Etiology of newborn hearing impairment in Guangdong province: 10-year experience with screening, diagnosis, and follow-up.

BACKGROUND: Hearing impairment is one of the most common birth defects in children. Universal newborn hearing screenings have been performed for 19 years in Guangdong province, China. A screening/diagnosis/intervention system has gradually been put in place. Over the past 10 years, a relatively complete data management system had been established. In the present study, an etiological analysis of newborn cases that failed the initial and follow-up screenings was performed. METHODS: The nature and degree of hearing impairment in newborns were confirmed by a set of procedures performed at the time of initial hearing screening, rescreening and final hearing diagnosis. Then, multiple examinations were performed to explore the associated etiology. RESULTS: Over a period of 10 years, 720 children were diagnosed with newborn hearing loss. Among these children, 445 (61.81%) children had a clearly identified cause, which included genetic factor(s) (30.56%), secretory otitis media (13.30%), maternal rubella virus infection during pregnancy (5.83%), inner ear malformations (4.86%), maternal human cytomegalovirus infection during pregnancy (2.92%), malformation of the middle ear ossicular chain (2.50%) and auditory neuropathy (1.81%). In addition, 275 cases of sensorineural hearing loss of unknown etiology accounted for 38.19% of the children surveyed. CONCLUSIONS: Long-term follow-up is needed to detect delayed hearing impairment and auditory development in children. The need for long-term follow-up should be taken into account when designing an intervention strategy. Furthermore, the use of the deafness gene chip should further elucidate the etiology of neonatal hearing impairment.

[Vertigo due to enlarged vestibule with lateral semicircular canal dysplasia: an analysis of clinical characteristics].

OBJECTIVE: To explore the clinical characteristics and possible pathological mechanisms of vertigo due to enlarged vestibule with lateral semicircular canal dysplasia. METHODS: A retrospective review was conducted for 5 cases of peripheral vertigo due to enlarged vestibule with lateral semicircular canal dysplasia. Their characteristics of medical history, precipitating factors, course of vertigo, auditory tests, vestibular tests and imaging examine results were analyzed. RESULTS: The clinical characteristics were as follows. (1) Specifics of medical history: 4 cases suffered delays in gross motor development and potential equilibrium dysfunctions. One case failed to recount an earlier medical history, but could maintain normal hearing and vestibular functions for a long time in adulthood. (2) Most cases could identify the precipitating factors of initial attacks, such as head-bumping, nose-blowing and constipation, etc. resulting in sudden rises of intracranial or abdominal pressures. (3) Paroxysmal vertigo and progressive hearing loss were mimicking Meniere disease or large vestibular aqueduct syndrome. But its course of vertigo was different from those of Meniere disease and large vestibular aqueduct syndrome with regards to hearing levels and audiograms. (4) Some cases had positional vertigo. But the results of Dix-Hallpike and Roll tests were different from benign paroxysmal positioning vertigo (BPPV). (5) The inner ear imaging showed enlarged vestibule with lateral semicircular canal dysplasia. CONCLUSION: The enlarged vestibule with lateral semicircular canal dysplasia is a rare etiology of peripheral vertigo. The history of delays in gross motor development and potential equilibrium dysfunctions in childhood may offer important diagnostic clues. And audiological and vestibular tests, high-resolution computed tomography and magnetic resonance may help to ascertain the diagnosis.

[Acute aggravation of chronic tinnitus: a study of the hearing characteristic and prognosis].

OBJECTIVE: To investigate the hearing change when chronic tinnitus acute aggravated and the affect of prognosis due to hearing change. METHODS: The pure tone threshold and acoustic immittance were used for every case in the acute aggravation of chronic tinnitus (AACT) group, and for some of AACT group members the auditory brainstem response (ABR), distort-product otoacoustic emission (DPOAE), electrocochleogram (EcochG), glycerin test and MRI were used at the same time for further diagnosis. For the chronic tinnitus, the patients accepted the intervention of tinnitus retraining therapy (TRT) both for the AACT group and the control group. As for the recent hearing loss appeared in the AACT group members, the patients were treated the cases as the sudden deafness, and then the hearing characteristic of the AACT group was analyzed and the effect of the chronic tinnitus both for the AACT group and control group after the TRT for 3, 6, 9 and 12 months was evaluated. RESULTS: Three kinds of hearing changes appeared when chronic tinnitus acute aggravated. Based on the dated high frequencies hearing loss ranged 4-8 kHz, recent hearing loss appeared in 1-2 middle frequencies near the impaired high frequencies; Based on the dated single middle frequency hearing loss, another middle frequency hearing impaired near the dated hearing loss frequency, the sawtooth-like audiogram changed to groove-like audiogram; Hearing fluctuation in low frequency area, hearing threshold increased 10-30 dB in 125-1000 Hz but kept unchanged in the high frequency area. The recent local frequency hearing loss healed after the vasodilator and neurotrophic drugs therapy, but the dated hearing loss can't rehabilitate. Along with the rehabilitation of recent local hearing loss, 25 of 32 cases chronic tinnitus victims get to habituation; this finding showed that the experience of tinnitus aggravation and relief accelerated the habituation. Until the 12 months after the TRT, the curative effect difference between AACT group and control group showed statistical significance. CONCLUSIONS: When the chronic tinnitus acute aggravated, 3 kinds of local area hearing impaired. Timely diagnosis and effective treatment had brought not only hearing rehabilitation but also promotion to habituation of chronic tinnitus.