[Cogan's disease. Report of two cases]. / Das Cogan-Syndrom. Zwei Fallberichte.

BACKGROUND: Cogan's disease is a diagnostic challenge. The typical form of this chronic inflammatory disease is characterized by non-syphilitic interstitial keratitis and acute audiovestibular impairment. CASE REPORTS: The first patient had "typical" symptoms of Cogan's syndrome with recurrent keratoconjunctivitis, severe hearing loss and vertigo. Systemic cardiovascular manifestations occurred in the further course of the disease with multiple vascular stenoses of the carotids and vessels of the lower limbs. The second patient had "atypical" symptoms of Cogan's disease with progressive bilateral hearing loss and vestibular deficits, unilateral conjunctivitis and orbital swelling and unspecific symptoms, such as fever and headaches. Therapy with systemic and local corticoids was successful in both cases concerning ocular and systemic symptoms. The first patient had received additional therapy with methotrexate, but had already become deaf before therapy had started. Cochlear implantation was successfully carried out. In the second patient hearing loss was stationary under therapy and the patient could be provided with hearing aids. CONCLUSION: Because of obligatory cochleovestibular symptoms the otorhinolaryngologist has a key position in diagnosis and therapy of this rare entity. Early diagnosis is a crucial prognostic factor concerning preservation or restitution of hearing function.

[Large endolymphatic duct and sac syndrome : part 2: clinical manifestations]. / Der erweiterte Ductus und Saccus endolymphaticus : Teil 2: Klinische Manifestationen.

OBJECTIVE: The aim of this study was to clarify the clinical course of large endolymphatic duct and sac syndrome (LEDS). Although LEDS is the most common form of radiologically detectable inner ear malformation associated with sensorineural hearing loss (SNHL), it is relatively unknown in the German-speaking countries. We hoped to derive useful pointers for clinical care. METHODS: We evaluated the clinical audiometric records and imaging findings of 169 patients with clinically suspected inner ear malformation seen between 1994 and 2003. Following identification of all LEDS cases, we serially investigated the anamnestic and functional data with regard to severity and course. RESULTS: Among 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Clinically, most cases were of prelingual or perilingual onset and displayed steady or fluctuatingly progressive severe SNHL with emphasis on the high frequencies or deafness. Episodes of sudden hearing loss were relatively frequent. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL. CONCLUSION: Progression of SNHL and episodes of sudden hearing loss were seen mainly in late childhood or adolescence. This should be taken into account when informing and advising patients and planning therapy.

[Large endolymphatic duct and sac syndrome (LEDS) : part I: analysis of imaging findings]. / Der erweiterte Ductus und Saccus endolymphaticus : Teil 1: Analyse bildgebender Befunde.

OBJECTIVE: Large endolymphatic duct and sac (LEDS) syndrome is the most common radiologically detectable form of inner ear malformation (IEM) associated with sensorineural hearing loss (SNHL). Nevertheless, it is relatively unknown in the German-speaking countries. METHODS: Among all patients referred from ear, nose, and throat specialists to the radiology department with clinically suspected IEM between 1994 and 2003, we identified the cases of LEDS. The retrospective study included clinical records, high-resolution computed tomography, and magnetic resonance imaging. RESULTS: Of 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Ten patients (6%; 15 ears) had isolated LEDS, while seven patients showed additional IEM (4%; 13 ears). The most frequent of these was dysplastic vestibule (13/13 ears), followed by Mondini deformity (10/13 ears) and dilated semicircular canals (7/13 ears). Three of 13 ears revealed severe IEM of the cochlea, vestibule, and semicircular canals. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL. CONCLUSION: LEDS may be an underestimated cause of SNHL. Imaging is necessary for a confident diagnosis.

[The role of MRI in suspected inner ear malformations]. / Stellenwert der MRT bei Verdacht auf Innenohrmissbildung.

