Ioannis Kastanioudakis

Inner ear involvement in rheumatoid arthritis : a prospective clinical study

Sensorineural hearing loss in rheumatoid arthritis (RA) has been reported to be the result of the extra-articular manifestation of the disease (rheumatoid nodular vasculitis) or due to drug ototoxicity. In an attempt to investigate the presence of sensorineural hearing loss and the possible causes for it we investigated prospectively 45 RA patients (42 female; three male) with a mean age of 52.5 +/- 10.7 years and a mean disease duration of 8.5 +/- 7.3 years. All patients underwent a complete physical examination and audiological evaluation which included pure tone audiometry and impedance audiometry (tympanogram, static compliance, acoustic reflex, reflex decay, acoustic reflex latency test. We found a sensorineural hearing loss > 20 dB HL in 44.4 per cent (40/90) ears. In all cases the site of hearing loss was the cochlea and in most of them it was bilateral and symmetric (16 patients out of 45 had bilateral sensorineural hearing loss i.e. 35.5 per cent. There was no correlation between sensorineural hearing loss and age, sex, disease duration, articular and extra-articular manifestations and the presence of autoantibodies in our patients. In addition, no correlation was found between sensorineural hearing loss and drug therapy for one at least of the following drugs: NSAIDs, D-penicillamine, plaquenil and methotrexate. We noticed a prologation of acoustic reflex latency in five patients (10 per cent) which was found to be correlated with the temporomandibular joint involvement and the presence of rheumatoid factor (RF). We conclude that inner ear involvement in RA is expressed by: (1) mild symmetric, bilateral sensorineural hearing loss of cochlear type in 35.5 per cent of patients; (2) normal acoustic reflex thresholds; (3) nondecay; and (4) prologation of acoustic reflex latency which appeared in a small number of patients (10 per cent).

Ear involvement in systemic lupus erythematosus patients: a comparative study

Ear damage in systemic lupus erythematosus (SLE) patients has been occasionally reported but the frequency and the mechanisms of ear involvement are not well documented. In an attempt to investigate the presence of hearing loss and the possible causes for it we prospectively evaluated 43 SLE patients. All patients underwent a complete ear-nose-throat physical examination and audiological evaluation with pure tone, impedance and speech audiometry. In addition, systemic manifestations of the disease and drug therapy were recorded. Finally, all patients were tested for the presence of autoantibodies. The results were compared with those of 50 age-matched healthy subjects. Hearing loss (HL) was found in nine patients (22.5 per cent). More specifically, eight patients presented sensorineural hearing loss (SNHL) (21.5 per cent) and only one had conductive hearing loss (CHL) (2.63 per cent). From the patients with SNHL, one had bilateral symmetrical damage, four had bilateral but no symmetrical damage and three patients showed unilateral SNHL. Finally, the patient with CHL had unilateral involvement. There were no statistically significant differences between patients with HL and those without regarding age, disease duration, clinical disease manifestations, autoantibody profile and drug therapy. In conclusion, one fourth of our SLE patients presented HL, expressed as SNHL affecting mainly the middle and high frequencies, while only one patient had CHL. This is a lower percentage of ear involvement in SLE than that reported by other investigators. The mechanism of ear damage remains unknown. Thus, additional prospective studies are needed to elucidate its pathogenesis.

Hearing loss in progressive systemic sclerosis patients: A comparative study

OBJECTIVE: To investigate the middle and inner ear involvement in patients with progressive systemic sclerosis (PSS). STUDY DESIGN AND SETTINGS: We prospectively evaluated 34 PSS patients. All patients underwent a complete ear-nose-throat physical examination and audiological evaluation with pure tone, impedance, and speech audiometry. In addition, systemic manifestations of the disease and drug therapy were recorded. Finally, all patients were tested for the presence of autoantibodies. The results were compared with those of 45 age-matched healthy subjects. RESULTS: We found a sensorineural hearing loss in 20% and mixed type hearing loss in 3.3% of the patients. There was no correlation of hearing loss with age, systemic manifestations of the disease, presence of autoantibodies, and drug therapy. Ten percent of the patients had patulous eustachian tubes. CONCLUSION: One fourth of PSS patients had a hearing loss affecting the middle and mainly the high frequencies. This is a lower percentage than that reported by other investigators. A significant prevalence of bilateral patulous eustachian tubes was noticed as well. Further investigation is needed for a better understanding of the mechanism of ear damage in PSS patients.

