Cholesterol granulomas of the petrous apex; endonasal endoscopic approach.

OBJECTIVE: Cholesterol granulomas are benign lesions that sometimes occur on the petrous apex (PA). We report our experience using an endoscopic endonasal approach to remove PA cholesterol granulomas. MATERIAL AND METHODS: A retrospective patient chart analysis was conducted at a tertiary care university hospital. RESULTS: Four patients (3 females, 1 male) were included in this study. Patients' ages ranged from 27 to 78 years. Computed tomography (CT) and magnetic resonance imaging (MRI) for diagnosis and computer-assisted navigation were performed. The most common symptom was abducens nerve palsy. The largest granuloma measured 5 x 2 cm and was located on the left side. An endoscopic endonasal approach was chosen and navigation was applied (3/4 patients) to identify the optimal area for opening the granuloma. No complications occurred, and patients were free from recurrence during the follow-up period. CONCLUSION: The endoscopic endonasal approach to PA cholesterol granulomas is feasible and safe. Intra-operative navigation is recommended to identify the position of the internal carotid artery and determine the safest area for opening the granuloma without damaging the artery. Another advantage of this approach is an easier follow-up through diagnostic nasal endoscopy.

Suction-generated noise in an anatomic silicon ear model.

The objectives of this study were to evaluate noise levels generated during micro-suction aural toilet using an anatomic silicon ear model. It is an experimental study. In an anatomic ear model made of silicone, the eardrum was replaced by a 1-cm diameter microphone of a calibrated sound-level measuring device. Ear wax was removed using the sucker of a standard ENT treatment unit (Atmos Servant 5(®)). Mean and peak sound levels during the suction procedure were recorded with suckers of various diameters (Fergusson-Frazier 2.7-4 mm as well as Rosen 1.4-2.5 mm). Average noise levels during normal suction in a distance of 1 cm in front of the eardrum ranged between 97 and 103.5 dB(A) (broadband noise). Peak noise levels reached 118 dB(A). During partial obstruction of the sucker by cerumen or dermal flakes, peak noise levels reached 146 dB(A). Peak noise levels observed during the so-called clarinet phenomena were independent of the diameter or type of suckers used. Although micro-suction aural toilet is regarded as an established, widespread and usually safe method to clean the external auditory canal, some caution seems advisable. The performance of long-lasting suction periods straight in front of the eardrum without sound-protecting earwax between sucker and eardrum should be avoided. In particular, when clarinet phenomena are occurring (as described above), the suction procedure should be aborted immediately. In the presence of dermal flakes blocking the auditory canal, cleaning with micro-forceps or other non-suctioning instruments might represent a reasonable alternative.

Neuroendocrine adenoma of the middle ear (NAME) mimicking as chronic otitis media with an episode of facial nerve palsy.

OBJECTIVES: To increase awareness of neuroendocrine adenomas of the middle ear (NAME), rare lesions often mistaken for other entities or chronic otitis media. Histogenesis remains controversial, although the consensus tends toward a pluripotent stem cell of the middle ear mucosa as the origin of the lesion. The tumour is characterised by dual differentiation with exocrine and endocrine components. The most common symptoms are conductive hearing loss, tinnitus and vertigo. The treatment of choice is complete surgical removal of the tumour with no adjuvant radiotherapy being required. CASE REPORT: We report the case of a 23-year-old man presenting with chronic otitis media, conductive hearing loss, vertigo and tinnitus who, some years previously, had suffered from an episode of facial nerve palsy. Conservative therapy failed and so surgery was performed. Tumour-like masses were encountered and histological and immunohistochemical examination revealed a neuroendocrine adenoma of the middle ear. CONCLUSION: This rare entity should be considered as differential diagnosis when treating chronic inflammatory disease not responding to conservative therapy or dealing with unclear expansive processes of the middle ear. MRI scans should be performed since CT scans are inconclusive.

Effect on hearing of ganciclovir therapy for asymptomatic congenital cytomegalovirus infection: four to 10 year follow up.

