial hypercholesterolemia (FH) and familial combined hypercholesterolemia (FCH), resistant/intolerant to lipid lowering drugs, and hyperlipoproteinemia(a) for which drugs are not available. To assess the effect of LA on the incidence of adverse cardiac or vascular events (ACVE) at the time period of pre-initiation of apheresis and during the LA treatment. METHODS: We collected data of 30 patients (mean age 62 ± 8 years, males 73%), with FH, or FCH and cardiovascular disease on maximally tolerated lipid lowering therapy and LA treatment (median 5 years, interquartile range 3-8 years). Associated hyperlipoproteinemia(a) was present in 16/30 subjects. The LA treatment was performed biweekly as clinically indicated by dextran-sulfate or heparin-induced LDL precipitation apheresis. The ACVE incidence, before and after treatment, was evaluated by statistical analyses. RESULTS: The ACVE incidence occurred before and after the LA treatment inception, were 86 and 15 events respectively. Notably, 6/15 of ACVE were secondary to stent restenosis and 7/15 follow-up events occurred during the first 5 years. The AVCE rates/year were 0.58 and 0.13 respectively (p < 0.001). CONCLUSIONS: Our data confirm long-term efficacy and positive impact of LA on morbidity in patients with FH and FCH and atherosclerotic disease at maximally tolerated lipid lowering therapy.

Cochlear implantation in chronic otitis media and previous middle ear surgery: 20 years of experience.

Cochlear implantation in the setting of chronic otitis media or previous middle ear surgery poses several problems for the surgeon: possible spread of infection to the cochlea and the subarachnoid spaces with consequent meningitis, risk of electrode array extrusion and possible recurrence of the original disease. Several surgical strategies have been proposed to overcome these problems. In the present study, clinical and functional results of cochlear implantation in 26 patients with chronic otitis media (8 cases) or previous middle ear surgery (18 cases) in the ear most suitable for implantation were retrospectively reviewed. Among the 8 patients with chronic otitis media, in 7 cases a subtotal petrosectomy associated with external auditory canal closure and mastoid and Eustachian tube obliteration was performed, while in the remaining patient cochlear implantation was done 6 months after a myringoplasty. The only complication observed was a reperforation of the tympanic membrane in this latter patient. Among the 18 patients with previous middle ear surgery, 2 had undergone intact canal wall tympanomastoidectomy and were implanted utilising the previous surgical approach. In the remaining 16 patients who had a radical cavity, an open technique was maintained in 3 cases; a cavity revision associated to external auditory canal closure, Eustachian tube and mastoid obliteration was performed in 12 patients, while in one case a middle cranial fossa approach was utilised. Two of the 3 patients in whom an open technique was maintained have experienced electrode array extrusion. The only complication observed in the remaining patients was the breakdown of the external auditory canal closure in one case. No problems were noted in patients who had undergone intact canal wall tympanomastoidectomy as well as in the subject implanted via the middle cranial fossa approach. All patients achieved and maintained good hearing performance over time. Subtotal petrosectomy associated with external auditory canal closure, Eustachian tube occlusion and mastoid obliteration is an effective procedure to facilitate cochlear implantation in presence of chronic otitis media. The open cavity technique offers the advantage of a close clinical examination, but may expose the patient to the risk of electrode array extrusion, mainly in the long-term period.

Long-term surgical and functional outcomes of the intact canal wall technique for middle ear cholesteatoma in the paediatric population.

In this paper, we report the postoperative outcomes in canal wall up procedures with second stage surgery in 40 children undergoing intervention for cholesteatoma of the middle ear. The residuals, recurrences and the hearing results were analysed. All 40 patients had a follow-up of at least five years. Of the 39 patients who underwent two staged surgery, 18 (46.1%) had a residual lesion that was identified and excised during the second surgery. Over a five year follow-up period, there were five (12.5%) patients with recurrences, all belonging to the group in whom a residual cholesteatoma was identified during the second staged surgery. The rate of residual cholesteatoma tends to decrease as age increases. The type of cholesteatoma, acquired or congenital middle ear, were not statistically related to the incidence of residual cholesteatoma. Hearing analysis showed that hearing recovery was excellent with canal wall up procedures and remained stable over five years.

