Respiratory papillomatosis is a rare and often severe disease, usually localized in the larynx. It may cause respiratory distress and even life-threatening obstruction of the airways. Treatment is generally based on the evaporation of the lesions with a CO2 laser, but microsurgery, cytotoxic and/or cytostatic drugs, interferons, and vaccines are also used. Cidofovir [(S)-1-(3-hydroxy-2-phosphonylmethoxypropyl)cytosine] (HPMPC) was shown to suppress the growth of tumors induced by rabbit papillomavirus as well as human papillomavirus (HPV). The efficacy of cidofovir was assessed in 17 patients with severe respiratory papillomatosis. Cidofovir at a concentration of 2.5 mg/ml was injected directly in the different laryngeal papillomatous lesions during microlaryngoscopy under general anesthesia. Biopsies were taken before the treatment was started both for anatomopathology and viral typing. HPMPC kinetics in serum was monitored in three patients, the drug levels being determined by high-performance liquid chromatography. Complete disappearance of the papillomatosis was observed in 14 patients. Four patients relapsed and were successfully treated again with cidofovir. Of the three remaining patients, one progressed while under treatment with cidofovir, after an initial marked response. One patient had a partial remission and remained stable for more than 1 year after the last injection. He had a very aggressive and extensive disease originally. Finally, one patient was lost to follow-up after four injections. Intratumoral injections of cidofovir for the treatment of severe laryngeal papillomatosis is a powerful new therapeutic approach for this disease. Treatment was well tolerated, and no significant side effects were noted.

Experience with dichotic multiple-stimulus auditory steady-state responses (ASSRs) in clinical practice is described. ASSR thresholds were assessed in a sample of 60 high-risk newborns and young children between birth and 4 years of age. Amplitudes and signal-to-noise ratios (SNRs) of the ASSR were compared between normal-hearing infants and adults. Age-related changes within a group of infants younger than 3 months of age were investigated. A comparison was made between ASSR, the click-evoked auditory brainstem response and behavioral hearing thresholds in infants with a wide range of hearing threshold levels. Mean ASSR thresholds for normal-hearing infants at an average corrected age of 12 days were 42 ± 10, 35 ± 10, 32 ± 10 and 36 ± 9 dB SPL for 0.5, 1, 2 and 4 kHz, respectively. Compared to adults, these thresholds were elevated by on average 11 dB and SNRs were 1.7 times smaller. However, based on ASSRs, reasonably accurate estimations could be made of behavioral hearing thresholds obtained at a later age (median delay of 7 months). The predicted thresholds were in 61% of the cases within 10 dB of the corresponding behavioral thresholds, and in 83% of the cases within 15 dB. In less than 1 h, thresholds at four frequencies per ear could be obtained. The optimal age of testing is between 1 week and 3 months corrected age. The dichotic multiple-stimulus ASSR technique is a valuable extension of the clinical test battery for hearing-impaired children, as a follow-up diagnostic after the neonatal hearing screening.

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We present sound localization results from 30 children with bilateral cochlear implants. All children received their implants sequentially, at ages from 6 months to 9 years for the first implant and 1.5–12 years for the second implant, with delays of 10 months to 9 years. Localization was measured in the sound field, with a broadband bell-ring presented from 1 of 9 loudspeakers positioned in the frontal horizontal plane. The majority of the children (63%) were able to localize this signal significantly better than chance level. Mean absolute error scores varied from 9 to 51° (root mean square error scores from 13 to 63°). The best scores were obtained by children who received their first implant before the age of 2 years and by children who used hearing aids prior to implantation for a period of 18 months or longer. Age at second implantation was important in the group of children who did not use a contralateral hearing aid during the unilateral implant period. Additionally, children who attended a mainstream school had significantly better localization scores than children who attended a school for the deaf. No other child or implantation variables were related to localization performance. Data of parent questionnaires derived from the Speech, Spatial and Qualities of Hearing Scale were significantly correlated with localization performance. This study shows that the sound localization ability of children with bilateral cochlear implants varies across subjects, from near-normal to chance performance, and that stimulation early in life, acoustically or electrically, is important for the development of this capacity.

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The estimated incidence of sensorineural hearing impairment (>40 dB HL) at birth is 1.86 per 1000 newborns in developed countries and 30-40% of these are unilateral. Profound sensorineural unilateral hearing impairment or single sided deafness (SSD) can be treated with a cochlear implant. However, this treatment is costly and invasive and unnecessary in the eyes of many. Very young children with SSD often do not exhibit language and cognitive delays and it is hard to imagine that neurocognitive skills will present difficulties with one good ear. In the current paper we review the most recent evidence on the consequences of unilateral hearing impairment for auditory and neurocognitive factors. While data of both adults and children are discussed, we focus on developmental factors, congenital deafness and a window of opportunity for intervention. We discuss which etiologies qualify for a cochlear implant and present our multi-center prospective study on cochlear implants in infants with one deaf ear. The large, state-of-the art body of research allows for evidence-based decisions regarding management of unilateral hearing loss in children.

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Frequency tuning and phase-locking are two fundamental properties generated in the cochlea, enabling but also limiting the coding of sounds by the auditory nerve (AN). In humans, these limits are unknown, but high resolution has been postulated for both properties. Electrophysiological recordings from the AN of normal-hearing volunteers indicate that human frequency tuning, but not phase-locking, exceeds the resolution observed in animal models.