Secondary, profound, sensorineural hearing loss after recovery from haemolytic uraemic syndrome due to enterohaemorrhagic Escherichia coli, and subsequent cochlear implantation, in two Japanese children.

OBJECTIVES: To describe two cases of profound hearing loss secondary to enterohaemorrhagic Escherichia coli infection, and to report the efficacy of subsequent cochlear implantation. RESULTS: The first case was a four-year-old girl admitted to hospital with Escherichia coli O157 infection and haemolytic uraemic syndrome. Mild hearing loss was confirmed five months after discharge, progressing to profound loss three months later. At the age of seven years, she underwent cochlear implantation, with remarkable improvement in speech perception and production. The second case was a three-year-old boy admitted with haemolytic uraemic syndrome caused by Escherichia coli O111 infection. One year after disease onset, profound hearing loss was confirmed. Cochlear implantation at the age of five years produced significant recovery of auditory function. CONCLUSION: This study represents the first published report of secondary hearing loss after recovery from haemolytic uraemic syndrome caused by enterohaemorrhagic Escherichia coli. It indicates that cochlear implantation can restore hearing function in such patients.

A prevalent founder mutation and genotype-phenotype correlations of OTOF in Japanese patients with auditory neuropathy.

Auditory neuropathy is a hearing disorder characterized by normal outer hair cell function and abnormal neural conduction of the auditory pathway. Aetiology and clinical presentation of congenital or early-onset auditory neuropathy are heterogeneous, and their correlations are not well understood. Genetic backgrounds and associated phenotypes of congenital or early-onset auditory neuropathy were investigated by systematically screening a cohort of 23 patients from unrelated Japanese families. Of the 23 patients, 13 (56.5%) had biallelic mutations in OTOF, whereas little or no association was detected with GJB2 or PJVK, respectively. Nine different mutations of OTOF were detected, and seven of them were novel. p.R1939Q, which was previously reported in one family in the United States, was found in 13 of the 23 patients (56.5%), and a founder effect was determined for this mutation. p.R1939Q homozygotes and compound heterozygotes of p.R1939Q and truncating mutations or a putative splice site mutation presented with stable, and severe-to-profound hearing loss with a flat or gently sloping audiogram, whereas patients who had non-truncating mutations except for p.R1939Q presented with moderate hearing loss with a steeply sloping, gently sloping or flat audiogram, or temperature-sensitive auditory neuropathy. These results support the clinical significance of comprehensive mutation screening for auditory neuropathy.

[The duty of hospice for holistic medicine].

The hospice program has a sense of absolute in the context of the provision of treatment by the medical establishment. The aim of the medical establishment is humanistic treatment. The aim of the hospice program is to allow doctors in charge, thru their medical skills, to make the most effort for the life of the patient. As such, the goal of the hospice program is not just to ease the physical pain of terminal patients, but rather to help the patient live the remainder of his or her life in a meaningful way, offering intensive support and energy to help the patient challenge and even defy his or her life. Today in Japan, the hospice care program has established a system of renumeration for use of special cancer wards and pain-easing treatments. Because of this, hospice care is becoming wide-spread among medical care organizations. Such wide-spread care is a very desirable thing. But if the economic value of hospice care is only to ease physical pain, then hospice care is essentially a formless act. If we do not effect the appropriate value both socially and economically, one wonders if the hospice care in Japan will be able to develop the correct approach.