Bilateral congenital cholesteatoma: report of an emblematic case.

OBJECTIVES: To describe a case of bilateral congenital cholesteatoma (CC) of the middle ear with a focus on diagnostic clues, treatment and a review of the pertinent literature. PATIENT AND METHODS: An 8-year-old child was incidentally noted to have whitish bilateral retrotympanic masses with normal hearing and referred to our department in January 2005. Microscopic examination of the ears and CT scan of the temporal bones led to a presumptive diagnosis of bilateral CC. The lesion on the right side was surgically removed, followed by that on the left side after 6 months; a retroauricular transcanal approach was adopted in both ears. RESULTS: Anatomic integrity of the middle ear was achieved with preservation of pre-operative hearing. No signs of recurrence were evident 20 months after the last surgery. CONCLUSIONS: Bilateral CC is a rare finding but otologists must be aware of it. Surgery must be planned early in order to achieve radical removal of the pathology and the preservation of middle ear structures.

Stress echocardiography.

Stress echocardiography is the combination of 2D echocardiography with a physical, pharmacological or electrical stress. The diagnostic end point for the detection of myocardial ischemia is the induction of a transient worsening in regional function during stress. Stress echocardiography provides similar diagnostic and prognostic accuracy as radionuclide stress perfusion imaging, but at a substantially lower cost, without environmental impact, and with no biohazards for the patient and the physician. Among different stresses of comparable diagnostic and prognostic accuracy, semisupine exercise is the most used, dobutamine the best test for viability, and dipyridamole the safest and simplest pharmacological stress and the most suitable for combined wall motion--coronary flow reserve assessment. The additional clinical benefit of myocardial contrast echocardiography, tissue Doppler imaging and real time 3-D echocardiography has been inconsistent and disappointing, whereas the potential of adding coronary flow reserve evaluation of left anterior descending coronary artery by transthoracic Doppler echocardiography adds another potentially important dimension to stress echocardiography. In spite of its dependence upon operator's training, stress echocardiography is today the best possible imaging choice to achieve the still elusive target of sustainable cardiac imaging in the field of noninvasive diagnosis of coronary artery disease.

Paget's disease and cochlear implantation.

Paget's disease of bone is a common disorder of unresolved etiology characterized by excessive bone resorption followed by excessive bone formation. If the skull is affected this may result in hearing loss and eventually develop into profound deafness. To date, no cases of cochlear implantation in patients with Paget's disease have been reported. The authors present a case of radiographically confirmed Paget's disease of the skull in a 77-year-old man with a 20-year history of progressive bilateral sensorineural hearing loss who underwent cochlear implantation. A successful insertion of the Nucleus 24 Contour electrode array was achieved without surgical and postoperative complications. At the 10 months' postoperative evaluation, the patient had gained useful open-set speech perception. In quiet conditions, his performance scores on the word and sentence recognition tests were 100 and 98 per cent, respectively. In the presence of noise (at +10 dB. signal-to-noise ratio), his performance scores on the word and sentence recognition tests were 96 and 94 per cent, respectively.

Speech recognition in elderly cochlear implant recipients.

The benefits of cochlear implantation in the adult and paediatric populations are well established. Cochlear implantation in the geriatric population still remains controversial because of the misconception that elderly patients might perform poorly. The purpose of this study was to report the speech performance of 16 patients over 65 years of age implanted with a Nucleus multichannel cochlear implant and to compare it with that of a control group of 14 adults aged between 41 and 59 years. At the 12 months postoperative evaluation, no significant differences were detected on speech performances between the elderly patients and the control group. The mean word recognition scores were 72.5% for the elderly group and 82% for the control group. The mean everyday sentence recognition scores were 72.5% for the elderly group and 85.7% for the control group. Overall, the results are encouraging and demonstrate that the elderly population with profound hearing loss obtain significant benefits from cochlear implantation despite the age-related auditory processing problems.