Comparative study of evoked electromyography and facial nerve latency test in the prognosis of idiopathic facial nerve palsy in childhood

We report our results from the application of evoked electromyography (EEMG) and facial nerve latency testing (FNLT) in 30 children aged 4–14 years with idiopathic facial palsy. Our aim was to define the value of these tests as prognostic tools in Bells palsy. From the EEMG results we ascertained that, when the amplitude of the compound muscle action potential varied between 51% and 95% of the normal value, the neuronal damage is slight (neurapraxia). When the percentage value of muscle response decreases, the prognosis is worse. The results of the FNLT showed that, when the latency is within normal limits, the damage to the nerve is slight (neurapraxia). If the latency is prolonged, the prognosis is worse. Evaluation of our results and comparison with the index of facial nerve functional recovery showed that those two tests have a high percentage of correctness (100% for the EEMG and 96.7% for the FNLT) and a low percentage of error. We conclude that these tests are excellent for predicting the outcome of facial nerve palsy in childhood and we suggest that young patents undergo both so that the determination of the lesion may be as correct as possible.

Huge nasopharyngeal true teratoma with cleft palate and lip and unilateral choanal atresia.

The following report describes the case of a male born to 28-year-old healthy Greek woman. The baby was delivered by caesarean section at 34 weeks’ gestation and weighed 1.770 g at birth. The mother originally presented with hydramnios during week 34 of her pregnancy and ultrasound examination performed at this stage suggested fetal abnormality. Because of progressive dyspnea, the mother was transferred from an outlying hospital to Athens where a repeat ultrasound scan confirmed fetal abnormality. A caesarean section was undertaken to deliver the baby. The neonate presented with a prominent, mobile mass attached to his nasopharynx with a pedicle through a cleft palate and lip (Fig 1). After delivery of the baby, a second mass, resembling an upper extremity, was removed from the uterus. The baby was immediately transferred to a neonatal intensive care unit at a neighboring children’s hospital. On admission the infant was noted to be small for gestational dates. Clinical examination revealed: (1) a prominent mass 8-cm long with a pedicle about 2 cm thick extending from oral cavity and upper lip, hairs and islands of sclerotic tissue were evident on the surface of the mass; (2) cleft palate and lip; (3) bleeding from the area of the upper lip and cleft palate; and (4) unilateral choanal atresia. The neonate was hypotonic and cyanotic with signs of severe respiratory distress. Endotracheal intubation and umbilical artery catheterization were carried out and packed red cells were transfused. A CT scan revealed a huge congenital dysplasia originating from the neonate’s nasopharynx, extending through a cleft palate and lip to the oral cavity, with attached pedicle, protruding from the mouth. The mass incorporated fragments of spinal tissue, suspected cardiac ventricles, and pulmonary tissue. Malformations in the hard palate were also noted. No pathologic findings were found in the brain, posterior cranial fossa, and ventricles. In view of bleeding, the neonate was taken to the operating room. The mass was removed from the posterior wall of the nasopharynx with the patient under general anesthesia, revealing a huge defect between the oral and nasal cavities. The defect was occupied by the pedicle. The horizontal part of the palate was absent and surgical repair of the cleft lip and palate was carried out. The specimen’s dimensions were 8 × 4 × 4.5 cm. Its surface was rough and was covered with normal skin except the resection area. Histologic sections demonstrate mainly solid and partially cystic structure. The mass consisted of mature brain tissue, smooth muscle, fat, cartilage, and cystic glandular formations. In the resection area, a salivary gland was found. There was no evidence of malignancy. These findings were compatible with benign nasopharyngeal true teratoma. Postoperatively the baby developed metabolic acidosis, bleeding from the oral cavity, cyanosis, and mydriasis, and died 5 hours later as a result of cardiac arrest.

Cutaneous Horn of the Auricle

Abstract An 81-year-old man came to the ENT Department of the University Hospital in Ioannina Greece with a large cutaneous horn of the right auricle. The horn was almost 4.5 cm long, skin colored, and had a thick, reddened base and two antler-like projections (Fig. 1). Fig. 1. Cutaneous horn of the auricle. The lesion had first appeared on the upper part of the helix of the right auricle when the patient was 60 years old. Although originally resembling an animal horn in miniature, it had gradually grown to its current dimensions. Clinical examination revealed a hard, yellowish-brown excrescence, with two opposite antler-like projections, curved with circumferential ridges, and surrounded by a somewhat acanthotic collarette, 4.5 cm and 2.5 cm in height (Fig. 1). The horn together with its base was surgically excised with the patient under local anesthesia, and the skin defect was reconstructed with a local skin flap. Histologic examination of the specimen revealed parakeratotic keratin masses consisting of an extensively thickened epidermis with atypical cells. No invasion of squamous cells into the dermis was seen. The auricle is almost normal 2 years after the patient’s initial treatment.