BACKGROUND: Congenital cytomegalovirus infection is the leading identified nongenetic cause of congenital sensorineural hearing loss. Most of the infections are asymptomatic but may be detected from umbilical cord vein and/or newborn serum positivity for human cytomegalovirus immunoglobulin M, and from urine positivity (on polymerase chain reaction) for human cytomegalovirus deoxyribonucleic acid in the newborn period. Children infected by cytomegalovirus may later develop sensorineural hearing loss. In symptomatically infected infants, ganciclovir therapy administered in the neonatal period prevents hearing deterioration. However, preventative therapy of asymptomatic congenital cytomegalovirus disease with ganciclovir is controversial, as side effects such as severe neutropenia may occur during treatment. METHODS: The study population consisted of 23 asymptomatic children with congenital cytomegalovirus infection. Twelve children were treated just after diagnosis of cytomegalovirus infection in the newborn period, with ganciclovir 10 mg/kg bodyweight for 21 days. The other 11 children were observed without therapy. Over a four to 10 year follow-up period, we evaluated all the children's hearing status using pure tone audiometry. RESULTS: All 23 children had normal sensorineural hearing at one year follow up. Five of the 23 children (21.7 per cent) were lost to follow up over the four to 11 year follow-up period. Of the remaining 18 children, sensorineural hearing loss occurred in two (11.1 per cent). Neither child had been treated with ganciclovir in the newborn period. An eight-year-old boy showed bilateral high frequency loss and a 10-year-old girl showed severe unilateral sensorineural hearing loss. In the ganciclovir-treated group (nine children), none showed sensorineural hearing loss. During ganciclovir therapy, moderate neutropenia occurred as a side effect in two out of 12 (16.6 per cent) treated children. Speech and general development were normal in all children. CONCLUSION: Asymptomatic congenital cytomegalovirus infection is likely to be a leading cause of sensorineural hearing loss in young children. Intravenous ganciclovir therapy seems to offer a medical option to prevent subsequent sensorineural hearing loss. Further studies including a greater number of children are needed. Cytomegalovirus screening models are mandatory if medical therapy is to be implemented in time.

Hyperbaric oxygen in tinnitus: influence of psychological factors on treatment results?

INTRODUCTION: The standard treatment of subjective tinnitus hardly reaches the level of placebo controls. Though the effectiveness of hyperbaric oxygenation (HBO) for subjective tinnitus has never been objectified, it is still advocated by some institutions. We analyzed the effectiveness of hyperbaric oxygen treatment in the context of accompanying factors. PATIENTS AND METHODS: We randomized 360 patients suffering from tinnitus into 2 HBO treatment protocols (group A: 2.2 bar for 60 min bottom time and group B: 2.5 bar for 60 min bottom time once a day for 15 days). All patients were asked to fill in a questionnaire (social and medical history, tinnitus characteristics, pre-HBO duration of tinnitus, prior therapy, pretreatment expectation, accompanying symptoms). A subjective assessment of the therapeutic effect was obtained. RESULTS: Twelve patients (3.3%) experienced complete remission of tinnitus, in 122 (33.9) the intensity lessened, and 44 (12.2%) had a subjectively agreeable change of noise characteristics. No change was found in 157 cases (43.6%) and 25 (6.9%) experienced deterioration. There was no statistically significant difference between groups A and B (p > 0.05). Out of 68 patients with a positive expectation of HBO effects, 60.3% stated that the tinnitus had improved whereas only 47.2 and 19%, respectively, out of patients who underwent therapy with an indifferent (n = 271) or negative expectation (n = 21) reported an improvement. The influence of subjective expectation on the outcome was statistically significant (p < 0.05). CONCLUSION: The therapeutic effects of HBO on subjective tinnitus may be substantially influenced by psychological mechanisms.

Technical and clinical success of infrarenal endovascular abdominal aortic aneurysm repair: A 10-year single-center experience.

OBJECTIVE: The aim of our retrospective study was to review our single-center experience with aortic abdominal aneurysm (AAA) repair retrospectively. MATERIAL AND METHODS: From 1995 to 2005, 70 consecutive patients affected by AAA were treated by endovascular stent-graft repair. Mean follow-up was 23.9 months. Follow-up investigations were performed at 6 and 12 months and yearly thereafter. Five different stent-graft designs were compared to each other. Primary technical success (PTS), assisted primary technical success (APTS), primary clinical success (PCS) and secondary clinical success (SCS) were evaluated. RESULTS: All over PTS was achieved in 94.3%, APTS in 97.1%, PCS in 61.4%, APCS in 64.3% and SCS in 70%. There were 3 type I endoleaks, 25 type II endoleaks, 4 type III endoleaks, 8 limb problems, 5 conversions to open surgery, 10 aneurysm sac expansions and 14 device migrations. Patients with newer generation devices showed better results than patients with first generation prosthesis. In addition results were better for grafts with suprarenal fixation (versus infrarenal fixation) and grafts with barbs and hooks (versus grafts without barbs and hooks). Patients with bad anatomic preconditions showed a higher complication rate. CONCLUSION: Contrary to first generation products, new stent-graft designs show acceptable technical and clinical results in endovascular AAA aneurysm repair. However, this therapy still should be reserved only for patients with significant comorbities and suitable anatomic conditions.