[Multi-channel cochlear implant in cochlear ossification]. / L'impianto cocleare multicanale nelle ossificazioni cocleari.

Cochlear ossification, considered until only a few years ago as a contraindication for cochlear implants (C.I.), may now be managed by means of a wide variety of surgical techniques. In cases with massive ossification, the drill-out circummodiolar technique described by Gantz et al. in 1988 and successively modified by Balkany et al. in 1997 may be adopted. The technique of electrode insertion in the scala vestibuli, perfected by Steenerson et al. in 1990, may be used when cochlear ossification has spread no further than the scala tympani. Other methods call for a groove to be drilled along the proximal tip of the basal turn of the cochlea (Cohen and Waltzman, 1993), the insertion of electrodes through the middle cranial fossa (Colletti et al., 2000), or the utilization of a double electrode array (Bredberg et al., 1997, Lenarz et al., 2001). This study reports the experience conducted at the Cochlear Implants Centre of the Otorhinolaryngoiatrics, Otological and Otoneurological Microsurgery Section of the University of Parma in a group of 15 patients who underwent C.I. in the presence of varying degrees of ossification. In 3 cases the ossification was limited to the region of the round window and a few millimetres of the scala tympani; cochleostomy was performed anteriorly and inferiorly to the anterior niche of the round window. In 11 cases (of which 3 of pediatric age), the ossification had spread to the horizontal portion of the scala tympani; in these cases, the electrodes were inserted in the scala vestibuli. The scala vestibuli was opened by drilling anteriorly to the round window and superiorly to the spiral ligament. In the only case of massively ossified cochlea, it was possible to partially insert the electrodes in a circum-modiolar tunnel. In the 12-month follow-up hearing test, the 3 patients with ossification of the round window region and the first millimetres of the scala tympani respectively averaged 61.6% in recognizing 2-syllable words and 59% in recognizing words embedded in phrases. The averages on the 12-month follow-up hearing test in the 8 adult patients who received the implant in the scala vestibuli were 80.6% in recognizing 2-syllable words and 89.1% in recognizing words in phrases. The 3 pediatric patients were classified on the Geers and Moog scale, which situated 2 of them in the 6th category of perception and 1 of them in the 4th category of perception. As regards the only case of massive cochlear ossification, the patient underwent surgery recently, and the sole follow-up available is the one conducted after only 3 months; the vowel identification average was 55%; the average on the VCV test was 31%; and the 2-syllable word recognition average was 20%.

Congenital cholesteatoma of the tympanic membrane.

We present two cases of congenital cholesteatoma of the tympanic membrane. Congenital cholesteatoma within the tympanic membrane is a rare entity with only few cases documented. The aetiopathogenesis of this lesion is still unknown. An embryologic origin is hypothesized when cholesteatoma develops in patients without previous history of otitis as in the two cases we report. In cases with previous history of inflammatory process of the external or middle ear an acquired origin is suspected due to the proliferation of the basal cell layer of the tympanic membrane epithelium. Despite the rarity of the congenital tympanic membrane cholesteatoma, we think that its early diagnosis is of utmost importance to allow an easy removal and avoid middle ear involvement.

Cochlear effects of mesna application into the middle ear.

Mesna (sodium 2-mercapto-ethane sulphonate) belongs to a class of thiol compounds that produce mucolysis by disrupting the disulphide bonds of the mucus polypeptide chains. The registered indications of mesna include the treatment of pathologies of the respiratory tract and, in oncology, the prevention of toxic lesions of the urinary tract by antineoplastic agents. In the E.N.T. Clinic of the University of Parma, it has been found that mesna can be used to facilitate the dissection of the various tissue layers in any surgical procedure. One of these indications is surgical treatment of cholesteatoma, which is mainly composed by keratin, a protein rich is disulphide bonds that are easily disrupted by mesna. The aim of this study was to evaluate the toxicity of mesna application into the middle ear on the cochlear anatomy and physiology. Three groups of guinea pigs were used as subjects. Mesna solution (10 or 20%) was applied in one ear, while the opposite ear received a placebo (saline solution). Toxicity of mesna was assessed by means of transmission electron microscopy (TEM), scanning electron microscopy (SEM), and auditory brain-stem response (ABR). TEM and SEM did not show any toxic effect on cochlear morphology. There were no differences in ABR thresholds and wave III amplitude and latency between mesna-treated and control ears.