Diagnosis of Sensorineural Hearing Loss with Neural Networks versus Logistic Regression Modeling of Distortion Product Otoacoustic Emissions

We investigated whether modeling with artificial neural networks or logistic regression of distortion product otoacoustic emissions (DPOAE), across diverse frequencies, may achieve an accurate diagnosis of sensorineural hearing loss (SNHL) of cochlear origin. 256 ears (90 with SNHL and 166 with normal hearing) were evaluated with pure-tone audiometry, impedance audiometry, speech audiometry and DPOAE. Ears were split into training (n = 176) and validation (n = 80) sets. Input variables included gender, age, examination time, DPOAE intensity at f2 frequencies 593, 937, 1906, 3812 and 6031 Hz, and respective values corrected for noise levels. In the validation data set, an average network had an area under the receiver operating characteristic curve (AUC) of 0.86 (accuracy 84%). Logistic regressions including all these variables or those selected by backward elimination had AUC values of 0.91 and 0.92, respectively (accuracy 85% both). Eleven of 12 trained networks had better specificity than the backward elimination logistic regression, and the backward elimination logistic regression had a better sensitivity than 11 of the 12 networks. Both modeling approaches correctly identified all ears with sudden hearing loss, congenital hearing loss, head trauma, nuclear jaundice and ototoxicity, and 2–3 of 5 ears with acoustic trauma, but missed 1–3 of 3 ears with Ménière’s disease and 4–6 of 8 ears with abnormal pure-tone thresholds on audiometry which had no accompanying findings. For SNHL exceeding 45 dB HL on a pure-tone threshold, sensitivity was 83% (15/18) by neural networks and 84 or 94% (16/18 or 17/18) by logistic regression. Both neural-network-based analysis and logistic regression modeling of the DPOAE pattern across a range of frequencies offer promising approaches for the objective diagnosis of moderate and severe SNHL.

Permanent bilateral acoustic trauma due to air bag deployment in a young female adult.

Air bag safety systems have significantly reduced the number of occupant injuries from road traffic accidents (RTA). However air bag deployment is also associated with unavoidable risks. We report the acoustic trauma incurred by a young female driver who was a heavy smoker as a consequence of air-bag deployment in a low speed RTA and the sparing of her child seated in the rear.

Value of the facial nerve latency test in the prognosis of childhood Bell's palsy

In the present study we evaluated the facial nerve latency test (FNLT) as a prognostic tool in cases of childhood Bells palsy. Twenty-five children aged 4–14 years were studied. We divided our subjects into three groups according to duration of latency time (LT). Group A patients had an LT within the normal range, with average of 3.27 ms, group B a slightly prolonged LT averaging 5.7 ms, and group C a markedly prolonged LT averaging 10.5 ms. Analysis of the recovery index by group showed that group A patients experienced complete and quick recovery, while in group B 50% had complete but delayed recovery and 50% slightly impaired facial nerve function, and in group C 50% had slightly impaired function and 50% incomplete recovery. The more prolonged the LT, the worse the clinical results. The FNLT is thus a valuable prognostic tool in cases of Bells palsy in childhood.

Measuring of distortion product otoacoustic emissions using multiple tone pairs

In order to reduce the examination time of the Distortion Product Otoacoustic Emissions (DPOAE) test, we investigated the applicability of the multiple-tone pairs method by comparing the DPOAE evoked by 4- and 3-tone pairs (Sim) with the conventional 1-tone pair (Seq) in 116 ears of human adults with normal hearing (NH) and 86 ears with sensorineural hearing loss (SNHL). We found that: (1) DPOAE amplitudes in the NH group obtained with the Sim method were strongly correlated with those of Seq. (2) DPOAE amplitude and noise level mean values obtained with the Seq method were significantly higher than those obtained with Sim. (3) DPOAE amplitude was independent of the stimulation side (right vs. left ears). (4) Both methods can equally distinguish ears with NH and a low degree of SNHL from those with higher degrees of SNHL. (5) Examination time was shorter with the Sim method in both groups. In conclusion, the Sim method produces results similar to those of Seq. However, a significant difference between the two methods in relation to DPOAE amplitudes and noise levels was found. Because of this, normal data for both the DPOAE amplitude and noise level are required to improve the sensitivity of the Sim method. The mean examination time in both groups (NH and SNHL) was shorter with the Sim method. Therefore, the multiple-tone pairs stimulation method seems to be more suitable when it is used as an auditory screening test.