Cochlear nerve dysplasia in unilateral severe to profound congenital sensorineural hearing loss - Prevalence in Australian children and the impact of socioeconomic disadvantage on its management.

INTRODUCTION: Congenital unilateral sensorineural hearing loss (cuSNHL) carries potentially significant social, educational, and developmental consequences. Early diagnosis enables investigation, and consideration of options for management and early intervention, helping to mitigate the effects of hearing loss. Cochlear nerve dysplasia (CND) is a prominent cause of cuSNHL and may affect candidacy for cochlear implantation. Socioeconomic disadvantage may impact on a patient's family's capacity to participate in necessary intervention and follow-up. METHODS: Infants with severe-profound cuSNHL referred to a large Australian quaternary pediatric center between October 2004 and December 2020 were retrospectively included. Audiometric and clinical data, and the presence of hearing loss risk factors were obtained from a prospectively collated database. In Australia MRI scans are provided free-of-charge to citizens and residents. MRI scans were reviewed to determine the status of the nerves within the internal acoustic meatus (IAM grade) along with attendance rates. Travel distance to the hospital was also calculated. Reasons for non-attendance at MRI were obtained from patient medical records and correspondence. Socioeconomic, educational, and occupational indices, and travel distances were obtained using patient residential postcodes with reference to Australian Bureau of Statistics data. RESULTS: A total of 98 patients were reviewed, 64.3% (n = 63) of whom underwent MRI. The median age at diagnosis was 40 days (IQR 27). The prevalence of CND was 75% (n = 47). Importantly, there was no significant difference in the degree of hearing loss between IAM grades (F(4,57) = 1.029, p = 0.405). Socioeconomic indices were significantly lower in patients not attending MRI investigations compared with patients who did attend. Travel distance was not significantly different between the two groups. CONCLUSION: CND is a prominent cause of cuSNHL in Australian infants. MRI at a young age allows parent education regarding management options and timely intervention where indicated. Socioeconomic disadvantage significantly impacts on participation in further routine assessment of cuSNHL, potentially limiting management options for these children long term.

Management of otitis media with effusion in children with primary ciliary dyskinesia: a literature review.

OBJECTIVE: Primary ciliary dyskinesia is an autosomal recessively inherited group of disorders of ciliary ultrastructure. Otolaryngologists are frequently involved in the management of some of the most common symptoms of primary ciliary dyskinesia including chronic rhinitis, sinusitis and otitis media with effusion. A dilemma for otorhinolaryngologists is whether ventilation tubes are of benefit in children with primary ciliary dyskinesia and otitis media with effusion and what effective alternatives exist. This paper aims to address this issue via a literature review and case presentation. METHODS: An extensive review of the literature was undertaken and a discussion of the advantages and disadvantages of ventilation tubes in the management of otitis media with effusion in these children is presented and compared with that of the general population. We present a case of a 9 months old boy with Kartagener's Syndrome and chronic bilateral otitis media with effusion to illustrate our findings. RESULTS: Eight papers were identified, all with small study numbers. The main outcome measures were hearing, otorrhoea and tympanic membrane structural changes. The natural history of otitis media with effusion and hearing loss in primary ciliary dyskinesia appears to be fluctuant into adulthood. Therefore, otitis media with effusion in primary ciliary dyskinesia does not resolve by the age of 9 years, regardless of treatment, as previously assumed. Ventilation tube insertion improves hearing in primary ciliary dyskinesia, but may lead to a higher rate of otorrhoea when compared to the general population. Tympanic membrane changes were clinically insignificant. Our patient eventually underwent successful insertion of bilateral ventilation tubes with a marked improvement in hearing and language with minimal otorrhoea. CONCLUSION/DISCUSSION: The highest level of evidence found for the management of otitis media with effusion in children with primary ciliary dyskinesia was level IV. Currently, the evidence is inconclusive and conflicting. Whilst our results are promising, clearly higher quality research on a larger number of patients is required to definitively evaluate the management options for otitis media with effusion in these children.

Hearing loss associated with large internal auditory meatus: a report of five paediatric cases.

This paper describes the abnormality of a large internal auditory meatus (LIAM). Computed tomography (CT) scans show the otic capsule to be affected by a widened, bulbar internal auditory meatus with loss of or reduction of the bony wall dividing the lateral fundus of the meatus from the cochlea. The vestibule is abnormally dilated. We report five cases of children with LIAM and profound hearing loss. Three of these children are girls and two children were boys. Three had congenital progressive hearing loss, one of these had an accompanying large vestibular aqueduct and dysplasia of the cochlea. Two patients had had meningitis resulting in profound loss.

Medial canal stenosis--chronic stenosing external otitis.

Chronic stenosing external otitis is an indolent condition that is difficult to treat. Twelve cases that were operated on during a 3-year period were reviewed. The results of treatment are presented and the surgical methods are discussed. This involves excision of all fibrous tissue and if necessary the drum, a wide canalplasty, a meatoplasty, and then reconstruction with a fascial graft if necessary followed by split skin grafts, with emphasis on maintaining the anterior tympanomeatal angle.