Hyperbaric oxygen (HBO2) in tinnitus: influence of psychological factors on treatment results?

UNLABELLED: HBO2 for subjective tinnitus has never been objectified, yet it is still advocated by many institutions. We analyzed the therapeutic effect of HBO2 treatment in the context of accompanying factors, especially focusing on the patients' attitude towards HBO2 prior to therapy. METHODS: 360 patients suffering from tinnitus were investigated in this study. They were randomized into 2 hyperbaric treatment protocols, one at 2.2, the other at 2.5 bar, 60 min bottom time each. All patients were asked to fill in a questionnaire (social and medical history, tinnitus characteristics, pre-HBO2 duration oftinnitus, prior therapy, pre-treatment expectation, accompanying symptoms) immediately before and one month after HBO2 treatment. The subjective impact of tinnitus on daily life was assessed using a score from 1 to 10 prior to HBO2 and one month after the treatment. RESULTS: 12 patients (3.3%) experienced complete remission of tinnitus, in 122 (33.9%) the intensity lessened, and 44 (12.2%) had a subjectively agreeable change of noise characteristics. 157 (43.6%) stated no change and 25 (6.9%) experienced deterioration. There was no statistically significant difference between the two hyperbaric protocols (p > 0.05). Out of 68 patients with positive expectation towards HBO2 therapy 60.3% stated that the tinnitus had improved whereas out of the patients who underwent therapy with indifferent (N = 271) or negative expectations (N = 21) only 47.2% and 19%, respectively, reported an improvement. The influence of both positive and negative anticipation on the outcome was statistically significant (p < 0.05). CONCLUSION: The therapeutic effects of HBO2 on subjective tinnitus might be greatly due to psychological mechanisms triggered by the attitude of the patient towards HBO2 therapy prior to the treatment.

Combined endoscopic surgery and radiosurgery as treatment modality for olfactory neuroblastoma (esthesioneuroblastoma).

OBJECT: Treatment of esthesioneuroblastoma (olfactory neuroblastoma) has been considerably improved by microsurgical techniques. Nevertheless, these rare tumours of the frontal skull base are still associated with high rates of tumour recurrence and mortality, thus remaining a challenge even for experienced surgeons. A novel therapeutic approach that combines endoscopic sinus surgery and Gamma Knife radiosurgery is presented here. Taking into account the rarity of the disease the present study comprises a relatively large series of patients treated in a similar manner. METHODS: 14 patients (8 males, 6 females) aged 27-75 years (median 38) were treated between May 1993 and December 2003. This series comprises 12 newly diagnosed esthesioneuroblastomas. Two more patients had already previously undergone surgery (24/39 months earlier). Paranasal and nasal endoscopic sinus surgery was performed. Marginal irradiation doses ranging from 15-34 Gy were given to the residual tumours by means of radiosurgery (Gamma Knife) involving 1-7 isocentres within 3 months after surgery. Median follow-up is 58 months (range 13-128). RESULTS: There was no mortality. In all patients tumour control was achieved within the treated area. 4 patients underwent a second radiosurgical procedure 6-79 months (median 34 months) after initial radiosurgery. One patients had to undergo an additional craniotomy because of extensive neoplastic infiltration, 1 developed postoperative liquorrhea, 1 case was complicated by bilateral frontal sinusitis. All patients complained of nasal discharge and crusts. Karnovsky Index--preoperatively ranging from 80%-100%--remained stable in 12 patients, an improvement was observed in 2 cases. CONCLUSIONS: Based on the favourable results recorded so far, the combination of endoscopic sinus surgery and radiosurgery can be considered a promising treatment option for esthesioneuroblastoma that merits further consideration.

Cranial nerve preservation after radiosurgery of vestibular schwannomas.

