Bacterial meningitis is an important cause of acquired sensorineural deafness in childhood. Deafness following meningitis may be progressive. Previous reports have shown deterioration in hearing up to 12 years after the illness. We present two cases of sensorineural deafness following meningitis. Severe to profound sensorineural hearing losses were detected immediately after meningitis in these patients. The hearing subsequently deteriorated in both cases. Deterioration in hearing thresholds occurred 17 years after the illness in one case. In the other patient the hearing got progressively worse three years after meningitis. She subsequently required a cochlear implant.

ObjectiveThe aim of this study was to obtain national data regarding adherence to national guidelines for aetiological investigations for hearing loss in children and highlight any variations in practice. Information was also collected on possible factors affecting lack of adherence.DesignAn online questionnaire based on the national guidelines for aetiological investigations for deafness was designed.SettingThe questionnaire was distributed to the leads of all the Newborn Hearing Screening Programme (NHSP) sites across England through the Medical Research Council Hearing & Communication Group.ParticipantsThe questionnaire was sent to 100 recipients; from this 52 responses were obtained.Outcome measuresVariability in the investigations offered for hearing loss.ResultsThere was a 52% response rate. Analysis of the responses showed that audiovestibular physicians and paediatricians in audiology were more likely than other specialists to request level 1 investigations (investigations that are recommended to be offered in all cases). Respondents from London and the North West were more likely to request level 1 investigations compared with those from other regions. In all, 14 of the 19 audiovestibular physicians and paediatricians in audiology requested level 1 investigations routinely, but only 11 of 33 from other specialties did likewise. Of the 20 respondents from London and the Northwest, 15 requested level 1 investigations routinely, whereas only 10 of the 32 respondents from the other regions did the same. The difference was statistically significant in both cases. The geographical variation was specially marked for family audiograms and MRI.ConclusionsThere is significant variation from the national guidelines in requesting aetiological investigations for permanent hearing impairment (PHI) in children, depending on the specialty of the clinician and the geographical region, these variations appear partly to be due to the availability of local resources but also due to lack of awareness of the importance of some investigations.

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Connexins are the building blocks of gap junctions. Mutations in connexin are the most common cause of non-syndromic hereditary deafness. The authors looked at the results of connexin mutations in children identified with varying types (unilateral and bilateral) and degrees of their hearing losses since 2002 in Bolton.Our case mix consisted of children identified through the Newborn Hearing Screening Programme, which was introduced in our area in 2003, and older children with hearing loss already in the system.Connexin 26 was tested in all children identified with permanent childhood hearing impairment, but more recently connexin 30 is also being tested.The authors present the variability of mutations in connexin in relation to their phenotype.