[The "Muenster classification" of high frequency hearing loss following cisplatin chemotherapy]. / Die "Münsteraner Klassifikation": Eine neue Einteilung der Hochtonschwerhörigkeit nach Cisplatingabe.

BACKGROUND: Slight high frequency hearing loss following cisplatin chemotherapy can be proof of an ototoxic effect even when hearing ability is not yet clinically affected. To answer scientific questions, such as the relationship between cisplatin ototoxicity and drug regime or individual tolerance, early detection of ototoxicity and a classification relating to intensity and the affected frequencies are required. A search for relevant literature resulted the WHO-classification (1991) describing clinically relevant hearing loss and two high frequency hearing loss classifications published by Khan et al. (1982) and Brock et al. (1991). Their application is compared to a new, proprietary classification. PATIENTS AND METHODS: 55 patients (32 boys, 23 girls) undergoing cisplatin chemotherapy at Muenster University Hospital from 1999 to 2004 underwent audiometric tests in our department. From this data we developed a grading system, that was based on the WHO classification, but paid special attention to early ototoxic effects, to intensity of hearing loss and to the frequencies affected: Grade 0 (normal hearing) includes hearing loss of not more than 10 dB in all frequencies. Grade 1 (beginning hearing loss) encompasses > 10 dB up to 20 dB in at least one frequency or tinnitus. Grade 2 (moderate impairment) describes hearing loss > or = 4 kHz and differentiates 2a (> 20 to 40 dB), 2b (> 40 to 60 dB) and 2c (> 60 dB). Hearing loss < 4 kHz > 20 dB in grade 3 (severe impairment, hearing aids needed) is further classified according to grade 2 in a, b and c. Grade 4 (loss of function) finally describes average hearing loss < 4 kHz of at least 80 dB. This classification is compared to the two high frequency hearing loss classifications (Khan et al. and Brock et al.). RESULTS: The Muenster classification, compared to Khan et al. and Brock et al., demonstrated the best results in the early detection of hearing loss: All children with hearing loss of at least 20 dB after therapy had already shown pathological audiograms during treatment, when those audiograms were assessed by our classification. All children whose audiograms were flagged as pathological by our classification finally developed hearing loss. In terms of the prediction of hearing loss, our classification evaluated processing audiograms with a sensitivity, specificity and efficiency of 1.0. Progressive hearing loss was detected in 45 patients (Khan et al. 30, Brock et al. 38). Therefore our classification showed a better suitability for monitoring hearing loss than the other classifications. CONCLUSION: The Muenster classification is a suitable new basis for scientific questions concerning cisplatin ototoxicity. It detects hearing loss earlier and maps progression of hearing loss more precisely than the existing high frequency classifications (Khan et al. and Brock et al.).

[Cisplatin-induced hearing loss in children in relation to eye color]. / Cisplatininduzierte Hörstörungen bei Kindern in Abhängigkeit von der Pigmentierung der Iris.

BACKGROUND: Cisplatin is commonly used as a chemotherapeutic agent in the treatment of solid tumors. Ototoxicity is an important side-effect. Melanin in the inner ear either plays an otoprotective role or has a negative influence on hearing. The concentration of cochlear melanin correlates with its concentration in the iris. PATIENTS AND METHODS: We retrospectively examined 65 children (37 males, 28 females, average age 7.5 years) treated with cisplatin at the University Clinic of Muenster, Germany. We checked whether their eye color could be inferred from the prevalence and extent of cisplatin-induced hearing loss. RESULTS: We found a hearing loss of >20 dB in 29 light-eyed and in 21 dark-eyed patients. Seven light-eyed and eight dark-eyed patients did not suffer from hearing impairment. Using the chi(2)-test on these four parameters, we found no significant connection between iris pigmentation and the prevalence or extent of hearing loss, although light-eyed children (80.6%) suffered more from hearing loss than dark-eyed children (72.4%). After the end of therapy with cisplatin, the prevalence of hearing loss was 83.3% in children up to 6 years and 71.4% in children older than 6 years. The average cumulative dose of cisplatin was 372 mg/m(2) of body surface in children with hearing loss, compared to 390 mg/m(2) in children without hearing loss. CONCLUSION: We found no significant correlation between iris pigmentation (eye color) and hearing loss. Cisplatin-induced hearing loss occurs frequently and requires repeated monitoring.

Melatonin is a useful alternative to sedation in children undergoing brainstem audiometry with an age dependent success rate--a field report of 250 investigations.

Though brainstem audiometry is one of the most important investigations in pediatric audiology, it often necessitates sedation or general anaesthetics, especially in newborns and infants. Melatonin, inducing natural sleep without the risks of sedation, has been successfully used to induce sleep prior to EEG investigations. 250 children (142 male, 108 female) with suspected hearing loss underwent ABR (auditory brainstem responses) tests in melatonin-induced sleep. Click-induced and notched-noise ABR tests were performed. Click tests were successfully performed in 216 of 249 children or 86.7% (123 male, 93 female), notched-noise tests in 115 of 155 children or 74.2%. Failure rates showed an age dependence increasing from 4% in children <1 year to 25%>3 years, but no gender difference. In conclusion, melatonin-induced sleep is a good alternative to sedation, especially in children younger than 3 years. This method is widely accepted by parents and permits earlier diagnosis of hearing impairment in a routine clinical setting. The number of children undergoing general anaesthesia for ABR investigation was reduced from over 60 per year in 2000-2002 to 12 in 2005, which means >80% less general anaesthesia.