Radiosurgery is a management approach used to treat patients with vestibular schwannomas. The goals are long-term tumour growth control, maintenance of cranial nerve function and prevention of new deficiencies. We sought to determine long-term outcomes measuring the potential benefits against the neurological risks of primary radiosurgery. Gamma Knife radiosurgery was applied as a treatment modality for 289 patients with vestibular schwannomas from April 1992 to April 2002. The long-term results of 100 patients who underwent radiosurgery were evaluated. 60 patients received a primary treatment, 40 other cases presented with previously performed subtotal microsurgical resection or recurrence of disease (12-96 months, median 39). The median treatment volume was 3.4 ccm and the median dose to the tumour margin was 13 Gy. The median patient follow-up time was 76 months (range 60-120 months). Four tumours progressed after primary radiosurgery. Tumour control rate was 96%. Useful hearing (Gardner-Robertson I/II) was preserved in 16 patients (55%). Clinical neurological improvement occurred in 50%. Adverse effects comprised neurological symptoms (incomplete facial palsy) (House-Brackman II/III) in six cases (four recovered completely), mild transient trigeminal neuropathy in five cases, and morphological changes displaying rapid enlargement of preexisting macrocysts in two patients and tumour growth in two other patients. Microsurgical resection was performed in four cases (4%) and two patients underwent a shunting procedure because of hydrocephalus formation (2%). In patients who had undergone previous microsurgery, no new cranial nerve deficit was observed. Radiosurgery is an effective method for growth control of vestibular schwannomas and is associated with both a low mortality rate and a good quality of life. Accordingly, for the preservation of cranial nerve function radiosurgery is a useful method for the management of properly selected patients and is comparable to microsurgery.

Long term results of radiosurgery for vestibular schwannomas.

UNLABELLED: Radiosurgery is either a primary or an adjunct management approach used to treat patients with vestibular schwannomas. The goals are long-term tumour growth control, maintenance of cranial nerve function and prevention of new neurologic deficiencies. We sought to determine long-term outcomes measuring the potential benefits against the neurological risks of radiosurgery. METHODS: 278 patients with vestibular schwannomas underwent Gamma Knife radiosurgery as a treatment modality for from April 1992 to November 2001. The long-term results of 60 patients were evaluated who received radiosurgery as primary treatment. 12 cases presented with previously performed subtotal microsurgical resection or recurrence of disease (12-96 months, median 39). The median treatment volume was 3.8 ccm and the median dose to the tumour margin was 12 Gy. The median patient follow-up time was 88 months (range 72-114 months). RESULTS: Four tumours progressed after primary radiosurgery. Tumour control rate was 93%. Useful hearing (Gardner-Robertson I/II) was preserved in 16 patients (55%). Clinical neurological improvement occurred in 36 patients (60%). Adverse effects comprised neurological symptoms (incomplete facial palsy) (House-Brackman II/III) in five cases (three recovered completely), mild trigeminal neuropathy in three cases, and morphological changes displaying rapid enlargement of preexisting macrocysts in two patients and tumour growth in two other ones. Microsurgical resection was performed in three cases (5%) and one patient underwent a shunting procedure because of hydrocephalus formation. In patients who had undergone previous microsurgery, neither new cranial nerve deficit nor any tumour growth was observed. CONCLUSIONS: Radiosurgery performed with current techniques proved to be an effective method for growth control of vestibular schwannomas with both a low mortality rate and a good quality of life. An increasing percentage of patients will undergo radiosurgery as accessibility to this alternative increases, and more data regarding long-term follow-up are available. It is a post-operative complementary treatment for partially removed tumours. Accordingly, radiosurgery is a useful method for the management of properly selected patients.

Radiosurgery of residual and recurrent vestibular schwannomas.

Radiosurgery is either a primary or an adjunctive management approach used to treat patients with vestibular schwannomas. We sought to determine outcomes measuring the potential benefits against the neurological risks in patients who underwent radiosurgery after previous microsurgical subtotal resection or recurrence of the tumour after total resection. Gamma Knife radiosurgery was applied as an adjunctive treatment modality for 86 patients with vestibular schwannomas from April 1992 to August 2001. We evaluated the results of 50 patients who had a follow-up of at least 3.5 years (median 75 months, range 42-114 months). In 16 patients a recurrence of disease was observed after previous total resection. The median treatment volume was 3.4 ccm with a median dose to the tumour margin of 13 Gy. Tumour control rate was 96%. Two tumours progressed after adjunctive radiosurgery. Useful hearing (Gardner-Robertson II) (4 patients (8%)) and residual hearing (Gardner-Roberson III) (10 patients (20%)) remained unchanged in all patients, who presented with it before radiosurgery, respectively. Clinical neurological improvement was observed in 24 patients (46%). Adverse effects comprised transient neurological symptoms and signs (incomplete facial palsy, House-Brackman II/III) in five cases (recovered completely), mild trigeminal neuropathy in four cases, and morphological changes displaying rapid enlargement of a pre-existing macrocyst in one patient and tumour growth in another one. No permanent new cranial nerve deficit was observed. Radiosurgery appears to be an effective adjunctive method for growth control of vestibular schwannomas and is associated with both a low mortality rate and a good quality of life. Accordingly, radiosurgery is a rewarding therapeutic approach for the preservation of cranial nerve function in the management of patients with vestibular schwannoma in whom prior microsurgical resection failed.

