Longitudinal analysis of the audiological phenotype in osteogenesis imperfecta: a follow-up study.

OBJECTIVE: This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta. METHODS: Audiometric results from 36 osteogenesis imperfecta patients (age range, 6-79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing. RESULTS: Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed. CONCLUSION: After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions.

A pilot cost-of-illness study on long-term complications/sequelae of AOM.

OBJECTIVES: Acute otitis media (AOM) commonly affects young children and occasionally results in serious complications/sequelae. This pilot cost-of-illness study aimed to assess the economic burden of long-term AOM complications/sequelae in Belgium, and to establish a thorough methodology for a larger study. METHODOLOGY: We retrospectively reviewed charts of patients aged 10-20 years with long-term complications/sequelae considered to be AOM-related, and > or = 8 years of follow-up. From a list of 215 eligible patients, we selected 25 patients representing each of seven categories of complications/sequelae. RESULTS: Included patients had a mean age of 12.9 years; nine had chronic suppurative otitis media with cholesteatoma; six sensorineural hearing loss; six chronic perforation of the tympanic membrane; and one each with conductive hearing loss, facial paralysis, neurological impairment after intracranial complications, and complications of surgery. During 8-15 years of follow-up, the most common complications were hearing loss, chronic otitis media (OM), and cholesteatoma. These generally occurred > 5 years after the first AOM event, although chronic OM occurred after a mean time of 3.3 years. Yearly public health care payer (PHCP) costs ranged from Euro 119 to Euro 7957 per patient, and were highest for patients with sensorineural hearing loss. Yearly costs to the patients ranged from Euro 7 to Euro 289 per patient, and were also highest for patients with sensorineural hearing loss. CONCLUSIONS: Although complications/sequelae of AOM are rare, they can result in substantial costs. The applied methodology should be feasible for a larger study, with some minor adjustments.

Cochlear implantation in 3 adults with auditory neuropathy/auditory dys-synchrony.

We describe 3 adult patients with auditory neuropathy/auditory dys-synchrony (AN/AD) who underwent cochlear implantation. All patients had absent or poorly formed auditory brainstem responses (ABRs) in combination with preserved otoacoustic emissions (OAEs). They exhibited various aetiologies and a large variation in clinical features known to be consistent with AN/AD. Cochlear implantation was successful in 2 out of 3 cases. We conclude that AN/AD implantee candidates should be counselled with care.