Hearing loss in Sjögren's syndrome patients. A comparative study.

OBJECTIVE: In an attempt to investigate the presence of hearing loss in primary Sjögren's syndrome (SS) patients and to determine the factors that might be involved in its pathogenesis, we prospectively evaluated 45 female SS patients with a mean age of 56.8 +/- 9.23 years and a mean disease duration of 8.32 +/- 5.39 years. METHODS: Forty patients underwent a complete ear-nose-throat physical examination and audiological evaluation with: (a) pure tone audiometry thresholds at octave frequencies of 250 to 8000 Hz; (b) impedance audiometry (tympanogram, static compliance, acoustic reflexes, reflex decay; and (c) speech audiometry and auditory brainstem response where indicated. In addition, glandular and extraglandular manifestations of the disease and drug therapy were recorded. Finally, all patients were tested for the presence of autoantibodies, including: rheumatoid factor, antinuclear antibodies, antibodies to Ro(SSA), La(SSB) nuclear antigens, anticardiolipin antibodies and antineutrophil cytoplasmatic antibodies. The results were compared with those of 40 healthy, age-matched women. RESULTS: We found sensorineural hearing loss (SNHL) in 9 patients (22.5%): 4 patients bilaterally, 4 patients in the left ear only and one in the right ear only. In all cases the site of the ear damage was cochlear. A correlation between SNHL and the duration of the disease was found, while there was no correlation with age, systemic manifestations of the disease or the presence of autoantibodies. In addition, no correlation was found between SNHL and drug therapy. CONCLUSION: Approximately one-fourth of our SS patients presented SNHL of cochlear origin affecting mainly the high frequencies. This prevalence was lower than that reported by other investigators. SNHL was associated only with disease duration. Further investigation is needed to attain a better understanding of the mechanism of inner ear involvement in SS patients.

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.

Perceptive bilateral deafness following clinical tetanus to a neonate.

A rare case of bilateral perceptive deafness following clinical tetanus is reported. The patient was a 15-day-old neonate. The clinical tetanus was treated with tetanus antitoxin (human gamma globulins) and penicillin. The exact mechanism of auditory nerve damage associated clinical tetanus and treatment with human tetanus antitoxin and penicillin is still not clearly known. We believe that the damage of the hearing organ can be attributed to the tetanus toxin and not to the human gamma globulins (tetanus antitoxin).

Acquired nasopharyngeal obstruction and "Metsovo lung".

Acquired nasopharyngeal obstruction is a rare lesion today. Formerly it was the result of infections, but today it is more commonly seen as an unusual complication of surgical trauma. This article reports the first case, as we know it from the international literature, of nasopharyngeal obstruction which cannot be attributed to the already known causes and which occurred in a woman with "Metsovo lung" (i.e., occupational exposure to the asbestos-containing mineral tremolite). The ascertainment in the future of other cases like this will confirm the correlation between nasopharyngeal stenosis and Metsovo lung for which we have not any doubts.

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).

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 Bell's 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.

[Norrie-Wardburg syndrome]. / Norrie-Warburg-Syndrom.

We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

[Aberrant heterotopic salivary gland in a lateral neck cyst]. / Aberrierende heterotope Speicheldrüse in lateraler Halszyste.

An extra aberrant salivary gland located in a branchial cyst is presented. The branchial cysts are usually the result of a transformation disturbance of the branchial clefts and arches, or are cystic changes of the lymph nodes. The above finding can possibly be interpreted if we suppose that an aberrant salivary gland located in a lymph node can develop a cyst. The theory that branchial cysts originate from lymph nodes is encouraged by our case. This theory can also support the existence of unusual tumours in the neck, e.g. adenolymphomas. The content of these cysts can be easily approximately estimated via computed tomography.

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 Bell's 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 Bell's palsy in childhood.