[Configuration of ethmoid roof in children 0-14 years of age]. / Siebbeindachkonfiguration bei Kindern im Alter von 0 bis 14 Jahren.

BACKGROUND: Knowledge of the unique anatomy of the nose, paranasal sinuses and skull base, particular concerning dangerously low positioned or deep lying cribriform plates is most important, as functional endoscopic sinus surgery has become an increasingly popular procedure for the management of pediatric sinus disease. OBJECTIVES AND METHODS: In addition to Keros who studied the ethmoidal roof and cribriform plate in 450 adult specimen and divided them into 3 groups, retrospective analysis in 272 patients between 0 and 14 years was performed by means of coronal CT scans of the paranasal sinuses with a slice thickness of 2 mm. Measurements were carried out in the frontal, middle and dorsal section of the ethmoid. RESULTS: The depth and width of the fossa olfactoria were significantly less in patients aged 0 - 12 months than in other age groups (p < 0.001). Among the other age groups, beginning at 2 years no differences were found: 14.2 % presented with type I according to Keros, 70.6 % with Keros II and 15.2 % with Keros III. The prevalence of asymmetric position of the ethmoidal roof was 15 % (41 patients). The height of the ethmoidal sinuses consistently increased over the years from 5 - 7 mm to 15 - 20 mm. CONCLUSION: The current data may serve as a reference for evaluation of normal and abnormal development of the roof of the ethmoid and may be of great value in diagnostic and therapeutic management of pediatric sinus disease. Our data obviously show that the classification into the 3 types of positions of the ethmoid roof and cribriform plate according to Keros is possible in children from the second year of life.

Olfactory neuroblastoma (esthesioneuroblastoma): report of six cases treated by a novel combination of endoscopic surgery and radiosurgery.

Microsurgical techniques have considerably improved the results of surgical treatment for esthesioneuroblastoma (olfactory neuroblastoma). Nevertheless, these rare tumours of the frontal skull base are still associated with high rates of tumour recurrence and mortality, thus remaining a challenge even for experienced surgeons. A novel therapeutic approach that combines endoscopic sinus surgery and radiosurgery (gamma knife) is presented here. Six patients (3 males, 3 females) aged between 27 and 75 years (median 38 years) were treated between August 1993 and July 1999. Following paranasal and nasal endoscopic sinus surgery, marginal irradiation doses ranging from 16 to 34 Gy were applied radiosurgically involving up to 7 isocentres. At present, the median follow-up period is 57 months (range: 9 - 79 months). Without mortality, tumour control was achieved in all patients. One patient, who had to undergo additional craniotomy because of extensive neoplastic infiltration, developed postoperative liquorrhea. In another case the clinical course was complicated by a bilateral frontal sinusitis. All patients complained of nasal discharge and crusts. However, a preoperative Karnovsky Index ranging from 80 to 100 % remained stable in four patients whereas an improvement was observed in two patients. Based on the favourable results observed so far, the combination of endoscopic sinus surgery and radiosurgery can be considered as promising new option for the treatment of esthesioneuroblastoma that merits further investigation.

[Mondini dysplasia without functional impairment in the framework of a CHARGE association]. / Mondini-Dysplasie ohne funktionelle Beeinträchtigung im Rahmen einer CHARGE-Assoziation.