PURPOSE: This is a prospective analysis of the value of MRI in suspected inner ear malformations. MATERIALS AND METHODS: In 50 patients (43 children and young adults, 7 adults) with suspected inner ear malformation MRI (1.5 T) was performed. In addition, 42 of these patients underwent CT. For the analysis of the inner ear structures, the constructive interference in steady state (CISS) sequence with 0.7 mm slice thickness was used. Functional tests revealed a sensorineural hearing loss or deafness in 82 temporal bones (TB) and a combined hearing loss in 4 TB. The hearing loss was unilateral in 14 patients. MRI and CT findings were compared. RESULTS: Imaging findings were normal in 58 TB. The pathological findings included inner ear malformations (35 TB), inflammatory changes (4 TB), partial obliteration of labyrinth (2 TB) and congenital aural atresia (1 TB). An isolated absence of the cochlear nerve (1 TB) could only be found by MRI. In the remaining cases, an inner ear malformation was diagnosed by MRI and CT with the same confidence but MRI was superior in displaying the fine details. CONCLUSIONS: MRI will become the method of choice in the diagnosis of inner ear malformations.

[Hearing screening of newborn infants at risk for perinatal hearing damage]. / Hörscreening Neugeborener mit einem Risiko für perinatale Hörschäden.

630 neonates with risk factors of perinatal hearing impairment were screened of hearing loss by means of registration of transient otoacoustic emissions before discharge from the newborn nursery. Neonates were screened additionally by means of brainstem evoked response audiometry, if they had bilateral negative emissions. 810 healthy neonates were screened as control group. The investigations were carried out in incubator after the feeding of neonates. The prevalence of a bilateral negative cochlear response was 5.2% in the risk babies and 1.7% in healthy neonates. Neonates are high risk patients for hearing loss if they show craniofacial anomalies including alcohol embryofetopathy, connatal infections, or very low birth weight babies with additional risk factors. The pedaudiological control investigations of the babies with a negative bilateral cochlear response delivered in the risk group 15 cases (2.4%) with an important hearing impairment and in the healthy neonates 2 cases (0.25%) respectively. Prevalence and importance of perinatal hearing impairment explains the necessity of detection in the neonatal period.

[Results of neonatal hearing screening with transitory evoked otoacoustic emissions]. / Ergebnisse eines Neugeborenen-Hörscreenings mittels transitorisch evozierter otoakustischer Emissionen.

From 1. 10. 1992-31. 12. 1993 a hearing screening was performed in 994 preterm and term babies (765 healthy neonates and 229 neonates with risk factors for hearing loss) by means of transient evoked otoacoustic emissions (TEOAE). There was a bilateral absent of emissions in 4.3% of the healthy neonates and 11.8% of the risk babies. 8 from 32 healthy neonates and 10 from 22 risk babies also had a negative test result in the control investigation. 16 of these children showed in 7 cases a severe hearing impairment in expanded pedaudiological investigations. The registration of TEOAE is a recommendable method for a hearing screening in neonates.

[Parosmias--definition and clinical picture]. / Parosmien--Begriffsbestimmung und klinisches Bild.

The paper proposes the definition and technical terms with greater precision for the description of qualitative smelling disturbances. Thereafter, parosmias are relevant only for the ENT specialist. Their further classification seems to be unnecessary or even false. These parosmias are always described to be troublesome for the patient since he feels the abnormality of the olfactory sensation. Thus, a clear distinction against phantosmias (olfactory hallucinations) and pseudoosmias (olfactory illusions) is given. The cause of parosmias is frequently speculative. Most frequently there occur traumas and influenza. Their treatment is equally the same as in postinfluenza hyposmias. Additionally, the dripping of 4% novocaine or 10% cocaine on the olfactory mucosa is performed. The parosmia is defined to be an independent entity of disorder, not only accompanying hyposmia. The parosmia may occur separately, too, and it does not necessarily recede together with hyposmia and anosmia.