Scutumplasty: costal cartilage versus bone paté.

This study was carried out to evaluate the anatomical and hearing results of the reparation of attic defects in closed tympanoplasty. Reparation was carried out in 194 patients by using a costal cartilage allograft, and in 159 patients with a bone pate autograft. The follow-up was from 1 to 5 years. The study was not truly randomized owing to an occasional lack of allogenic costal cartilage. In the group 'costal cartilage' a partial resorption was observed in 5.7% and a complete resorption in 4.7% of the cases. In the group 'bone pate', partial resorption was observed in 5.5% and total resorption in 2.7% of the patients. Satisfactory hearing results were obtained in 86% of the patients of the group 'costal cartilage' and in 82% of the patients of the group 'bone paté'. Both graft materials may be recommended for repairing erosions caused by the cholesteatoma in the wall of the external auditory canal.

[Cleft palate and dysfunction of the eustachian tube]. / Labiopalatoschisi e disfunzione tubarica.

Secretory otitis media (SOM) is a frequent complication in infants with cleft palate. In cleft palate the muscles that open the Eustachian tube (tensor palatini and levator palatini) have abnormal connections thereby making the tube opening either difficult or impossible. This will lead to secretory otitis media in 95% of cases, since the middle ear will not be aerated. In this paper, 14 patients operated on for cleft palate during the first year of life were examined. SOM was treated only by medical therapy without the insertion of tympanostomy tubes. Post-operative follow-up ranged from 2 months to 5 years. In all patient SOM was still present at last follow-up with poor efficacy of medical therapy. Therefore, our therapeutic protocol includes myringotomy and insertion of tympanostomy tubes during the first general anesthesia for cleft palate treatment.

[Progressive sensorineural hearing loss: neoplastic causes]. / Le ipoacusie neurosensoriali progressive: le cause neoplastiche.

Acoustic neuromas represent about 90% of all space-occupying lesions of the cerebellopontine angle and account for approximately 6% of all intracranial tumors. Progressive unilateral sensorineural hearing loss is the most frequent initial symptom occurring in over 90% of patients. A sudden onset of hearing loss occurs in 5%, while 5% of cases present with normal hearing. The incidence of hearing loss does not seem to be related to tumor size. Accuracy of the tumor detection by gadolinium-enhanced magnetic resonance imaging has been reported as 99-100% even for intracanalicular tumors. The ABR false negative rate for intracanalicular tumors has been reported to be as high as 9-11%. Rare lesions of the cerebellopontine angle include: meningiomas, epidermoids, arachnoid cysts, trigeminal nerve neuromas, facial nerve neuromas, neurinomas of lower cranial nerves, glomus tumors and metastases. Therefore, a high suspicion index and awareness of symptoms and an adequate audiologic and neuroradiologic work-up are the clues to early diagnosis and appropriate surgical treatment.

[Intraoperative tonal-threshold audiometry in the surgery of otosclerosis]. / L'audiometria tonale liminare intraoperatoria nella chirurgia dell'otosclerosi.

At the end of stapedioplasty, performed under local anesthesia, the surgeon usually tests the hearing function making questions to the patient turning his voice from a soft whisper to a loud tone. At the ENT Department, University of Parma Italy, a more precise method is employed in order to measure the air conduction threshold of the patient at the beginning and at the end of surgery. From April 1996 to October 1996, intraoperative pure-tone audiometry was performed in 36 patients who underwent stapedioplasty. A portable audiometer "Amplaid 161/C Amplifon" was used in the operating room. Air conduction thresholds were measured at 125-8000 Hz. Intraoperative pure-tone audiometry allows an instantaneous and a more accurate evaluation of the surgical functional results.