UNLABELLED: A male child with fully developed CHARGE association was referred to our hospital several times because of choanal atresia and suspected hearing loss in his first two years of life. As far as they are a main symptom malformations of the ear appear frequently in CHARGE association. This report describes that an obvious anatomic dysplasia of the inner ear can have normal function, which is shown by objective measurement. METHODS AND RESULTS: Computed tomography of the petrous bone showed bilateral Mondini malformation of the cochlea combined with bulky deformation of middle ear ossicles. By the age of one year brainstem evoked response audiometry (BERA) was performed. The result demonstrated a hearing loss of 60 dB on the right ear, but no measurable hearing loss on the left ear. CONCLUSION: This case report proves that a Mondini-type malformation of the cochlea does not need to be combined with hearing loss.

[Radiosurgery of recurrent acoustic neurinoma or: the elegant solution of a surgical problem?]. / Die Radiochirurgie bei Akustikusneurinom-Rezidiv oder: Die elegante Lösung eines chirurgischen Problems?

BACKGROUND: Stereotactic radiosurgery has proved to be an effective alternative to microsurgical resection in treatment of acoustic neuroma. Still, microsurgery is considered by many to be the therapy of choice. In case of recurrence microsurgical resection is much more difficult because of scarring and has a higher risk of complications. Therefore in cases of recurrence the role of radiosurgery needed to be evaluated. PATIENTS AND METHODS: From April 1992 to July 1997 135 patients suffering from acoustic neuroma were treated at the Neurosurgical Department of the University Medical School of Graz by means of the gamma-Knife. 12 patients had recurrence after a single or several microsurgical resections. The age distribution was between 38 and 71 years with a mean of 57 years. The diameter of the tumors varied between 10.5 and 31.2 mm. RESULTS: In all 12 cases the tumors could be inactivated biologically in a mean follow-up period of 58.8 months by means of stereotactic radiosurgery. Tumor shrinkage was achieved in 3 cases (25%), central necrotic areas were observed in 8 cases (67%). No additional cranial nerve palsies occurred. CONCLUSIONS: Stereotactic radiosurgery has proven to be a safe and effective treatment option instead of repeated microsurgery. Stereotactic radiosurgery should be considered as the therapy of choice in cases of recurrent acoustic neuromas.

[On the fate of lost gallstones]. / Zum Schicksal der verlorenen Gallensteine.

The spilling of stones during laparoscopic cholecystectomy occurs in 10% of the operations. Late complications associated with this type of surgical procedure are abscesses and fistulas in the abdominal cavity and abdominal wall. The time elapsing between laparoscopy and clinical manifestation may be as long as 12 months. We present a case of a patient with localised abdominal pain in the upper right abdomen two months after laparoscopic cholecystectomy. Sonography and CT revealed a fat necrosis posterior of the rectus muscle. Examination of the histological specimen confirmed a fat necrosis caused by two small pigmented stones, spilled during laparoscopic cholecystectomy.

Bilateral sensorineural hearing disorders in children: etiology of deafness and evaluation of hearing tests.

OBJECTIVE: The purpose of this study was to determine the etiology of bilateral sensorineural hearing disorders in children and to evaluate the performed hearing tests by comparison of the results of the objective and subjective tests. METHODS: The medical history and the hearing tests (behavioral observation audiometry, acoustic evoked potentials and pure tone audiometry) of 106 bilaterally hearing impaired children were analyzed in a retrospective follow-up study. RESULTS: The total group included 52 males and 54 females. The ages at first diagnosis ranged from 4 months to 11 years with a mean age of 42 months and a median of 33 months. The degree of hearing loss for the better hearing ear was mild in one child, moderate in 28 children, severe in 29 children, profound in 32 children and total in 16 children. The delay between the first examination and diagnosis ranged from 0 to 597 days with a mean of 83 days and a median of 28 days. In 47 children (44%) no cause of hearing impairment could be determined. Nineteen children (18%) had a history of familial hearing loss, 40 (38%) suffered from acquired hearing loss (seven children had prenatal causes, 21 perinatal and 12 postnatal). A comparison between behavioral observation audiometry and brainstem evoked response audiometry revealed a statistically good agreement. Twenty-nine children (32%) showed progressive hearing loss, which was defined as a threshold shift of +10 dB or more in the pure tone average in at least one ear. CONCLUSIONS: In a significant number of children with early hearing impairments the etiology still remains uncertain. Further research in the field of genetic disorders will diminish this number. Evaluation of hearing tests showed that behavioral observation audiometry still is an excellent tool in the hands of an experienced examiner. The age at identification of hearing disorders in industrialized countries still is unacceptably high. To obtain ideal care of hearing impaired children, universal neonatal hearing screening programs are mandatory.