[Model oriented assessment of literacy performance in children with cochlear implants]. / Vorschläge zur modellgeleiteten Erhebung der Schriftsprachkompetenzen bei Schulkindern nach Cochlea Implantation - eine Pilotstudie.

BACKGROUND: Although most hearing-impaired children lag behind normally hearing children in literacy acquisition, this aspect has hardly been addressed in the evaluation of language acquisition after cochlear implantation. The present study investigated written language abilities in 8 school-age children with cochlear implants. Neurolinguistic dual-route-models of written language processing indicate that literacy acquisition leads to the establishment of two distinct reading and writing strategies: a lexical one for the quick processing of known words and a sublexical one for decoding unfamiliar words or nonwords letter by letter. PATIENTS: 8 school-aged children were investigated, a very heterogeneous group concerning age of onset of hearing impairment, educational placement, and competences in sign language. However, this range is typical of the group of CI-children. METHODS: The aim was to investigate if children with cochlear implants are able to establish both strategies or if they need to find a differential and individual access to written language. Performance within the Salzburger Lese-Rechtschreib-Test was evaluated. Individual performance of each subject was analysed. RESULTS: Performance varied substantially ranging from only rudimentary spoken and written language abilities in two children to age-equivalent performance in three of them. Severe qualitative differences in written language processing were shown in the remaining three subjects. Suggestions for remediation were made and a re-test was carried out after 12 months. Their individual profiles of performance are described in detail. CONCLUSIONS: The present study stresses the importance of a thorough investigation of written language performance in the evaluation of language acquisition after cochlear implantation. The results draw a very heterogeneous picture of performance. Model-oriented testing and analysis of performance prove to be sensible in at least a subpopulation of children with cochlear implants. Based on a better understanding of their acquired word-processing strategies, remediation programs meeting the needs of each individual child can be derived.

Continuous or repeated prolonged cisplatin infusions in children: a prospective study on ototoxicity, platinum concentrations, and standard serum parameters.

BACKGROUND: To overcome the ototoxicity of cisplatin, single bolus infusions were replaced by repeated prolonged infusions of lower doses or by continuous infusions at still lower infusion rates. However, considering ototoxicity little is, in fact, known about the tolerance of repeated prolonged or continuous infusion in children. PROCEDURE: Auditory function was monitored along with plasma concentrations of free and total platinum (Pt), and with standard serum parameters (sodium, potassium, calcium, magnesium, phosphate, chloride, and creatinine) in 24 children receiving cisplatin by continuous infusion for the treatment of neuroblastoma and osteosarcoma or by repeated 1 or 6 hr infusions for the treatment of germ cell tumors. RESULTS: Hearing deteriorated in 10/15 osteosarcoma patients, 2/3 neuroblastoma patients, and 1/6 patients with germ cell tumors. Ototoxicity occurred after cumulative doses between 120 and 360 mg/m(2) cisplatin. In osteosarcoma patients, ototoxicity was associated with a comparatively higher mean plasma concentration of free Pt. However, Pt plasma concentrations did not discriminate between patients with or without ototoxicity. In patients experiencing ototoxicity serum creatinine increased by 45% compared to pre-treatment levels (mean). Serum creatinine increased by 26% in patients without ototoxicity (P < 0.05, Mann-Whitney Rank sum test). Despite standardized hydration, discrete but significant changes of potassium, sodium, magnesium, and phosphate were observed during and/or after cisplatin infusion, which, however, did not discriminate between patients with and without ototoxicity. CONCLUSIONS: While continuous cisplatin infusions are less nephrotoxic than repeated prolonged infusions, we observed considerable ototoxicity in patients treated with continuous cisplatin infusions, which necessitates further evaluations on the tolerance of continuous cisplatin infusions in children.

[The use of melatonin as an alternative to sedation in children undergoing brainstem audiometry]. / Melatonin als Alternative zur Sedierung bei Durchführung einer Hirnstammaudiometrie bei Kindern.

BACKGROUND: Though one of the most important investigations in paediatric audiology, brainstem evoked response audiometry (BERA) often necessitates sedation or general anaesthetics, especially in newborn and infants. In paediatric neurology, melatonin has been successfully used for some years to induce sleep prior to EEG investigations. Melatonin as a hormone regulating the circadian rhythm induces natural sleep without the risks of sedation. Side effects are not known. METHODS: Click-induced BERA was first performed in 10 adults with normal hearing with and without previous melatonin administration, and click thresholds and latencies of evoked potentials were compared. 50 children then underwent BERA in melatonin-induced sleep. RESULTS: Click thresholds in adults were mostly identical (r = 0,88), while the mean latencies of evoked potentials seemed to be minimally prolonged (r from 0,82 to 0,95). Click-induced BERA was successful in 45 of the 50 children, and notched-noise BERA in at least 2 frequencies in 38 of 43 children. CONCLUSIONS: Offering a high success rate with no side effects, melatonin-induced sleep seems to be a good alternative to sedation. This method is widely accepted by parents and permits earlier diagnosis of hearing impairment in the routine clinical setting.